Clinical Vignette and Research Abstract Compendium ACP Southern California Chapters I and III Scientific Meeting SGIM California Regional Meeting October 23-25, 2009 Hyatt Grand Champions Resort and Spa Indian Wells, California Table of Contents: (Within category, abstracts are organized by first letter of first authorвЂ™s last name) Clinical Vignette Abstracts: p. 3-77 Research Abstracts: p. 77-91 Dear Readers, The Program Committee of the first joint ACP Regions 1 and 3 Scientific Meeting/ California SGIM Regional Meeting congratulates the abstract authors on their excellent submissions. This year, authors submitted a record number of abstracts. The high quality reflects the diligence, intelligence, integrity, and creativity of the authors and their advisors/mentors. We thank the abstract reviewers for their difficult task of selecting abstracts for oral presentation. And we appreciate the oral and poster competition judges for their similarly-difficult task. Sincerely, Simerjot K. Jassal, MD, MAS, FACP, Co-Chair Nader Kamangar, MD, FACP, FCCP, Co-Chair Alpesh N. Amin, MD, MBA, FACP Daniel S. Anderson, MD, FACP Dennis W. Cope, MD, FACP Joel C. Diamant, MD, FACP Carla Fox, MD, FACP Charles G. Goldberg, MD Mark Richman, MD, MPH Soma Wali, MD, FACP Louise Walter, MD Natalie Kazcur 2 Clinical Vignette Abstracts Two for One: An Unusual Case of Weakness Pouya Afshar, MD; Nekhonti Adams; Payam Afshar Scripps Green Hospital A sixty-eight yearвЂ“old female was admitted for a oneweek history of progressive lower extremity weakness and associated falls. She denied any loss of consciousness, back pain, myalgias, numbness, paresthesias, bowel or bladder incontinence, recent illnesses, or change in medications. Immediately preceding her falls, the patient reported a transient weakness in her thighs that persisted for approximately thirty minutes and resolved spontaneously. Past medical history was significant for hyperlipidemia, essential tremors, and a seventy-five pack year history of smoking. Pertinent exam findings included inspiratory and expiratory wheezing, and bilateral 4/5 strength in the proximal lower extremity muscles with diminished reflexes. Laboratory evaluation revealed a sodium of 110 mmol/L, urine osmolality of 279 mOsm/kg, and serum osmolality of 230 mOsm/kg. Serum creatinine, blood urea nitrogen, random cortisol, thyroid stimulating hormone, creatine kinase, and erythrocyte sedimentation rate were all found to be normal. Given the pulmonary exam and the likely diagnosis of a syndrome of inappropriate antidiuretic hormone secretion, a chest xray was ordered and showed a mediastinal mass. A subsequent CT of the chest confirmed a mediastinal mass, as well as secondary compression of the right pulmonary artery and mainstem bronchus. To avoid further respiratory compromise, an emergent bronchoscopy/biopsy and radiation therapy were ordered. The pathology confirmed a diagnosis of limited stage small cell lung carcincoma, with no evidence of metastasis on further imaging studies. Despite correction of the hyponatremia from 110 to 130 mmol/L, the patientвЂ™s weakness did not improve during her hospitalization. Prior to discharge, a voltage-gated calcium channel (VGCC) antibody test was ordered to rule out the possibility of another paraneoplastic syndrome (Lambert-Eaton). Within two weeks, the VGCC was found to be positive, confirming a diagnosis of Lambert-Eaton syndrome as the etiology of her lower extremity weakness. After several rounds of chemotherapy and radiation, there is no further evidence of active tumor and the weakness has resolved without any further intervention. This case highlights a patient with two paraneoplastic syndromes, SIADH and Lambert-Eaton Syndrome. The latter represents a very rare, pre-synaptic defect that is associated with small cell lung carcinoma in fifty percent of cases. While there have been several documented cases describing SIADH or Lambert-Eaton Syndrome in association with small cell lung carcinoma, this is only the second case report where a patient is afflicted with both paraneoplastic syndromes. Intense Pruritus Requiring Hospitalization Secondary to Acute EBV Hepatitis Satyanisth Agrawal, MD; Alok Jain, MD; Kevin Mullen, MD; Neil Kaplowitz, MD University of Southern California Pruritus is a complication of chronic liver disease. The prevalence of pruritus in liver disease in general varies from 5% in patients with chronic hepatitis C to 70% in patients with Primary Biliary Cirrhosis. Epstein Barr Virus (EBV) infection causes asymptomatic liver-associated enzyme abnormalities in 80 to 90% of cases, which are often unrecognized. Patients with acute EBV infections may also develop cholestatic hepatitis with associated jaundice (< 5% of cases) and hepatitis with moderate elevation in transaminase. We report a case of intense pruritus in cholestatic jaundice secondary to EBV hepatitis requiting hospital admission for treatment. A 39 year old Caucasian male was referred to our out-patient clinic with 3-week history of generalized weakness, jaundice with worsening LFTвЂ™s, & pruritus. The onset of these symptoms was preceded with 5 days of fever, chills, nausea and vomiting, decreasing appetite, and diarrhea. A trial of hydroxyzine and SSRIs by his primary physician had failed to control his pruritus. His PE was remarkable for icterus, an enlarged right anterior cervical lymph node, and multiple linear excoriations on his extremities and trunk from pruritus. Lab tests showed a mixed picture with increased transaminases (resolving) and increased bilirubin (worsening). He had a (+) Monospot; EBV serology confirmed acute infection. Initial management included supportive care and ursodiol in addition to the antihistamines. However, the patient returned with worsening symptoms and was noted to have pruritus severe enough to cause significant insomnia and lethargy. During the next few appointments at the liver clinic he was tried on rifampicin, diphenylhydramine, cholestyramine and naltrexone, none of which helped him. He stopped naltraxone after the 1st dose due to development of electric shock like symptoms, shakes including numbness and paresthesias involving the extremities. Having exhausted all options and worsening pruritus, he was admitted for naloxone infusion to introduce an opiate antagonist slowly to prevent the withdrawal reaction. The patient was started on an ultra low dose naloxone drip (0.02 Вµg/kg/min) and over 24 hours titrated up to a rate of 0.8 Вµg/kg/min. He tolerated the treatment without adverse effects and reported complete relief from pruritus. The patient was discharged home on low dose naltrexone tablets. He remained asymptomatic from pruritus during follow up visits over one month. This report illustrates successful treatment of severe pruritus with naloxone drip in the setting of cholestatic liver disease secondary to acute EBV infection requiring hospitalization after failing out-patient therapy. Classic Presentation of Diffuse B-Cell Lymphoma Kevan Akrami, MD; Seanna Grob, BS University of California, San Diego Case: Diffuse Large B cell Lymphoma (DLBCL), the most common Non-Hodgkins Lymphoma, classically presents as a rapidly growing mass fatal if untreated. Here we present a case report of the prototypical presentation and clinical course of DLBCL. A 56 year old man with three week history of a dental abscess presents to the emergency department for evaluation of an enlarging neck mass. His initial symptoms include a severe sore throat and subjective fevers lasting one week that improved briefly with aspirin and fluids. Concurrent with this he developed a large minimally tender, hard left sided neck mass that grew over the course of three weeks causing a feeling of fullness in his throat. He notes вЂњchokingвЂќ sensation preventing him from lying on his right side or back. He has no myalgias, nausea, vomiting or night sweats, though reports intentional twenty-five lb weight loss over three months. No report of recent rash, arthralgias or changes in urinary or bowel habits. He has no recent travel or exposure to un-pasteurized milk or animals. On physical exam, he is comfortably lying in bed without any visible respiratory distress. Neck exam reveals a large, well circumscribed, hard mass extending from angle of the jaw to clavicle and a 2-3 cm left tonsillar mass partially obstructing his posterior oropharynx. He has no other lymphadenopathy, organomegaly, or skin lesions. Lungs are clear, and no peripheral edema. On admission, labs reveal a normal CBC and differential, negative RPR. Bacterial culture from the mass grew pansensitive Klebsiella pneumoniae with negative acid fast stains, fungal stains and cultures. Serologies were negative for Brucella, EBV, CMV. CT imaging revealed an irregular rim enhancing fluid collection in the left lateral neck surrounded by inflammation concerning for abscess versus a mass of necrotic masses secondary to neoplasm. He underwent an excisional biopsy and left tonsillectomy by surgery. Flow cytometry revealed a monoclonal population of B-lymphocytes and pathology of the biopsy showed high grade diffuse large B-cell lymphoma. Workup for possible metastatic disease was initiated including CT chest, abdomen, pelvis followed by PET-CT. All studies were negative for other involved sites. He underwent a bone marrow biopsy that revealed no evidence lymphoma and was started on CHOP chemotherapy. Discussion: This case report of rapid onset, history of molar abscess and sore throat initially seem consistent with an infectious. Clues to DLBCL include large, hard, non-tender mass in the neck and night sweats. An infectious etiology would more likely be associated with growth that is tender to palpation. DLBCL is known to be an aggressive subtype of Non-HodgkinвЂ™s lymphoma traditionally treated with chemotherapy regimens including doxorubicin with rituximab adjuvant therapy to improve overall survival and 5-year disease free intervals. Atrial Fibrillation as a Complication of Anomalous Pulmonary Venous Connection and Atrial Septal Imperfection Ashkan Amooee, MD; Omar Darwish, DO; Alexander Stein, MD; Ravi Menghani, MD; Jasminka Criley, MD, FACP, FHM St. Mary Medical Center and David Geffen School of Medicine at UCLA Case: A 44 year-old Hispanic man was admitted with a 2-day history of atypical chest pain. He was found to have atrial fibrillation (Afib) with rapid ventricular response. His past medical history included Afib, HTN, dyslipidemia, DVT and вЂњheart murmurвЂќ. Physical exam revealed an irregularly irregular heart rhythm and elevated JVP. ECG showed Afib with rapid ventricular rate and right bundle-branch block. A ОІ-blocker and digoxin were instituted. Patient was ruled-out for myocardial infarction. Repeated cardiac examination detected a fixed splitting of S2 and 2/6 midsystolic murmur best heard at the 2nd LICS. Upon obtaining outside records it was discovered that the patient was worked up extensively 2 years prior with right- and left heart catheterization disclosing a sinus venosus atrial septal defect (SVASD) and an anomalous pulmonary venous connection (APVC) but вЂњcleanвЂќ coronary arteries. He had been offered surgical intervention at that time, but had declined. At this admission, it was felt that patient would benefit from a surgical repair of the ASD and APVC given impending risk for worsening heart failure and related symptoms. He underwent repair with an autologous pericardial patch, covering the ASD and redirecting the pulmonary veins to the left atrium through the septal defect. He also underwent a modified maze procedure to eliminate Afib. Patient tolerated surgery well. Postoperatively, he required a dualchamber pacemaker due to persistent junctional rhythm with heart rates ranging from 40-50. He was discharged home in stable condition. Discussion: Atrial fibrillation is the most common cardiac arrhythmia. One of the less common causes of Afib is congenital heart disease. ASD is the most common congenital cardiac lesion presenting in adults. SVASD accounts for only 10% of the ASDвЂ™s. The more common Sinus Venosus type defect occurs in the upper atrial septum and is contiguous with the superior vena cava (SVC). It is almost always associated with anomalous pulmonary venous drainage of the right upper pulmonary vein into the SVC. People with congenital ASD can be asymptomatic through their 20вЂ™s, but by age 40, most will have symptoms including dyspnea, Afib and eventually heart failure. Individuals with small defects can live a normal life, but larger defects require surgical correction. The mortality from corrective surgery in people younger than 45 years of age is < 1%. In the adult population, congenital anomalies are commonly associated with Afib. This is an unusual case of SVASD with 4 anomalous pulmonary veins, who underwent surgical repair and maze procedure to eliminate Afib. Untreated ASDвЂ™s are associated with a significantly shortened life expectancy. Operation for SVASD is associated with low morbidity and mortality, and postoperative subjective clinical improvement occurs irrespective of age at surgery. SVASD repair should be considered whenever repair may impact survival or symptoms. 4 Innocent Presentation of a Malignant Pheochromocytoma Gaja Andzel, MD; Silvana M. Giannelli, MD; Mauro C. Bandeira Janoski, MD Loma Linda University Malignant Pheochromocytomas (MPCCs) are rare catecholamine producing tumors derived from chromafin cells from the adrenal medulla. Their diagnosis can be challenging and to date, there is no effective cure. We present a case with atypical clinical picture to illustrate manifestations, diagnosis and current treatment modalities. A 71 year-old African-American male was evaluated for a growing left adrenal incidentaloma. The mass was first seen by CT in 1991 and had been stable at 4 cm for 15 years. During his initial visit he complained of 20-pound weight loss in 6 months and fatigue. Past medical history included chronic hypertension controlled on three agents, stroke and diabetes. No relevant family history. His blood pressure was 138/78 mmHg and pulse was 109 bpm. His exam was only remarkable for cachexia. Twenty-four hour urine collection showed very high normetanephrine, slightly high norepinephrine, normal dopamine and normal epinephrine levels. Terazosin was started prior to left adrenalectomy. Pathology was consistent with pheochromocytoma (PCC). Surgical margins were clear, but the specimen showed necrosis and limphovascular invasion. Catecholamine levels normalized after surgery. Unfortunately, the patient was lost to the follow-up and returned after 2 years. PET/CT scan showed a recurrent 1.3 cm left adrenal mass and new multifocal liver lesions. Patient underwent chemoembolization of liver lesions which was complicated by liver abscess. Methyrosine was started to control his blood pressure, as catecholamine levels were increased. Radiolabeled metaiodobenzylguanidine scintigraphy (MIBG) showed uptake limited only to right hepatic lobe, the PET/CT scan was repeated and showed multiple foci of intense activity in both lobes of the liver as well as adrenal mass. The bone scan was negative for metastasis. Based on these results chemotherapy with Cyclophosphamide, Vincristine and Dacarbazine (CVD) was recommended. Although most MPCCs have a rapid progression they can have a highly variable clinical course. Our case did not have any of the classic triad of PCC (episodic headache, sweating and palpitations). Main symptoms were weight loss and fatigue, which are described occasionally. He also had sustained hypertension, which is actually more frequent than paroxysmal hypertension. Most PCCs produce both norepinephrine and epinephrine. Ours was a rare case of adrenal PCC secreting norepinephrine alone. Malignant and benign tumors are indistinguishable clinically; the only reliable criteria for malignancy are distant metastasis. Alpha blockage and Methyrosine are useful pharmacological treatments, but surgery is still the mainstay approach. Chemotherapy and MIBG have roughly the same results, with relapse of most cases within two years. Although prognosis is difficult to predict most MPCCs have a 50% five-year survival rate. There are no published randomized trials evaluating available therapies. In conclusion, it is important to the clinicians to have a working knowledge about adrenal incidentalomas, PCCs, their identification and treatment options. A Case of Coarctation of the Aorta in a Young Adult Female Anthony Arredondo, MD; Janine Vintch, MD Harbor-UCLA Medical Center A 19 year old woman with a history of HTN diagnosed 2 months prior to admission complained of a 2 week history of fatigue and light-headedness associated with intermittent palpitations. She denied chest pain, shortness of breath, nausea, vomiting, or headache. She reported strict adherence to her BP meds including HCTZ and clonidine. BP 220/110 mm Hg, HR 89. She was in no acute distress. PE was notable for no carotid bruits or elevated JVP, lung fields clear to auscultation, a 2/6 systolic ejection murmur over the left sternal border, and normal abdominal examination with no bruits. It was noted that she had non-palpable distal pulses and skin that was cool to touch in her lower extremities bilaterally as compared to her upper extremities. Upon noting this discrepancy in pulses and skin temperature, bilateral brachial and ankle blood pressures were measured. Her brachial blood pressures remained elevated and were equal, while her ankle blood pressures were equal but measured 100 mg Hg lower systolic BP as compared to the brachial readings. Electrolytes and renal function were normal. Tests to evaluate secondary causes of hypertension were unremarkable. Her chest x-ray was without any findings including no evidence of rib notching CT angiogram demonstrated coarctation of the aorta arising distal to the left subclavian artery with the narrowest point measuring only 7.5 mm. Several enlarged bronchial and intercostals arteries were also noted. A transthoracic echocardiogram confirmed this radiographic finding and measured a peak transcoarctation velocity of 4.7 m/s and a peak gradient of 89 mm Hg. Renal ultrasound demonstrated tardusparvus waveforms in blood flow to both kidneys; a renal captopril scan showed normal perfusion and cortical uptake bilaterally. BP was controlled with three oral medications; she was discharged with follow-up with cardiothoracic surgery. The presence of HTN requiring medical therapy in young adults may suggest the presence of a number of conditions including coarctation of the aorta, fibromuscular dysplasia, or an endocrine disorder such as pheochromocytoma, primary hyperaldosteronism, or CushingвЂ™s Disease. The clinical clue to this patientвЂ™s diagnosis was not only the young age of onset for severe HTN, but also the presence of non-palpable pulses in her lower extremities concerning for coarctation of aorta. 5 May-Thurner Syndrome: Coumadin Failure or Treatable Condition? Benton Ashlock, MD Scripps Green Hospital A 57 year-old female with a history of well controlled hypertension presented to the ED with acute onset of severe left leg pain, swelling, and discoloration. Three months prior, she suffered a pulmonary infarction and was found to have a concurrent 15cm left ilio-femoral thrombosis. At that time anticoagulation initiated and due to the size and appearance of the thrombus and inferior vena cava filter was placed. A CT-venogram performed at the time of the current admission demonstrated flattening of the left common iliac vein as it passed under the right common femoral artery with thrombosis extending down to the popliteal vein. A large embolic-like thrombosis on the inferior vena cava filter was also noted. Lab records indicated she remained therapeutically anticoagulated during the three months between incidents. She subsequently underwent successful thrombolysis with tissue plasminogen activator which resulted in complete clearing of the thrombosis. Reduced flow continued to be present despite absence of thrombosis. Based on the CT venogram and the continued reduced blood flow, a diagnosis of May-Thurner syndrome was made. May-Thurner syndrome describes the occurrence of deep vein thrombosis due to significant compression of the left common iliac vein by the overlapping proximal right common iliac artery. Anatomically the overlap of theses two vessels is normal but in some patients, particularly young women, the elevated arterial pressure and thickness of the vascular wall of the overlying iliac artery produces sufficient venous compression resulting in hemodynamic compromise and subsequent venous thrombosis. External vascular compression from May-Thurner Syndrome or other external compressive forces should be seriously entertained in all patients for whom therapeutic anticoagulation is unsuccessful in preventing thrombosis recurrence. A small number of case series have reported excellent long term success with iliac vein stent placement combined with short term anti-coagulation. If undiagnosed, patients with May-Thurner syndrome routinely continue to have thrombosis despite therapeutic anticoagulation. Patel, et. al. demonstrated that these patients can remain symptom free after thrombolysis and venous stent placement. Early recognition of MayThurner syndrome can prevent the severe morbidity and mortality associated with recurrent deep venous thrombosis as well as chronic anti-coagulation. CT venography to evaluate for extrinsic vascular compression should be considered, particularly in patients with large, proximal deep vein thrombosis of the left lower extremity and in all such patients who fail appropriate anticoagulation therapy. Steroid-Induced Hepatotoxicity in a Patient with Transverse Myelitis Kimberly Atianzar, MD; Cong Nguyen, MD; Mary El-Masry, MD; John J. Kim, MD; Neil Kaplowitz, MD University of Southern California Hepatotoxicity is an uncommon clinical course in patients requiring steroids. We report the case of a patient with transverse myelitis in which treatment with a 3-day course of high dose steroids resulted in acute hepatitis and cholestatic injury. A 52 year old Hispanic Male with a history of chronic hepatitis C and hypertension presented to the Emergency Department with jaundice, dark brown urine, generalized fatigue, fecal incontinence, and progressively worsening bilateral lower extremity weakness and numbness over the past 3 weeks. He denied any other systemic symptoms. Pertinent physical findings included bilateral sclera icterus, jaundice and 3/5 strength of lower extremities bilaterally. Remaining physical examination was unremarkable. Initial laboratory workup showed normal basic metabolic panel and complete blood count. Liver function panel was normal with the exception of aspartate aminotransferase (AST) level of 52 U/L and alanine aminotransferase (ALT) level of 77 U/L. MRI of the lumbar spine was unremarkable and showed no evidence of cord compression. CT scan of the brain was unremarkable. Lumbar puncture was negative for evidence of infection. The patient was treated with a 3day course of 1gram IV Methylprednisolone for transverse myelitis in which his symptoms significantly improved, and he was transferred to a rehabilitation facility. During his physical rehabilitation, the patient again complained of worsening bilateral lower extremity weakness. He was treated with a second 3-day course of 1gram IV Methylprednisolone followed by a tapering course of oral Prednisone. The patientвЂ™s symptoms improved after the pulse course of IV steroids but subsequent AST and ALT levels elevated to 177U/L and 235U/L respectively. Total bilirubin level was 11.8mg/dL and direct bilirubin level was 7.8mg/dL. He was admitted back to the hospital for acute hepatitis and cholestatic injury. HIV, RPR and Hepatitis B serology were negative. Abdominal ultrasound showed multiple gallstones, portal hypertension and no evidence of biliary dilatation. CT scan of the abdomen and pelvis showed gallstones in a non-distended gallbladder. MRCP showed multiple gallstones, cirrhotic liver, and no choledocholithiasis. His liver function panel continued to improve during his hospitalization but remained elevated at discharge with an AST of 114U/L, ALT of 78U/L, total bilirubin of 8.4mg/dL and direct bilirubin of 63mg/dL. Two months later during his outpatient visit, the patientвЂ™s liver function panel returned to baseline with an AST of 57U/L, ALT of 81U/L, total bilirubin of 0.9mg/dL, direct bilirubin of 0.2mg/dL. While other causes of liver injury must be excluded, steroid-induced hepatotoxicity, although rare, should always be considered in patients with transaminitis after receiving therapy with high dose IV steroids. Clinical course is highly variable ranging from asymptomatic transaminitis 6 to fulminant hepatic failure with improvement of symptoms by discontinuation of the offending steroid. Dasatinib Induces HUS and Pulmonary Hypertension Ashkan Attaran, MD; Reza Rafie, MD; Siamak Sadaat, MD; Sharmineh Shams, MD University of Southern California Introduction: Pulmonary hypertension has been reported in hemolytic uremic syndrome (HUS). There are several previous case reports about the development of HUS with different chemotherapy medications but there is no previous report about the side effects of dasatinib. In this case report, for the first time we describe the development of pulmonary HTN in the context of HUS in patient treated with datasinib. Clinical presentation: Patient is a 26 yo female with no pmhx, that initially presented with fibrolamellar cancer of liver. Patient was initially treated with nexavar and due to lack of response then was subsequently switched to dasatinib. Two months into treatment with dasatinib patient developed HUS and at that time treatment was stopped. Patient was treated by transfusions. Subsequently patient started to develop SOB on exertion, and eventually SOB at rest. Patient was then admitted, initially suspected of heart failure, vs. PE, both were R/O. On cardiac Echo patientвЂ™s RV pressure was measured to be greater than 60, and Dx of Pulmonary hypertension was made. At that time it was decided that the patient needs to be treated with plasma exchange. Patient received 6 treatment of plasma exchange with no improvement. It was decided that the patient needs to be started on Eculizumab. Discussion: Previous studies showed that HUS can result in pulmonary hypertension. The underlying pathology that predispose to pulmonary vascular disease in these patients has not been well defined. Different mechanisms such as endothelial dysfunction, decreased nitric oxide bioavailability and enhancement of coagulopathy have been suggested. Mitomycin C is an alkylating agent that reported to cause pulmonary hypertension and HUS. In this report we described the patient with underlying fibrolamellar CA of liver that was treated with dasatinib .She developed renal failure along with other signs of HUS that was subsequently complicated with pulmonary HTN. There has been no reported case of dasatinib as a potential cause of HUS, or Pulmonary HTN. LV Non-Compaction in Family with History of Sudden Cardiac Death Ashkan Attaran, MD; Reza Rafie, MD; Amir Tahbaz, MD; Sharmineh Shams, MD; Ali Farvid, MD University of Southern California Left ventricular (LV) non-compaction manifests as layering of myocardium of left ventricle with compacted and non-compacted layers. LV non-compaction is being diagnosed if the maximum ratio of non-compacted to compacted layer is > 2.3/1. We present a patient with palpitations, that had family history of sudden cardiac death. His cardiac MRI showed left ventricular non-compaction. Case: A 42 yo Caucasian male with no PMH requested a cardiac work-up. He gave a family history of sudden cardiac death in three family members at a young age. EKG did not reveal any abnormalities, cardiac Echo was suspicious for LV non-compaction. This was confirmed by Cardiac MRI showing LV non-compaction to compaction ratio of 2.88/1. Discussion: Left ventricular (LV) non-compaction has been described as arrest in the compaction process of fetal myocardium that can manifest in adult age as heart failure, atrial and ventricular arrhythmias and thromboembolic events. Although echo data has been used to diagnose but recent studies showed increased specificity with using cardiac MRI. The patient that we described here had sudden cardiac death in his immediate family that made us suspicious for structural heart disease. Subsequent echo and cardiac MRI were (+) for left ventricular pathology. Cardiac MRI showed the left ventricular non-compaction to compaction ratio 2.5/1 that confirmed our clinical suspicion. LV non-compaction should be more thoroughly evaluated in patients with unexplained heart failure and history of sudden cardiac death in first degree relatives. Normal BNP in Heart Failure Ashkan Attaran, MD; John Fan, MD; Leslie Saxon, MD University of Southern California Introduction: Brain natriuretic peptide (BNP) has diagnostic and prognostic significance in patients with heart failure. BNP has been considered as a sensitive marker for early diagnosis of heart failure. In this case report we describe a patient with advanced heart failure that despite of requirement for cardiac resynchronization therapy always had normal values of BNP. another interesting aspect of this case is that this atient is one of few patients in the country that their heart failure is being followed by a left atrial pressure device. This device transmits the left atrial pressure to a hand held device, and informs the patient if they need to adjust their medications in order to better control their heart failure. Case presentation: Pt is 54 yo female with pmhx sig for dialted cardiomyapathy, that initially presented with SOB, fatigue, DOE. Echocardiogram showed global hypokenesis, and elevated left atrial pressure. Patient symptomatically was classified as NY class II, however this patientвЂ™s serum BNP levels were low normal level. Discussion: Previous studies showed the sensitivity of BNP to diagnose heart failure is 97-99%. Subsequent studies showed normal level of BNP in minority but quite significant number of patients with heart failure. Young 7 women with non ischemic cardiomyopathy constitute the major part of heart failure patients with normal BNP level. The patient that we described here had history of non ischemic cardiomyopathy status biventricular pacemaker placement that serial checking of BNP level showed maximum that was in normal range. Although BNP is very sensitive marker to diagnose heart failure, it never should substitute the clinical judgment. Left atrial pressure device allows the patient to have an active role in mangment of their heart failure, and could eventually cut down on frequency of office visit. This device will transmit left atrial pressure, to a hand hel device, and based on this information the physician will set two sets of therapy. First set is of constant regimen of medical therapy (beta-blockers, ace inhibitors,вЂ¦) and a second set of dynamic therapy. Device will check left atrial pressure, and if the left atrial pressure is elevated then the patient will take a PRN dose of diuretics, or afterload reducing medications. These data are reviewed and the physician can make adjustment as needed. This device could potentially decrease the frequency of office visits as well as decrease the frequency of CHF exacerbation, and could cut down the cost of heart failure management. only evident in V3-V6, however this patient displayed early repolarization in all leads. Discussion: ST segment elevation has broad differential diagnosis such as myocardial infarction, pericarditis, myocarditis, ventricular hypertrophy , hyperkalemia. One of the main causes of ST elevation of J point in young healthy people is normal early repolarization variant. Although early repolarization involves V3-V6 most of the time but it can have a diffuse pattern. One of the methods that can differentiate the acute pericarditis from early repolarization is the ratio of ST elevation to T wave amplitude in lead V6 .If the ratio exceeded 0.24 the diagnosis will be most likely acute pericarditis. The patient that we described here had diffuse ST elevation in all leads That by using the ST/T ratio in lead V6, the diagnosis would be 0.30 . He had drug seeking behavior and used his ECG as a tool to get pain medications. West Nile Viral Myocarditis Ashkan Attaran, MD; Reza Rafie, MD; Sharmineh Shams, MD; John Fan, MD; Leslie Saxon, MD University of Southern California Pericarditis Versus Early Repolarization Ashkan Attaran, MD; Reza Rafie, MD; Sharmineh Shams, MD; Ali Farvid, MD; M. Pashmforoosh, MD Introduction: Early repolarization manifests as J point ST elevation that commonly can be detected in leads V3V6. It is a major ECG diagnostic dilemma. Sometimes it is difficult to differentiate early repolarization from STEMI or pericarditis. We present a patient with diffuse ST elevation that was diagnosed as pericarditis but retrospective inspection of his cardiac echo confirmed the early repolrization. Case presentation: Patient is a 42 yo aam with PMHx sig for reported recurrent pericarditis, and CAD (per patient) presented with sever pluritic chest pain. Patient described the pain as sharp, 10/10 in intensity, without radiation. Patient stated that his chest pain was worse when lying in supine position, and was somewhat alleviated when leaning forward. Patient also stated that he has had hx of NSTEMI, in Minnesota. PatientвЂ™s EKG showed diffuse ST segment elevations in all leads. Patient refused NSAIDs for treatment, and stated that his chest pain only was alleviated by IV Dilaudid. Given the significant CAD hx that the patient was claiming, we contacted the hospital that he was getting care in, in Minnesota. The records from outside hospital showed that the patient was admitted frequently with the same symptoms, and had an extensive work-up including cardiac ECHO, as well as cardiac MRI, which showed no evidence of pericarditis, off interst it was also established that the patient had no history of CAD(established by cardiac catherization). On follow-up exam it was established patient had drug seeking behavior, and that his EKG was representive of a normal variant of early repolariztion. This EKG was interesting because in most cases, early repolarization is Introduction: West Nile virus (WNV infection usually present as self limited febrile illness with fever, HA and myalgia. There are several previous case reports about pathologically diagnosed myocarditis, with all cases leading to patientвЂ™s demise. There is limited data clinically correlated cardiac dysfunction secondary to WNV infection. In this case report we describe a patient with WNV cardiomyopathy that showed cardiac function abnormality. Case: Patient is a 27 yo previously healthy female with no PMH. Presented with flu like symptoms, and then suffered a syncopal episode. Patient was admitted and initial work-up showed normal cardiac function, however her clinical picture worsen over the following two days. Patient started to develop dyspnea on exertion initially, then dyspnea at rest. Patient was placed on telemetry and was noticed to have episodes of v-tach. Echo showed EF15%. At this time patientвЂ™s WNV titers came back high. Based on clinical presentation a diagnosis of viral myocarditis was made patient was started on IV prednisone, as well as for hemodynamic support patient was supplemented with intra-aortic balloon pump, as well as an ECHMO device. Cardiac MRI and cardiac biopsy were also performed. MRI showed pericardial enhancement, as well as delayed enhancement in the subendocardium. Biopsy specimen was then send for immunohistochemical staining, which subsequent result confirmed diagnosis of west Nile myocarditis. PatientвЂ™s condition improved over the course of one week. Repeat cardiac echo showed EF 55-60%, and patientвЂ™s frequency of arrythmias decreased. Patient was subsequently discharged with a life vest, and close follow-up. Discussion: Only 20% of patients with West Nile virus infection become symptomatic. It can also present with 8 severe neurologic manifestations such as meningitis or encephalitis. Rare presentation of this infection can be rhabdomyolysis, hepatitis, and pancreatitis. Myocarditis is one of the unusual presentation for these patients. There are 2 previous case reports about cardiac arrhythmia and myocarditis in patient with West Nile virus infection that in both of these case reports, Patients died of possible fatal arrhythmia. The patient that we described here showed evidence of myocarditis with rhythm abnormalities (episodes of V-tach, and frequent PVCs). As the diagnosis for cases with Wet Nile virus increases, we should pay more attention to unusual but clinically important aspect of this infection such as myocarditis. Future studies may show the necessity of close monitoring of WNV infected patient with any sign or symptoms of cardiac involvement. Aortic Valve Thrombosis Presenting as Non-ST Elevation Myocardial Infarction Emil Avanes, MD; Grace Nam, MD; Kenneth Narahara, MD Harbor-UCLA Medical Center A 73 year old man presented with 9 hours of constant nonexertional, pressure-like chest pain. He also complained of shortness of breath, a decreased exercise tolerance, and 2 pillow orthopnea, but no PND. He denied upper respiratory symptoms/fevers. He had a past h/o diabetes, hypertension, and myocardial infarction 9 years previously with subsequent coronary bypass grafting and aortic valve replacement (AVR). On exam, the patient appeared comfortable with vital signs: T 97, BP 127/58, HR 62, RR 18, oxygen saturation 96% on 2L oxygen. He had normal jugular venous pressure but rales ВЅ up the back bilaterally with muffled aortic closure sound. A grade II/VI systolic murmur that radiated to the carotids was present as was a left-sided S3 gallop. Routine lab values were notable for INR of 1.2 and troponin I of 0.45 (normal <0.4). The EKG demonstrated sinus rhythm, 1В° AV block and nonspecific ST-T changes. An enlarged heart and bilateral pleural effusions were seen on X-ray. A TTE revealed normal RV and LV function with LVH. A mechanical aortic valve was present but the leaflets were not well seen. A 49 mmHg gradient was recorded across the prosthesis. Aortic valve thrombosis from inadequate anticoagulation was suspected. Initially, the patient was treated with aspirin and a heparin infusion. Warfarin was restarted. Over the next 4 days, the patientвЂ™s heart sounds took on a more normal mechanical quality, the S3 and rales disappeared. A repeat TTE revealed essentially normal aortic valve function and a transesophageal echocardiogram (TEE) confirmed the absence of significant thrombus. The patient was discharged with an INR of 3.2. Mechanical heart valves were introduced in the 1950s. Our patient had a St. Jude bileaflet valve, a model in use since 1977. The recommended INR for a St. Jude valve is 2.5-3.5 although one series recently suggested an INR of 2 for an AVR. The AHA/ACC recommends INRs based on risk assessment. High risk patients (h/o atrial fibrillation, left ventricular dysfunction, hypercoagulable state, or history of previous thromboembolism) should maintain INR from 2.5-3.5 whereas those without these risk factors should aim for 2.0-3.0. When prosthetic heart valve thrombosis is diagnosed, there are 3 treatment modalities: surgery, fibrinolysis, or unfractionated heparin. Thrombus size & prior stroke are independent predictors of complications with thrombolytic therapy. Traditionally, primary care physicians & anticoagulation clinics have managed warfarin dosing for patients with prosthetic valves, but self-management methods have the potential for maintaining more accurate and stable INRs. A Benign Tumor and a Great Imitator Kendan Baban, MD; D. Rebenson, MD; M. Kyle, MD Scripps Green Hospital A 61 year-old female admitted to the hospital with one day history of slurred speech and left arm weakness. Her past medical history was unremarkable. Her review of system was significant for twenty pounds weight loss, chills, fatigue, myalgia, arthralgia and bilateral lower extremities painful rash with paresthesias for 3 months. On examination patient was slightly hypertensive, cardiovascular exam revealed loud S1, normal S2 with regular rate and rhythm and no murmurs noted. Her neurological exam was remarkable for left facial droop and 3/5 left arm muscle strength with discordant finger to nose. Examination of her extremities revealed blanching papules on the palms of hands and the sole of foot bilaterally. Initial laboratories revealed no leukocytosis, and normal electrolytes. Previous workup included skin biopsy, cryoglobulin level, UPEP, SPEP, ESR, ANCA panel, ANA titer which were all normal. Brain MRI/MRA on admission revealed multiple foci of restricted diffusion in both hemispheres, and all lobes of the brain as well as both cerebellar but spearing the brainstem. The multiple vascular distributions suggested multiple small infarcts from an embolic source. Based on the finding on the MRI and her constitutional symptoms TTE was performed. Initial TTE showed EF of 72% and a large highly mobile mass plopping through the mitral valve orifice suggestive of left atrial myxoma. A transesophageal echocardiogram confirmed the presence of left atrial myxoma. The patient promptly under went cardiac surgery to prevent further embolization. A 5.2 x 4 x 1.8 cm gelatinous, red brown aggregate of friable locally bloody tissue was resected from the left atrium. Pathology indicated benign myxoma with chronic hemorrhage. Patient tolerated surgery well and underwent extensive physical rehab. At three months follow up the patient was completely recovered from her symptoms of fatigue, rash and weakness. Primary cardiac neoplasms are rare with reported prevalence in autopsy series of 0.0017-0.03%. Approximately 80% of primary cardiac tumors are benign. The most common cardiac neoplasm is myxoma. Patients with cardiac myxoma present with a wide range of clinical signs and symptoms, which in part depend on the location and morphology of the tumor and its tendency to embolize. The most com- 9 mon presenting symptoms being heart failure, but it may also present with constitutional symptoms, lethargy, arthralgias, and petechiae. This case domenstartes a life threatning presentation of histologically benign cardiac tumor. High index of suspicion is fundamental in clinical approach and correct diagnosis of cardiac myxoma because of the potential life-threatening sequelae of this tumor. In addition this tumor can mimic not only every cardiac disease but also, inefective, immounologic and malignant processes. Acute Herpes Hepatitis in Pregnancy symptoms of HSV hepatitis include fever, transaminitis, abdominal pain, encephalopathy, and coagulopathy. Vaginal or oral lesions are present in only 50% and jaundice is uncommon. Definitive diagnosis requires liver biopsy with immunohistochemical staining and PCR techniques. Treatment is high dose intravenous Acyclovir although liver transplantation should be considered for failure of therapy or further clinical deterioration. HSV hepatitis must be considered in febrile pregnant patients with anicteric transaminitis as early detection and initiation of antiviral medication is associated with close to 100% survival. Vidhya Balasubramanian, MD; Sarah Khan, MD; Erica Palys, MD; Suzanne Donnovan, MD, MPH; Stanley Dea, MD; Glenn Mathisen, MD Olive View-UCLA Medical Center PCP Pneumonia and Granulomas? Case: A 17 year old G1P0 female presented at 34 weeks gestation with three days of fevers, right flank pain and uterine contractions. Her medical history was significant for a Chlamydia infection that was treated three months prior to admission. She denied recent travel and had one male sexual partner. Exam revealed a fever to 39.2C and right flank tenderness but no vaginal lesions. Fetal monitoring revealed tachycardia. Ceftriaxone was started for presumed pyelonephritis as urinalysis had one WBC/hpf. On hospital day four, antibiotics were broadened and she underwent emergent cesarean section due to preterm premature rupture of membranes and concern for chorioamnionitis. Despite an uncomplicated delivery of the baby, her fevers persisted so other sources of infection were sought. Serologies and PCR were sent for viral hepatitis, mycobacterium, coxiella, Herpes Simplex Virus (HSV), Cytomegalovirus, EBV, Varicella, Cryptococcus, Coccidiomycosis and HIV. Blood and placental cultures remained negative. Labs on hospital day seven revealed AST 2906 units/L, ALT 675 units/L, Alkaline Phosphatase 184 units/L, Total Bilirubin 0.6 mg/dL, INR 1.91 and Albumin 1.7 mg/dL. CT scan of the abdomen and pelvis showed hepatomegaly and multiple small low attenuation lesions in the liver. Serologies for HSV-1 (IgG 1.34), HSV-2 (IgG >6.0) and HSV 2 PCR were positive. All other tests were negative. Given the concern for fulminant hepatic failure, the patient was transferred to the Intensive Care Unit under the medicine service. On hospital day eleven, the patient underwent liver biopsy which showed patchy necrotizing hepatitis and stains for HSV were weakly positive. Cultures were later positive for HSV-2. The patient was started on high dose intravenous Acyclovir and her abdominal pain, fevers and transaminitis resolved. The baby had a negative evaluation for congenital HSV-2. Introduction: Hypersenstivity pneumonitis (HP) is an inflammatory condition caused by immunological reactions to environmental antigens. This case will review the details of this disease. However, the major learning objective will be for the clinician to remain open to the idea of having co-existing diagnoses. Discussion: Hepatitis is a rare presentation of HSV infection in immunocompetent patients. Fulminant hepatic failure can progress quickly with an untreated mortality rate of 80-90%. Pregnant patients are at increased risk for HSV hepatitis and in the 24 cases reported from 19691999, all occurred during the third trimester. Common Vidhya Balasubramanian, MD; Nader Kamangar, MD, FACP Olive View-UCLA Medical Center Case: A 29 year old male presented after a syncopal episode. On review of systems he endorsed having one month of dry cough, fevers, and anorexia. His social history was significant for tobacco use and unprotected heterosexual intercourse. The patient was born in Guatemala and moved to Idaho four years prior to admission where he worked as an agricultural laborer. He denied any other travel history or animal exposures. Notable findings on physical examination included a temperature of 38.6C , HR of 50 bpm, but no evidence of respiratory distress. Laboratory results showed a WBC of 5,200 cells/uL with lymphopenia. CT scan of the chest revealed diffuse ground glass opacities and extensive reticulonodular and centrilobular infiltrates predominant in the bilateral upper lobes. He was started on Ceftriaxone and Levofloxacin, as well as on treatment for Tuberculosis given a high clinical suspicion. Additional laboratory tests revealed a positive HIV screen with a CD4 count of 1 (0%). Cryptococcal antigen, coccidiomycosis antibody, and urine histoplasmosis antigen were all negative. On hospital day 3, the patient underwent bronchoscopy. The bronchoalveolar lavage (BAL) AFB smears were negative while Pneumocystis jirovecii Pneumonia (PCP) DFA stains were positive. The pathology report on the transbronchial biopsies showed Pneumocystic organisms, however, it also showed non necrotizing granulomas. The patient was started on Bactrim with improvement in symptoms. However, the radiographic findings and pathology report were not consistent with PCP. Given his occupational exposure, the notable centrilobular ground glass nodules seen on CT, and the non-necrotizing granulomas on transbronchial biopsy, a diagnosis of concurrent subacute hypersensitivity pneumonitis was made. 10 Discussion: Diseases characterized by profuse centrilobular nodules includes subacute hypersensitivity pneumonitis, respiratory bronciolitis-interstiial lung disease (RB-ILD), diffuse panbronchiolitis, endobronchial spread of tuberculosis, and cryptogeneic organizing pneumonia. The only one of these entities also associated with noncaseating granulomas is HP. HP encompasses more specific diseases such as bird fancierвЂ™lung and farmerвЂ™s lung. This patient likely had farmerвЂ™s lung, where aspergillus has been identified as the major antigen. Clinically these patients present with acute onset of cough, fevers, and dyspnea after exposure to the offending agent. Characteristic CT findings in the subacute stage are discussed above while chronic disease causes fibrotic changes. Diagnosis is based on a combination of clinical history with known environmental exposure, radiographic findings, and biopsies showing non-caseating granulomas. Treatment includes removal of the offending antigen and glucocorticoids for those with persistent symptoms. The majority of patients recover and have no functional limitations. Transfusion Related Lung Injury and Cardiac Stent Thrombosis Raj Balasundaram, MD; Sarah Elsayed, MD University of Southern California This case identifies a blood transfusion causing both TRALI (transfusion related lung injury) and cardiac stent thrombosis in 3 RCA stents. A 64 year old male with a past medical history of COPD presented with the chief complaint of left sided chest pressure, 10/10, radiating to his back and with nausea/vomiting, shortness of breath, and diaphoresis. The patient was diagnosed with an STEMI and taken for cardiac catheterization. He was had 3 vessel disease including total occlusion of the right coronary artery. Balloon angioplasty of the RCA was performed. After the cardiac catheterization the patient was started on Aspirin, Plavix, and Integrellin. He developed AV block with bradycardia and was subsequently started on a dopamine drip. The patient then developed hemoptysis and was intubated for airway protection. He was weaned off pressors and extubated, however, on hospital day #5 the patient again developed AV block and ST elevation in the inferior leads. Three bare mental stents were then placed in the RCA. The patient remained stable until hospital day #12 when he was transfused four units of packed red blood cells for a progressive decrease in Hb from 14.0 to 7.8. Four hours after the transfusion the patient developed tachypnea, oxygen saturation decreased to 75% and he was reintubated. EKG again showed inferior ST elevation. Trans-thoracic echo showed an EF of 65%. The wedge pressure obtained from the Swan Ganz catheter was 13. CXR showed bilateral patchy infiltrates, right > left, consistent with ARDS secondary to TRALI. Cardiac catheterization revealed total RCA occlusion, including thromboses in all prior RCA bare metal stents. The patient survived after wire passage and additional stent placement to the RCA. TRALI is defined as acute lung injury caused by a transfusion of blood products occurring within six hours after a transfusion. Known causes of TRALI include transfusions of platelets, PRBCвЂ™s, whole blood, and stem cells. It is the leading cause of transfusion related mortality in the United States. Acute or subacute thrombosis after coronary dilatation is a dramatic event with high mortality. Three cases by Cornet have described patients who had been treated with coronary stents and anti platelet therapy. Patients subsequently suffered GI bleeds requiring platelet transfusions causing stent thrombosis. 2/3 patients died and have no angiographic evidence of thrombosis. One patient survived after wire passage and placement of another stent. This case represents a transfusion of packed red blood cells causing both TRALI and stent thrombosis, raising a new risk for the transfusion of packed red blood cells following cardiac catheterization. Diffuse Pulmonary Hemorrhage in an Elderly Patient: An Uncommon Presentation of Wegener's Granulomatosis Linda Barkodar, MD; Meika Fang, MD Cedars-Sinai/ Greater Los Angeles VA Healthcare System Wegener's Granulomatosis (WG) is a disease with a characteristic necrotizing granulomatous vasculitis of small and medium sized blood vessels, which principally affects the kidneys and the upper and lower respiratory tracts. It is an uncommon and diagnostically challenging disorder that is usually revealed through a combination of clinical criteria, serum cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA) and histology. WG usually presents by the sixth decade, and the typical lesions in the lung consist of necrotizing granulomatous inflammation associated with a necrotizing vasculitis. We describe an unusual case of WG in which massive pulmonary hemorrhage was the initial manifestation in an 87 year old male. The late age of presentation along with isolated massive pulmonary hemorrhage without necrotizing nodules is rare and presents a difficult diagnostic challenge for the clinician in the acute care setting. The diagnostic criteria as well as management of this disease will be reviewed here as well. Hey Doc, Why Are My Eyes Yellow? Robert Basseri, MD; Benjamin Kianmahd, BA Dept. of Internal Medicine, Cedars-Sinai Medical Center/Greater Los Angeles VA The patient is a 30-year-old female with a history of depression, who presented to Cedars Sinai Medical Center after being told by a co-worker, that вЂњher eyes were yellow.вЂќ The patient stated that she had been feeling well until approximately one month prior to presentation when she noticed the onset of decreased appetite and increased fatigue. She herself did not note any yellowing of her eyes or skin. She notes that she drinks alcohol on a social basis, which includes 5 to 6 alcoholic beverages per week. She denies associated abdominal pain, abdominal 11 fullness, nausea, vomiting, fever, chills, or sick contacts. She denies a change in bowel habits. She denies bright red blood per rectum, hematochezia or melena. She denies any prior notice of intermittent jaundice of the skin or scleral icterus. She denies a known history of liver disease or history of liver disease within her family. She denies heavy use of Tylenol. The patient works as a bookkeeper, has a 10 pack year history smoking although she has been tobacco free for over 5 years, drinks five to six alcoholic beverages per week and denies intravenous drug use. She has a family history of brain cancer and alcoholism. The patient was afebrile and her vitals were within normal limits. In general she was an ill-appearing, cachectic, and jaundice female appearing older than stated age. She had obvious scleral icterus. Pertinent abdominal exam findings include hepatomegaly, ascites and presence of a fluid wave. Furthermore, mild pitting edema bilaterally with right greater than left to the level of the ankles. Slit lamp examination demonstrated an early KayserFleischer ring in the patientвЂ™s right eye. Pertinent workup laboratory values include mildly elevated liver function tests and total bilirubin, and low total protein, as well as elevated CA 125 (663), CA 19-9 (113), urinary copper and low serum ceruplasmin. Of note, CEA, AFP, alpha-1 antitrypsin, smooth muscle antibody, EBV, hepatitis B surface antigen, mitochrondial antibody, and anti-nuclear antibody were all negative on admission. CBC demonstrated a mild anemia and macrocytosis, however peripheral smear did not demonstrate polychromasia or signs of hemolysis. Ultrasound imaging demonstrated hepatomegaly, a heterogeneous appearance consistent with fatty infiltration of the liver as well as splenomegaly and ascites. Liver biopsy was performed and ultimately diagnosed WilsonвЂ™s disease. Discussion: WilsonвЂ™s disease is an autosomal recessive genetic disorder where excessive copper accumulates in the liver and brain, causing liver failure and neuropsychiatric conditions. This case highlights the importance of a complete history in diagnosing a rare condition (incidence 1/100,000 patients), with textbook presentation. Recognition of new onset neuropsychiatric symptoms in the context of acute liver failure and the discovery of kayser flescher rings are key in the diagnosis of WilsonвЂ™s disease. Diagnosis is facilitated with serum ceruloplasmin, urinary copper excretion, and ultimately liver biopsy with special copper stains. Hepatosplenic T-Cell Lymphoma in a Young Man with CrohnвЂ™s Disease Jennifer Bennitt, MD; Hillary Bownik, MD University of California, Los Angeles Hepatosplenic T-cell lymphoma (HSTCL) is a rare form of non-HodgkinвЂ™s lymphoma and comprises only 5% of peripheral T-cell lymphomas. While the majority of cases develop de novo, approximately one-third of cases have been associated with organ transplantation or other conditions that require immune-modifying agents. We present a case of HSTCL in a 22 year-old male with CrohnвЂ™s disease previously treated with infliximab and 6mercaptopurine. Two years after his last course of infliximab, he developed fevers and jaundice, with associated hepatomegaly and marked splenomegaly, and acute leukemia, which are typical features of HSTCL. The diagnosis was confirmed with flow cytometry and bone marrow biopsy. Because it is so rare, recognizing the intersection between the clinical features and those patients at risk of developing HSTCL is paramount to making the diagnosis. The clinical features, treatment options, and prognosis of HSTCL will be discussed. In addition the evidence suggesting an association between HSTCL and inflammatory bowel disease is reviewed. Neurofibromatosis Presenting as SVC Syndrome Antonio Bonet, MD; Shefali Samant, MD; Mallory Witt, MD; Fernando Antelo, MD Harbor-UCLA Medical Center Case: A 39 year-old Caucasian female presented to the emergency department complaining of right neck pain and swelling of one dayвЂ™s duration. Her symptoms were preceded by a sensation of fullness and soreness in the superior right chest. She did not use tobacco, and denied shortness of breath, cough, hemoptysis, dysphagia, changes in her voice, fevers, chills, or weight loss. On physical examination, multiple cafГ© au lait spots were noted on her back, chest and arms, with axillary freckling and fleshy nodules. Prominent right-sided neck veins were noted, along with right sided supraclavicular swelling, facial fullness, and venous dilatation over the chest wall, consistent with superior vena cava syndrome No underlying neck mass was palpable. Auscultation of the lungs revealed decreased breath sounds in the right upper lung field. Upon further questioning, the patient reported that she and her son had both been given a diagnosis of neurofibromatosis by previous physicians. Computed tomography (CT) imaging of the chest revealed a 9 cm round, heterogeneous mass in the right upper lobe. The patient underwent CT-guided biopsy of the lung mass, and examination of the tissue was consistent with neurofibroma, with positive S-100 stain and a high mitotic index. Discussion: This patient presented with features of superior vena cava syndrome (SVC syndrome) secondary to neurofibromatosis. Neurofibromatosis type 1 is an autosomal dominant disease that manifests as cutaneous cafГ© au lait spots and neurofibromas, with thoracic rib and spine deformities that result from plexiform neurofibromas. Thoracic neurofibromas most commonly occur in paravertebral and mediastinal locations, or adjacent to ribs. Approximately twenty percent of neurofibromatosis type 1 can be associated with diffuse lung disease, especially upper lobe cystic disease and lower lobe fibrosis, as well as solid pulmonary masses. Approximately 1-25% of 12 all neurofibromas have the potential for malignant degeneration into neurofibrosarcoma; therefore, treatment of these masses tends to be surgical resection, with subsequent chemotherapy and/or radiation when malignancy is diagnosed. To our knowledge, only two other cases of neurofibromatosis leading to superior vena cava syndrome have been reported. Recommended treatment in this case was surgical resection. Abdominal Pain: Expanding the Differential Diagnosis Hilary Bownik, MD; Nasim Afsar-Manesh, MD; Benjamin Waterman, BA University of California, Los Angeles pregnancy, obesity, and coagulation disorders (1,3,6,4). Inciting factors include: straining, coughing, exercising, prophylactic subcutaneous heparin injections, or trauma. Measures should be taken to prevent the occurrence of RSH as its mortality in patients on anticoagulation reaches 25% (6,12). Patients with cough or on ventilation should have prophylactic heparin injections only twice daily and administration to the thigh (4). Treatment for RSH is usually conservative. Surgical evacuation and hemostasis is necessary if the hematoma fails to resolve, if hemostasis cannot be achieved, or if a secondary infection develops (6,7). Failure to recognize RSH leads to abdominal compartment syndrome, hemodynamic instability, and multi-organ dysfunction. Diabetic Myonecrosis: A Case of Less is More Learning Objectives: 1) Rectal Sheath Hematomas (RSH) should be included in the differential diagnosis for acute abdominal pain in all patients on systemic or subcutaneous anticoagulation. 2) Clinicians must maintain a high suspicion for RSH as only 50% of patients will have a visible hematoma at the time of presentation. 3) Early diagnosis and appropriate treatment is necessary to prevent complications including hemodynamic instability, abdominal compartment syndrome, and even multiorgan dysfunction. Case Information: We present a case of acute right lower abdominal pain in an obese 45 year old female with past medical history of hypertension, anti-phospholipid syndrome, and end stage renal disease, who was admitted for development of both a dvt and SMA clot despite lovenox therapy. Four days after conversion from subcutaneous to IV heparin drip anticoagulation, the patient complained of RLQ pain. She had a small 2x3 cm hematoma in her RLQ from previous lovenox and insulin injections, no rebound or guarding. Vital signs were stable and hemoglobin unchanged. An abdominal series was negative for obstruction or free air. That evening, she developed significantly increased RLQ pain and an expanded RLQ mass of approximately 20 x10 cm. She was tachycardic to the 150s. Abdominal CT showed a 9.7 x 16.1 cm anterior rectal sheath hematoma. Her repeat hemoglobin was now 5.5. The patient underwent CV angiogram with embolization of the right inferior epigastric artery and required surgical evacuation of the hematoma. Implications/Discussion: RSH are a rare cause of abdominal pain. They are an uncommon complication of systemic or subcutaneous anticoagulation, usually developing 4 to 14 days after its initiation (6). Symptoms are non-specific and include: abdominal pain, palpable mass, nausea, vomiting, fever, and leukocytosis (3,8). The Fothergill sign is diagnostic (3,6). This sign is positive if the palpated mass, which shows lateral mobility, becomes fixed when a patient contracts the rectus muscle (6, 9,10). Only 50% of patients will have a visible hematoma at the time of presentation (7). Diagnosis is confirmed by Abdominal CT. Medical conditions predisposing patients for RSH development include: hypothyroidism, abdominal surgery, ascites, hypertension, collagen vascular disease, Hilary Bownik, MD; Jennifer Bennitt, MD; Benjamin Waterman, BA University of California, Los Angeles Introduction: Diabetic Myonecrosis is a rare complication of diabetes that often goes unrecognized, leading to delayed diagnosis, unnecessary procedures, and hospital acquired infections. Case Description: A 46 year old Hispanic female with poorly controlled type 2 diabetes for 12 years, obesity, peripheral neuropathy, and retinopathy presents with a 3 month history of bilateral lower extremity edema. For 2 weeks, she had experienced extreme left leg pain and worsening of her edema to the point that she could no longer ambulate. At a local ER, a MRI showed diffuse quadricep edema consistent with cellulitis, and the patient was started on multiple broad spectrum antibiotics, with no improvement of symptoms. Given extreme pain out of proportion to exam, there was concern for necrotizing fascitis. The patient refused all recommended surgical interventions or wound debridement except for a small 2.5 cm left medial thigh incision. 5 Days after a PICC line was placed, she became febrile and hypotensive. She was found to have candida glabrata fungemia and pulmonary infiltrates. She refused bilateral lower leg amputation and was transferred for a higher level of care. Upon arrival, she had extremely painful pitting 2+ edema up to the groin bilaterally, 2 oozing right inner thigh lesions measuring 2-3 cm, and one left thigh non healing surgical incision. MRI of legs revealed profound muscle edema along the left vastus intermedius muscles and lateral aspect of the vastus medialis, findings consistent with diabetic myonecrosis. Discussion: Diabetic Myonecrosis is a rare complication of diabetes with less than 50 case reports in the literature. Often initially unrecognized, it can lead to delayed diagnosis, unnecessary procedures, hospital acquired infections, and patient suffering. It occurs more often in insulin dependent, poorly controlled, longstanding (>15 years), and type 1 diabetics. The most common muscles involved are the quadriceps (62%), hip adductors (13%), hamstrings (2%), or hip flexors (2%). Patients present 13 with acute onset of painful swelling, normal pulses, no strength nor sensation abnormalities, and no history of trauma. They are normally afebrile, non toxic appearing, and have extreme tenderness to palpation. One third of patients will have bilateral physical findings. Only 8 % will have leukocyotosis. Muscle biopsy shows muscle necrosis and edema. MRI with gadolinium is the gold standard for diagnosis, which shows extensive edema of the vastus lateralis, vastus medialis, and occasionally the rectus femoris muscle. Treatment is immobilization, pain control, and tight glucose control (100-115). With tight glucose control and aspirin, most patients have spontaneous resolution in a few weeks (average 5). Surgical incision is contraindicated because of poor wound healing and risk of superinfection (average healing >13 weeks). Overall 5-year mortality rate after initial diagnosis is high owing to severe underlying diabetic complications. left breast mass resulted in the diagnosis of infiltrating ductal carcinoma that was estrogen receptor positive, progesterone receptor negative, Her-2/neu negative, and BCL-2 positive. Patient was treated with radiation therapy, high dose steroids, bisphosphonates, and subsequent chemotherapy. From Cat Scratch to Can't Walk Acute, Reversible Cardiomyopathy in a Patient with Group A Streptococcal Toxic Shock Syndrome Leticia Campbell, MSIII; Vivek Ramanathan, MD; Enma Alvarado, MD; Sassan Farjami, MD; Jasminka Criley, MD Charles R. Drew University of Medicine and Health Sciences David Geffen School of Medicine at UCLA Case: A 52 year old woman presents to the hospital with a complaint of lower extremity weakness and difficulty ambulating. The patient was bed ridden for 3 months, after experiencing a series of falls. The patientвЂ™s history includes a work injury, which resulted in an L5 disk herniation, otherwise she has been healthy. Two months prior she presented to an outside hospital with a lesion above her left breast extending to the left axilla diagnosed as cellulitis. She believes that sitting for 6 hours in a wheelchair at that time lead to the difficulty walking. She was adamant that the lesion started as a bump 5 years ago after her cat pounce off her left breast. It erupted 1.5 years ago into a fungating lesion, which she believed to be healing until she became bed ridden. The patient has experienced a 30lbs weight loss in the last 2 months. Her mother has been in remission for breast cancer for 12 years; her aunt also had breast cancer. Physical examination revealed decreased sensation in her lower extremities bilaterally in the L3-S1 dermatomes and left upper extremity in the 3rd and 4th digits to pin prick. Strength: 5/5 upper extremities and left hip flexion and knee extension, 3/5 right lower extremity. Babinski sign was positive bilaterally. There was no pronator drift. Breast exam: Left breast 21x22 cm firm mass in the 12 oвЂ™clock position with overlying fungation lesion extending to the axilla and total breast retraction. Cervical CT scan showed extensive destructive lesions involving C6 and the posterior region of C5 with extension into the spinal canal and involvement of the skull and clavis; CT chest/ab/pelvis revealed a large left breast mass with axillary lymphadenopathy, right liver lesions, and lytic lesions at L5 extending to the thecal sac, left sacroiliac joint, right ischium, ilium, and acetabulum. Labs on admission were significant for increased WBC 14.5, platelets 576, alkaline phosphatase 414, and AST 44. A core biopsy of the Discussion: This case illustrated the importance recognizing signs and symptoms of metastatic disease as the initial presentation of underlying malignancy, and emphasizes the importance of health education and access to screening. Breast cancer is the most common cancer and the second most common cause of cancer death in U.S. women; most of which are infiltrating ductal carcinoma. There is a 3-4 time increase risk if it has affected a primary relative. When detected early, breast cancer has a increased survivorship and lower morbidity rates. Steven Carlson, MD; Barbara Natterson, MD; Daniel Uslan, MD University of California, Los Angeles Introduction: Toxic Shock Syndrome (TSS) is a rare complication of Group A Streptococcal infections. Little is known on the time course and prognosis specifically in relation to the cardiac manifestations of this disease. Case: A 26 year old white male presented with a 4 day history of sore throat, fevers and myalgias. On examination, vital signs: 39.4В° C, HR125, BP 100/56. He was initially saturating 100% on room air but over the next 24 hours became tachypneic requiring increasing amounts of oxygen. He was toxic appearing. Exudates were noted on his tonsils bilaterally. There was cervical lymphadenopathy with tenderness to palpation. Neck was supple without meningismus. Lung exam was notable for bibasilar crackles and wheezing. A diffuse maculopapular rash was noted on the patientвЂ™s trunk, lower and upper extremities as well as on the palms and soles, however the face was spared. Lab studies disclosed the following significant values: WBC of 6.9 with a left shift. CSF analysis including WBC and cultures were negative. On the day of admission the student health center, where the patient had initially been seen 4 days prior called to inform the admitting team that a throat culture from 4 days prior was growing Group A Streptococcus. Approximately 24 hours after admission the patient acutely decompensated becoming hypotensive and requiring mechanical ventilation. He was transferred to the Medical Intensive Care Unit (MICU) and started on broad spectrum antibiotics including Meropenem and Clindamycin for presumed Streptococcal toxic shock syndrome as well as initiated on pressor support with Levophed. IVIG infusion was also started at that time. 14 Hemodynamic monitoring via Swan Ganz catheter was implemented and revealed a cardiac output of 3L/min corresponding to a cardiac index (CI) of 1.6 for this patient. CVP was 21. Echocardiogram was obtained on the day of ICU transfer which revealed severe biventricular dysfunction with a quantitated left ventricular ejection fraction of 18.5%. He was started on dopamine in addition to levophed for treatment of cardiogenic shock. Over the next 3 days the patient was weened completely off of pressor support. Repeat echocardiogram on Day 8 after admission revealed EF of 50%. On Day 16 patient was walked himself out of the hospital Discussion: This case demonstrates the unique cardiac manifestations of Streptococcal TSS. The hemodynamic pattern seen in these cases is of cardiogenic shock and not of septic shock, in that cardiac output is decreased and systemic vascular resistance is not significantly decreased. The likely mechanism of cardiogenic shock seen in these patients is toxin mediated, thus administration of IVIG and anti-toxin antibiotics such as clindamycin are useful. Prompt detection of Streptococcal TSS and administration of the appropriate supportive and therapeutic measure can lead to a full reversal of even severe cardiomyopathy. A 29-Year-Old Man with Paraneoplastic Angioedema Randall Chan, MD; Salima Thobani, MD, Peter Hyunh, MD University of Southern California A 29-year-old Hispanic man with no past medical history presented with a chief complaint of swelling of his lips for six months. Over the course of the 6 months, his lips became progressively swollen and his eyelids too became swollen over 2 months. He had been treated by multiple physicians with trials of antihistamines, oral corticosteroids as well as acyclovir. Physical exam revealed marked swelling of his upper and lower lips with pink discoloration consistent with chronic vascular congestion, as well as edematous eyebrows bilaterally that were also pink with vascular congestion. He had a faint violaceous rash to be nasal bridge, cheeks and forehead without sparing of his nasolabial folds. Oral examination revealed thrush on his tongue and ulcerations of his gingiva. Chest auscultation demonstrated crackels throughout and abdominal exam revealed a mild splenomegaly. Petechaie were scattered diffusely. The remainder of his exam was normal. A complete blood count revealed a profound thrombocytopenia of 8,000/cumm, as well as an absolute lymphopenia. Lactate dehydrogenase was markedly elevated at 1204 units per liter. Chest x-ray revealed an infiltrative lung disease and CT scan revealed multiple pulmonary nodulesвЂ”the largest of which was nearly 3 centimeters in sizeвЂ”as well as splenomegaly. A workup for chronic angioedema was performed. TSH and HIV were noted to be negative. C1-inhibitor levels were normal, as well as a C1-inhibitor functional assay. Additionally, C2 and C4 serum levels were normal as well. An autoimmune workup was unrevealing. Serum protein electrophoresis did not reveal a monoclonal paraproteinemia. Given the above findings, lymphoma was suspected and the patient underwent a bone marrow biopsy, which was unrevealing. However, prior to diagnosis being made, the patient rapidly developed a large effusion with necrotizing pneumonia and septic shock. Cytology from fluid confirmed malignancy, however patient expired prior to receiving chemotherapy. Paraneoplastic angioedema is a rare but known complication of B-cell lymphoma and lymphoproliferative disorders, due to the production of an antibody against C1-inhibitor. This can be detected by decreased levels and function of C1-inhibitor, as well as decreased levels of C2 and C4 which are consumed in the classical complement pathway. Additionally, a monoclonal paraprotein may be detected. Cases of paraneoplastic angioedema associated with lymphoma but with normal levels and function of C1-inhibitor have been described, but are far rarer. Nonetheless, normal complement workup does not exclude the possibility of paraneoplastic angioedema, and it is useful to continue to consider the possibility of malignancy in this setting. The Worm That Got Away: Can Hookworm Infection Cause Frank Hematemesis? Beverly Chen MD; Tung Nguyen MD University of Southern California There are more than 700 million persons in tropical and subtropical regions infected with hookworms. Most patients have mild, nonspecific symptoms and mild degrees of anemia. There have been pediatric cases that presented with occult gastrointestinal bleeding and adult cases that presented with melena; however, to date, there have been no cases of hookworm infestation in an adult presenting as overt acute hematemesis. A thirty-one year old Hispanic male, with a history of alcohol abuse, presented with hematemesis and bright red blood per rectum. His hemoglobin was 7.2 and he was intubated for airway protection. Emergency EGD revealed small amount of blood in the fundus which was suctioned but normal esophagus with no varices, and no evidence of esophageal or gastric ulcer. A large fungating mass was found in the duodenum with visible purple lesions near the ampulla but there was no evidence of active bleeding. The GI bleed was attributed to the fungating periampullary mass. The patient was placed on intravenous esomprazole and transfused a total of six units of packed red blood cells and two units of plasma, with repeat hemoglobin stabilizing at 11. Computed tomography was notable for thickening of the first and second portions of the duodenum, but no apparent mass. On repeat endoscopy a week later, no masses or lesions were seen up to the ampulla. Five centimeters distal to ampulla, an area of thickened mucosa with slight erythema was noted. When the endoscope was advanced into this area and biopsies were being obtained, a worm arose from the abnormal mucosa. Subsequently, three 15 additional worms were seen, two of which were removed with biopsy forceps. Infectious disease was consulted and the patient was started on albendazole on suspicion of hookworm infection. Duodenal biopsy was notable for mild focal increase in eosinophilic infiltrate. However, no parasites were identified on biopsy of the duodenum. The patientвЂ™s hospital course was complicated by aspiration pneumonia for which he was started on antibiotics but discharged home in stable condition with no further episodes of bleeding. While biopsy results did not confirm the presence of hookworm infection, stool studies were notable for hookworm ova. The abnormal pale mucosa and eosinophilia in the duodenum noted on biopsy may have been secondary to chronic changes from hookworm infestation. Although laboratory, radiographic, and pathologic work-up negative did not reveal a clear etiology to this patientвЂ™s hematemesis, there is a distinct possibility that his presentation may have been secondary to a hookworm infection, although this cannot be definitively proven. A Case of Acute Interstitial Nephritis Presenting with Markedly Elevated Erythrocyte Sedimentation Rate Eric Cheung, MD; H. Madkour, MD University of Southern California Acute interstitial nephritis (AIN) is a form of nephritis most commonly induced by drug therapy. Additional causes include autoimmune disorders, as well as infectious etiologies. Although a definitive diagnosis can only be made via kidney biopsy, most cases are diagnosed and managed through history and laboratory findings. Classically, cases of drug-induced AIN present with a triad of rash, fever, and eosinophilia, though recent studies have demonstrated that the presence of all three findings is less common previously thought. A 51 year old female who was recently discharged for atypical chest pain presented with a chief complaint of bilateral lower extremity swelling, fevers, and fatigue. On review of her medical records, she had recently been started on several medications, including omeprazole, furosemide, and labetolol. She denied any chest pain, rashes, dizziness, or HA. Initial physical examination revealed an obese female with significant pitting edema in bilateral lower extremities. Her lung exam revealed scattered wheezes with crackles at bilateral bases. The remainder of the exam was unremarkable. On admission, the patient had an elevated serum creatinine of 2.89mg/dL, compared with her creatinine of 1.1mg/dL one week prior. A urinalysis showed trace protein, trace blood, moderate leukocytes, specific gravity of 1.012, and a pH of 6.5. Given her complaint of fevers at home, a CRP and ESR were checked, which were elevated at 102.9mg/L and 116mm/hr, respectively. Urine and blood cultures were negative. A repeat urinalysis with WrightвЂ™s stain was obtained the day following admission, and was positive for 1-2 eosinophils per highpowered field. A renal ultrasound showed normal sized kidneys without evidence of obstruction. Given the patientвЂ™s recent medical history and acute onset of renal failure, the decision was made to discontinue her omeprazole secondary to concerns for AIN. Her serum creatinine subsequently dropped to 2.5-2.7mg/dL over the following 4 days, but without noticeable improvement in her lower extremity edema. Corticosteroid therapy was initiated on day 5 of her hospitalization secondary to lack of improvement in her renal function. Over the following 5 days, the patientвЂ™s serum creatinine decreased from 2.5mg/dL to 1.87mg/dL, with notable diuresis and decreased swelling in her lower extremities. There has been extensive literature reporting the association of proton-pump inhibitors and acute interstitial nephritis. Though a majority of patients demonstrate significant improvement in their renal function after withdrawal of the proton-pump inhibitor, certain subsets of patients continue to have chronic renal insufficiency despite prompt intervention. Elevated ESRs have been noted in several studies, but rarely to the degree this patient exhibited. Recognition of the variety of abnormal laboratory findings associated with AIN is crucial for early diagnosis, given that significant improvement has been demonstrated with prompt intervention. Angina as a Rare Manifestation of a Rare Disease Raj Chudasama, MD; Stanley Dea, MD Olive View-UCLA Medical Center Case: A 60 year old Armenian male with a history of hypertension, dyslipidemia, and coronary artery disease presented with chest pain and shortness of breath for three days. He has a history of two coronary stents placed three years ago. In addition, he reported bowel movements that were darker than usual for the past day but denied hematemesis or bright red blood per rectum. He noted a ten pound unintentional weight loss over the past 10 days. There were no other significant GI symptoms. He denied use of tobacco, alcohol or illicit drugs. Medications included aspirin 325mg and meloxicam 7.5mg daily as well as ibuprofen 400mg which he took often for pain. Physical exam was unremarkable. Significant lab data included Hgb 6.5, MCV 69, Iron 13 and TIBC 428 (iron saturation=3%). After being cleared of acute cardiac disease, the patient was scheduled for esophagogastroduodenoscopy (EGD) the following day to evaluate for upper GI bleeding with suspicion of NSAID induced ulcers. Although, the EGD showed three small clean-based antral ulcers, the patientвЂ™s melena and anemia was thought to be from a large ulcerated mass in the first and second portion of the duodenum. Multiple biopsies were taken which later confirmed a diagnosis of moderately differentiated, invasive duodenal adenocarcinoma. Based 16 on the location, this was felt not to originate from the ampulla. Discussion: Primary duodenal adenocarcinoma is a rare malignant neoplasm of the small bowel, representing 0.5% of all gastrointestinal (GI) tract cancers. Although duodenal cancers make up half of all cancers found in the small bowel, the majority of these cancers are in the periampullary region. Thus duodenal adenocarcinoma originating from outside this area is relatively rare. A possible reason for this predilection to the ampullary region is interactions of carcinogens with pancreaticobiliary secretions. The clinical presentation of duodenal adenocarcinoma may be vague, or even asymptomatic, leading to late diagnosis with advanced stage at the time of diagnosis. When present, the most common initial symptoms include abdominal pain and/or obstructive symptoms. Ulceration of the mucosa is common and may result in occult GI bleeding or chronic anemia. However, in this unusual case, the anemia was so profound it caused angina in a patient with underlying cardiac disease. All patients able to tolerate surgery should be given the option of aggressive resection regardless of tumor size, invasion or appearance of positive lymph nodes. Pancreaticoduodenectomy is usually necessary if the tumor is localized to the proximal portion of the duodenum. Unresectable tumors should be considered for treatment by radiotherapy. Many patients with advanced cancer of the duodenum will become obstructed and therefore require palliative GI bypass or endoscopically placed stents. Fabry Disease Masquerading as Hypertrophic Cardiomyopathy David Cohen, MD; Stephen Hu, MD; Nestor Mercado, MD; Allen Johnson, MD Dept. of Internal Medicine/Cardiovascular Disease Scripps Green Hospital The patient is a 52 year old gentleman who initially presented for cardiovascular consultation on May 22, 2007 in regards to two previous symptomatic episodes of rapid atrial fibrillation. The patient sought medical attention with his second episode in August 2006 and was chemically converted. His resting electrocardiogram during that hospitalization showed dramatic T-wave inversions in the lateral precordial leads with small Q-waves and prominent R waves in V1 and V2. Subsequent echocardiography revealed a hypertrophic cardiomyopathy, with apical cavity obliteration and no outflow tract obstruction or valvular lesions. He was discharged on Norpace CR and atenolol. At the time of his initial consultation in May of 2007 he was without recurrence of arrhythmia, but was struggling with intermittent headaches associated with lightheadedness. Norpace was held as a suspected culprit for possible symptoms of orthostasis. A repeat echocardiogram showed moderate concentric left ventricular hypertrophy with an echogenic myocardial texture consistent with an infiltrative myocardial disorder. A serum protein electrophoresis showed no monoclonal proteins. In the meantime, the patient continued to struggle with headaches off Norpace, now constant. MRI of the brain showed no abnormalities. The patientвЂ™s family history was ultimately most revealing as his brother was diagnosed with Fabry disease as part of a work-up for renal insufficiency. This would provide a rare yet unifying diagnosis in this patient with symmetic hypertrophic cardiomyopathy in the setting of neurological symptoms. Repeat echocardiogram with optimal views revealed the presence of a binary appearance of the left ventricular endocardial border, with a hyperechogenic layer along the ventricular endocardium paralleled by hypoechogenic myocardium. Serum alphagalactosidase A levels were then assessed and found to be low with an increased ceramide trihexoside in his urinary sediment. These findings were consistent with Fabry disease. Although uncommon, atypical Fabry disease can present with left ventricular hypertrophy and no other obvious disease manifestations. Our patient had symmetric hypertrophic cardiomyopathy without the neuropathy, skin lesions, or renal involvement typical of Fabry disease. His echocardiogram elucidated the binary appearance of the LV endocardial border seen in patients with Fabry cardiomyopathy, reflecting the endocardial and subendocardial compartmentalization of glycosphingolipid material. This case demonstrates the importance of screening for Fabry disease in patients with hypertrophic cardiomyopathy, particularly if symmetric, given the availability of effective therapy. Metastasis or New Primary? Dana Copeland, MD Scripps Green Hospital Introduction: Biopsy is not considered necessary in patients who are likely to have a brain metastasis, yet alternative diagnoses are more frequent than one might suspect. This may lead to inappropriate treatment and its associated morbidity, particularly with chemotherapy. Case Description: A 63 year-old female with a history of stage I ductal cell carcinoma (T1N0M0) diagnosed in1998 who was status post lumpectomy, radiation and chemotherapy presented with nausea and vomiting up to ten times per day. She also complained of a dull frontal headache which was 4/10, constant and non-radiating. For the past 6 months she had noted intermittent nausea, photosensitivity and a five pound weight loss. Two months prior, she also began experiencing diplopia, dizziness and word-finding difficulties. In addition, she had subjective fevers for five days prior to admission. Her cancer screening was up to date, although her last mammogram 1 month prior was BI-RADS 2. Her family history was significant for a mother and sister with breast cancer 70 and early 40s, respectively. She was otherwise healthy and had completed 5 years of hormonal therapy 8 months prior. On neurologic exam, the only finding was a left sided resting tremor. A non-contrast CT of the head 17 showed extensive left frontal edema with mass effect. A subsequent MRI showed an isolated neoplasm in the left mesiofrontal lobe with cystic and hemorrhagic features. A biopsy confirmed glioblastoma multiforme, WHO Grade IV. Discussion: Brain metastases occur in 20-40% of known cancer patients, of which lung and breast cancers are the most likely to metastasize. Brain metastases outnumber primary neoplasms by at least 10 to 1. Therefore, a brain tumor in a patient with known history of breast cancer is much more likely to be metastasis than new primary. Biopsy is not considered necessary in patients who are likely to have a metastasis; however, in cancer patients with brain tumors, 11% were found to have a new primary lesion, inflammatory process, or infection rather than metastasis. This case represents the importance of a biopsy before beginning treatment for brain malignancies even when a patient has known history of breast, lung, or other cancers that commonly metastasize to the brain. Our clinical suspicion for breast metastasis in this patient was low considering 70% of metastases have multiple lesions, and she had only a single lesion. A biopsy proved to be helpful since the treatment would be different in each scenario. A 17 Year Old Female Presenting with Multi-Vessel ST Elevated Myocardial Infarction from Systemic Lupus Erythematous Induced Coronary Artery Vasculitis Douglas Crowley, MD; Richard Friedman, MD; Katherine Nguyen, MD Scripps Mercy Hospital CASE: A 17 year old female presented to the ED with severe chest pain radiating to her left scapula, neck, jaw and left arm. The pain was constant over 5 hours and was associated with SOB, nausea, diaphoresis and bilateral arm numbness. Twelve days prior to this presentation the patient underwent laparoscopic cholecystectomy for acalculous cholecystitis. Gallbladder pathology showed necrotizing median vessel vasculitis resembling Polyarteritis Nodosum (PAN). She was discharged with the tentative diagnosis of PAN and given 60mg of prednisone daily. On this presentation, the patient was in moderate distress and diaphoretic, with vitals significant for a HR of 140. EKG showed inferior and lateral wall ST elevations and cardiac markers were elevated. Urine toxicology and pregnancy screens were negative. She was taken for cardiac catheterization which showed a large dominant right coronary with 100% occlusions at the mid posterolateral branch and the distal right. Acute marginals had multiple beaded areas consistent with thrombus. Normal left main, however the LAD was 100% obstructed in the mid portion. Large first diagonal and circumflex were both 100% obstructed in the mid portion with visible thrombus in circ with multiple occluded small branches. Due to the vast extent of coronary involvement and no evidence of atherosclerotic lesion or plaque no stents were deployed. The patient was continued on heparin drip, given full dose of aspirin and eptifibatide, loaded with clopidogrel, and started on reteplase. Echocardiogram showed cardiomyopathy with an EF of 15-20% and depressed right ventricular function. Lab studies showed a positive ANA and DS DNA, elevated ESR and CRP and depressed C3. Anti-phospholipid, HIV, hepatitis, ANCAs, and Anti-Smith labs were negative. The patient was diagnosed with SLE vasculitis of the coronary arteries and was started on solumedrol 60mg IV BID and cyclophosphamide 2mg/ kg/ day. For CHF management lisinopril, metoprolol, atorvastatin, aspirin and clopidogrel were initiated. Repeat echocardiogram showed an EF of 40%. The patient was discharged on day 30 on a prednisone taper, the above mentioned cardiovascular medications and was transitioned to a three month cyclophosphamide pulse regiment. DISCUSSION: Cardiac involvement in patients with SLE is not unusual with studies demonstrating a prevalence of 50%; however, SLE induced coronary vessel vasculitis resulting in an acute MI is rare. Most cases of acute coronary syndrome and SLE involve accelerated atherosclerosis secondary to the vasculitis leading to premature coronary artery disease. The rare cases of SLE induced coronary artery vasculitis have high morbidity and mortality and prompt recognition and alterations in therapy are required. Hysterical Dysphagia Matthew Crull, MD; Ian H. Jenkins, MD; Omeed Saghafi, BS University of California, San Diego Once commonplace in the differential diagnosis of dysphagia, Plummer-Vinson Syndrome is nowadays infrequently reported. Currently, the very existence of the syndrome is challenged. This syndrome may, however, still have clinical significance. A 41 year-old African American woman with a history of menorrhagia and no recent primary care presented complaining of fatigue. She also noted progressive dysphagia of solids for the past 3 years. Examination revealed koilonychia, cheilosis, atrophic glossitis, and conjunctival pallor. The hemoglobin was 4.3 g/dl, the MCV was 48.3, the iron was 6 mcg/dl, and the ferritin was undetectable (<1 ng/ml). A barium esophagram was done to better characterize the dysphagia, and showed probable esophageal webs. Esophageal webs were confirmed by upper endoscopy; the webs were successfully dilated with a balloon dilator. She was transfused 4 units of packed red cells, treated for menorrhagia with oral contraceptives, and started on ferrous sulfate with ascorbic acid for her iron deficiency anemia. At the time of discharge, she reported improvement in her dysphagia. Plummer-Vinson Syndrome presents with the classic triad of dysphagia, iron deficiency anemia and esophageal webs. Patterson and Kelly independently described 18 the triad of iron-deficiency anemia, dysphagia, and upper esophageal webs in 1919 following Plummer (1912) and VinsonвЂ™s (1919) similar but less comprehensive descriptions of вЂњhysterical dysphagia.вЂќ Plummer-Vinson syndrome is rare today; exact data about incidence and prevalence are not available. Whether iron deficiency truly causes esophageal webs is debated, but iron deficiency is thought to weaken esophageal musculature and cause epithelial cell atrophy. Autoimmunity and genetic predisposition are other postulated causes. Dilation of the webs is usually curative, although their association with an increased risk of upper alimentary cancers may justify surveillance endoscopy. Koilonychia, glossitis and angular cheilitis, which are often found in patients with varying degrees of malnourishment, are frequently reported in those with Plummer-Vinson syndrome in addition to the classic triad of findings. The proposed mechanisms are similar to those in the development of esophageal webs, and include depletion of iron containing enzymes involved epithelial cell turnover. While iron-deficiency anemia is frequently found in women of childbearing age, Plummer-Vinson Syndrome is rarely encountered in current times. Not only does this case illustrates unique physical exam findings, but may still have clinical relevance due to its association with cancer involving the upper esophagus. cardiomyopathy. Marked ascites was seen on abdominal US. She reported that over ten members of her family in three generations have had cardiac and cerebrovascular disease at relatively young ages. Her father had atrial fibrillation and ascites. Her family pedigree was significant for father to daughter transmission with no skip generations, seen typically with an autosomal dominant pattern. The patient was started on multiple anti-arrhythmic drugs to assist with treatment of her atrial flutter as well as anticoagulation therapy. She was also started on diuretics to assist with her right heart failure. Familial restrictive cardiomyopathy is an autosomal dominant cardiomyopathy histologically characterized by myocyte hypertrophy and interstitial fibrosis. Other causes of restrictive cardiomyopathy include endomyocardial fibrosis, infiltrative diseases such as amyloid and sarcoid, scleroderma, carcinoid heart disease, malignancies, and medications. Complications seen in familial restrictive cardiomyopathy include development of bundle branch blocks with eventual heart block and progressive skeletal myopathy. Interrupted Aortic Arch Presenting as Myocardial Infarction Daniel Daneshvar, MD; Kiran Philip, MD Cedars-Sinai Medical Center Familial Restrictive Cardiomyopathy Daniel Daneshvar, MD; Gautam Kedia, MD; Robert J. Siegel, MD Cedard-Sinai Medical Center Restrictive cardiomyopathy (CM) is a type of mixed primary CM with a primary abnormality of diastolic dysfunction. Common symptoms with this form of cardiomyopathy are increased jugular venous pressure (JVP), peripheral edema, and ascites due to right heart failure. There are many causes of this form of cardiomyopathy, with a number of reports of idiopathic restrictive cardiomyopathy with familial roots. A 35 year-old female presented with palpitations, shortness of breath, and abdominal bloating. She had always felt that her stamina was low & recently noticed her heart was beating faster than usual. The patient has a history of abnormal ECGs, told she had an abnormal p wave. Family history was significant for multiple family members dying at an early age. On exam, the patient was tachycardic, with a split S2 and a grade II/VI systolic murmur heard best at the left sternal border. JVP was increased to the angle of the jaw. Her abdomen was distended with a positive fluid wave and marked hepatomegaly. Her extremities showed dependent cyanosis in both feet. CXR revealed right ventricular enlargement with mild pulmonary vascular congestion. ECG exhibited tachycardia consistent with atrial flutter. An echocardiogram and cardiac catheterization revealed normal systolic function, abnormal left ventricular relaxation, marked biatrial enlargement, increased right atrial pressure, tricuspid regurgitation and increased end diastolic pressure. Doppler parameters were consistent with restrictive physiology consistent with restrictive Interrupted aortic arch (IAA) is defined as a loss of luminal continuity between the ascending and descending portions of the aorta. It is a rare congenital malformation with an incidence of approximately 3 cases/million live births and accounts for about 1% of congenital cardiovascular defects. IAA is typically associated with other cardiovascular anomalies and is usually diagnosed and repaired in the neonatal period. IAA in adults is exceedingly uncommon and may be misdiagnosed as primary HTN. A 29 year-old Hispanic male with a history of hyperlipidemia and HTN presents with chest pain. He reports intermittent shortness of breath and non-exertional chest pain for 4 days, worsened on the day of admission while driving. He described the pain as left-sided, heavy and burning, 10/10 in severity without radiation, nonpleuritic in nature, associated with some nausea without vomiting or light-headedness. Initial ECG suggested an acute MI. He was given ASA 325 mg and 2 NTG tablets sublingually with mild improvement. Patient was active as a child, playing football. He recently noticed increasing symptoms while standing at work. The patient is noncompliant with his medication regimen of gemfibrozil, amlodipine, and HCTZ. He smokes one pack of cigarettes daily, with occasional alcohol use. His family history is noncontributory. On exam, BP 140/86, other vitals were stable. Lung exam was clear to auscultation and heart exam was regular rate and rhythm with a grade III/VI systolic murmur at the base. Extremities had no edema and 1+ distal pulses. ECG revealed ST segment elevation in lead II, III, & aVF with reciprocal ST depressions in V2-6 & inverted T waves in V4-6 with left ventricular hypertrophy. Troponin was initially 0.1, then 10.97 with a CXR showing cardiomegaly. He was taken emergently 19 for cardiac catheterization; the guide wire was unsuccessfully advanced through the aorta, with angiogram and transthoracic echocardiography findings concerning for the presence of a aortic dissection vs. aortic coarctation. He was then taken to the OR for a diagnostic transesophageal echocardiogram which demonstrated a hypoplastic distal aortic arch and inferior wall hypokinesis. Chest CT angiogram and MRI showed no evidence of aortic dissection but found a 4.7 cm interruption of the aortic arch proximal to the expected origin of the left subclavian artery with enlarged intercostal artery collaterals. The patient was also found to have right coronary artery occlusion on repeat cardiac catheterization through the radial artery. He was scheduled for elective aortic repair after recovery from his MI. The case illustrates an extremely rare presentation of isolated IAA in adulthood with an ST elevation MI. Common Drug Causes Rare Life-Threatening Side Effect Omar Darwish, MD; Jasminka Criley, MD St. Mary Medical Center Hydrochlorthiazide (HCTZ), an inexpensive and effective drug, is one of the most common antihypertensive medications prescribed in the United States. Well-known side effects of the drug include electrolyte imbalances, hypotension, hyerglycemia, and various gastrointestinal complaints. However, a select few have had a rare lifethreatening side effectвЂ”non-cardiogenic pulmonary edema with shock. A 46-year-old woman with hypertension developed dyspnea, lightheadedness and vomiting 20 minutes after taking one tablet of 25 mg HCTZ. She felt lightheadedness with her first HCTZ ingestion a few months earlier and had not taken it since then until the day of admission. She was otherwise a healthy stay-at-home mother. Home medications included HCTZ 25 mg and Atenolol 50 mg. Physical examination revealed a lethargic woman with O2 sat 80% on room air, temperature 98В° F, pulse 98, respiratory rate 24, and a blood pressure 115/88. The neck veins were flat. Auscultation of the chest revealed bilateral rhonchi. Heart sounds were normal without gallop. Laboratory data were significant for WBC 5,100/uL, lactate level 3.5 and BNP 12. Arterial blood gas demonstrated a PaO2 56 mmHg, PaCO2 63 mmHg, and a pH of 7.16 on a non-rebreather mask. The electrocardiogram showed normal sinus rhythm with right axis deviation. Chest X-ray showed bilateral diffuse alveolar infiltrates. Transthoracic echocardiogram revealed normal ejection fraction, no right-sided heart strain, normal valves and chamber sizes. Spiral chest CT showed no pulmonary embolism but diffuse alveolar consolidations. The patient was intubated and mechanically ventilated. She then became febrile to 104.1Вє F and blood pressure dropped to 60/48 requiring 5 L of fluids and 20 micrograms/min of levophed. After thirty-five hours on the ventilator, the patient continued to require high positive end-expiratory pressures and pressors. Bronchoscopy was done and the brochoalveolar lavage revealed 12 percent lymphocytes, 18 percent macrophage, 70 percent granulocytes and no eosinophils. Alveolar hemorrhage was not seen. Bacterial, AFB smears, fungal, and viral cultures were all negative. Blood cultures were negative as well. In addition to mechanical ventilation, crystalloids, and pressors, she was treated empirically with broad-spectrum antibiotics and a stress dose of steroids for possible septic shock. The patient was successfully weaned from levophed within 36 hours and was extubated on day four of her hospitalization. Since her symptoms developed immediately after taking HCTZ, she was presumed to have developed HCTZ induced pulmonary edema, a recognized but rare side effect of the drug. She was discharged seven days after admission with instructions to abstain from hydrochlorothiazide in the future. Development of respiratory failure with shock is a rare consequence of hydrochlorothiazide ingestion. Since this medication is prescribed to millions of patients, knowledge of not only the common adverse reaction is important, but also the most lethalвЂ”pulmonary edema with shock. A Mysterious Red Rash: Don't Blame the Mosquitoes Monya De, MD, MPH; Kalpesh Patel, MD Olive View-UCLA Medical Center Case: A 55-year-old Guatemalan female presented with four days of fevers, chills, bitemporal headache, and itchy truncal rash. One month earlier, the patient had returned to the United States from visiting relatives in Guatemala for two weeks. She reported many mosquito bites during the trip and no sick contacts or new medications. Initial PE: normotensive, HR 107, T 39.1. She was flushed appearing, with an erythematous morbilliform rash with some areas of confluence over the entire body, sparing the face, lower legs, palms, and soles. Labs: WBC normal, BMP normal, new transaminitis (AST 262, ALT 223) alkaline phosphatase normal. UA: microscopic hematuria and pyuria. CXR: normal Infectious disease was consulted, and the initial impression was dengue fever. An extensive workup was ordered, including HHV6, parvovirus, dengue, HSV, VDRL, thick and thin smear, ASO titer, HIV, and HSV (all negative). Ceftriaxone and doxycycline were started empirically. The patientвЂ™s rash progressed and spread to the previously spared locations, with formation of large bullae. She had milia on the scalp in addition to erythema from the rash. She did not have any visible lesions in the oropharynx, but stated she had felt one blister pop. She developed a leukocytosis with lymphocyte predominance. She became very edematous and required aggressive fluid resuscitation for persistent hypotension and tachycardia. A skin biopsy showed bullous dermatitis. During the admission, there was a change of fellow on the ID service. The new fellow questioned the patient more directly about new medications taken, and she finally revealed that, while in Guatemala, her physician brother had run вЂњroutine testsвЂќ including serum uric acid. Despite that she was asymptomatic, he gave her 20 allopurinol for her elevated uric acid, which she took for approximately 5 weeks until her symptoms began. He also gave her terbenafine for a fungal infection of the feet. She had not thought these medications important to mention on admission as they were already stopped. The timing was consistent with a drug eruption, and IV steroids were initiated, resulting in dramatic improvement of the rash, transaminitis, and edema. As both allopurinol and terbenafine are associated with drug eruption, it is impossible to define a single offending agent, and both may have acted synergistically. Discussion: This case serves to remind us of several important attributes of a successful diagnosis: probing history-taking, вЂњfresh eyesвЂќ looking at a complicated patient, and keeping a wide differential. In particular, the dramatic severity and high incidence in the literature of the allopurinol drug hypersensitivity reaction and its underemphasis in medical education indicate a need for awareness among clinicians in general and specialty practice who initiate this very common drug. Cocaine Induced Peripheral Limp Vasculitis Requiring Amputation Rahul Dhawan, DO; Joseph Carey, MD; Roger Chan, MD; Divya Sachdev, BA University of Southern California Keck School of Medicine Department of Internal Medicine We present a case report of a 46-year-old Caucasian female with history significant for cocaine addiction and bipolar disease who presented to Los Angeles County+University of Southern California Medical Center with hypotension, hemoptysis, and coagulopathy. She was a known heavy inhalational and injection cocaine user. During her acute critical illness, she required intubation and transfusion, and developed full thickness necrosis of skin in the extremities. She demonstrated acute thrombosis of the distal radial and ulnar arteries, as well as the Dorsalis pedis and Posterior tibial arteries. She ultimately required below elbow amputation of her right arm and a below the knee amputation of her left foot, and debridement and grafting of the left foot. An acquired vasculitis was suspected. She had rheumatologic serologies drawn, and was found to be positive for Antineutrophil cytoplasmic antibodies (ANCAs), a group of mainly IgG antibodies against antigens in the cytoplasm of neutrophil granulocytes. Small artery biopsies confirmed the diagnosis of acute vasculitis. It should be studied whether individuals with positive ANCA serologies are more likely to suffer peripheral vasculitis if exposed to excessive catecholomines such as cocaine. T Wave Alternans Preceding Cardiac Arrest in Long QT Syndrome Jashdeep Dhoot, MD; Holly Middlekauff, MD University of California, Los Angeles Case Presentation: A 31 year old male with history of Goodpasture''s syndrome, two failed renal transplants and chronic hypocalcemia developed cardiac arrest with polymorphic ventricular tachycardia during a third renal transplant operation. Preoperative workup included negative cardiac stress testing. Prior to surgery, his ionized calcium was 0.89 mmol/L and his serum potassium was 3.2 mmol/L. An EKG showed normal sinus rhythm with old non-specific T-wave inversions and a prolonged corrected QT segment of 573 msec. Vancomycin, methylprednisolone, and anti-thymocyte globulin was administered prior to the procedure. Approximately four hours into his operation prior to reperfusion of the new renal allograft, the patient went into ventricular fibrillation arrest. Cardiopulmonary resuscitation was performed for 20 minutes, which included three defibrillations, before normal sinus rhythm with palpable pulses was regained. He was then hemodynamically stable for the rest of the procedure and the operation was completed without major complications. Post-operative labs were significant for a magnesium of 1.3 mEq/L. Post-operatively the patient was monitored on telemetry in the ICU without an event. He regained full neurological recovery after extubation. Despite repletion of his electrolytes and avoidance of QTprolonging medications, the patients corrected QT interval remained prolonged. Retrospective analysis of the patientвЂ™s intra-operative telemetry strip revealed beat-tobeat changes in T wave polarity, a phenomenon known as T wave alternans, for several minutes prior to the sudden onset of torsades de pointes polymorphic ventricular tachycardia. Given these unique clinical findings suggesting congenital long QT syndrome (LQTS), the patient had an automatic implantable cardioverter defibrillator (AICD) placed prior to discharge. Genetic testing for congenital LQTS is pending at this time. Discussion: Long QT syndrome (LQTS) involves marked prolongation of the electrocardiographic QT interval that predisposes to a distinctive form of ventricular arrhythmia known as torsades de pointes. Clinically, this syndrome presents as recurrent dizziness, syncope, or sudden death. The etiology of this syndrome may be either acquired or congenital. The distinction is important since therapies for the two forms differ. The congenital form generally requires long-term beta-blocker therapy which can be harmful in the acquired form. T-wave alternans has been observed in a wide variety of clinical and experimental conditions to be associated with ventricular arrhythmias including acute myocardial infarction, electrolyte imbalances, and LQTS. In association with LQTS, T-wave alternans immediately preceding the development of torsades de pointes is a finding relatively specific to the congenital form. Therefore it is helpful in distinguishing it from the acquired form. It also portends a possible impending ventricular arrhythmia. In patients diagnosed with the congenital form of LQTS, genotyping can give insight into the patientвЂ™s potential response to betablocker therapy and prognosis. Also, genotype testing aids in helping identify family members who may be at risk. 21 Pregnant Women: The Silent Victims of the H1N1 Influenza Pandemic Miliary Presentation of Bronchioloalveolar Carcinoma Mary Elmasri, MD University of California, San Diego Elysia Engelage, MD*; Shahriar Pirouz, MD; Nader Kamangar, MD, FACP, FCCP, FAASM *University of California, Los Angeles, Olive ViewUCLA Medical Center Case: A 28-year-old female at 26 weeks gestation presented to an outside hospital with four days of fever, shortness of breath, and cough as well as myalgias, rhinorrhea and sore throat. Initial chest x-ray at the outside hospital showed a right basilar opacity and her rapid influenza panel was positive for influenza A. She was initially started on oseltamivir and piperacillin/tazobactam. Her course was significant for worsening hypoxia requiring BIPAP and acute kidney injury and volume overload requiring dialysis. The patient was transferred to our medical center approximately forty-eight hours after initial presentation. Physical exam upon transfer was notable for tachypnea, oxygen saturation 100% on 60% BIPAP, mild respiratory distress, and bibasilar crackles. Labs were significant for a leukocytosis of 19,000 with a bandemia of 64%. Chest x-ray upon transfer showed a persistent right basilar opacity. She was continued on oseltamivir and her antibiotics were broadened to vancomycin, ceftazadime, and azithromycin. Influenza culture done at the outside hospital was positive for influenza A, which was then confirmed to be H1N1. On hospital day two at our institution the patientвЂ™s respiratory status continued to deteriorate and she was intubated for hypoxemic respiratory failure. Immediately afterward, the patient underwent emergent cesarean section. Her baby was taken to the neonatal intensive care unit and did well. The patient subsequently developed acute respiratory distress syndrome and was ventilated using lung protective strategies. The patient had a complicated month long hospital course requiring advanced modes of mechanical ventilation, such as bi-level ventilation, to maintain oxygenation. She ultimately required a tracheostomy and was discharged off the ventilator to an acute care facility. Discussion: The H1N1 influenza virus has caused a pandemic of febrile respiratory illness throughout the world. According to the CDC as of August 2009, greater than 98% of circulating influenza viruses in the United States were the H1N1 influenza virus. Initial data found that 30% of all women ages 20-39 who died from H1N1 infection were pregnant or postpartum. This case illustrates the fact that pregnant women are at higher risk for severe illness with increased morbidity and mortality from H1N1 infection and highlights their complicated and devastating course. During this pandemic it is imperative that pregnant women receive immediate treatment with antiviral medication when there is clinical concern for infection. It will also be essential that pregnant women receive early immunization when the vaccine becomes available later this fall. Case: A 54 year old woman with no significant past medical history presented to our hospital with one month of dry cough. After seeing her primary care physician, she promptly underwent a chest radiograph and was referred to our emergency room for further evaluation. She denied any fevers, chills, hemoptysis, night sweats, weight loss, or tobacco use. Her only other complaint was mild hip pain and decrease in exercise tolerance. She reports an abnormal chest radiograph approximately 5 years prior to presentation for which no further action was taken. She emigrated from Korea approximately 25years ago and lives in the San Fernando Valley. Chest x-ray obtained in the ER showed diffuse reticulonodular disease with upper lobe predominance. Subsequent CT of the chest showed confluent peribronchovascular opacities in bilateral upper lobes and imaging of the abdomen and pelvis showed nodules within the left adrenal gland, liver and several bony lesions. She was placed in TB isolation and started on RIPE therapy while awaiting AFB sputum, urine, stool, coccidioidomycosis and histoplasma serologies. She was taken for bronchoscopy with pathology from the biopsies showing bronchioloalveolar carcinoma. TB isolation and medications were discontinued, Oncology was consulted, she was begun on Tarceva and dexamethasone and discharged home. Discussion: We present an unusual case of BAC presenting radiographically similar to disseminated tuberculosis. Bronchioloalveolar carcinoma (BAC), once a rare subtype of pulmonary adenocarcinoma has in recent years increased its prevalence and now accounts for approximately 24% of all lung cancers. It is known as a cancer of non-smokers and more frequently affects women and Asians. The most common signs and symptoms are cough, chest pain, dyspnea and hemoptysis. BAC most commonly presents as a solitary nodule, but can also present as multiple synchronous nodules, or a diffuse parenchymal process. Radiographic findings that raise suspicion of BAC include ground-glass opacities, nonresolving consolidation, and satellite nodules. There is evidence that the presence of any BAC features predicts improved survival, stage for stage, other types of nonsmall cell lung carcinoma (NSCLC). A study of patients with IIIB or IV disease demonstrated a median survival time of 15 months versus 10 months for NSCLC. Our patient reports an abnormal chest radiograph approximately 5 years prior to her presenting at our hospital. This underscores the importance of regular follow up and further investigation into abnormal radiographs. Thyrotoxicosis Fails the Right Heart 22 Babak Eshaghian, MD, MS; Roshni Shah, DO; Michael Hochman, MD; Babak Saedi, MD; Grace Huang, MD; Jooby Babu, MD University of Southern California the increased metabolism of intrinsic pulmonary vasodilator. This case raises the awareness that isolated right heart failure is a complication of thyrotoxicosis. Chest Pain: The Great Masquerader Case: A 37 year old female with a past medical history of hypertension came to the ER complaining of shortness of breath for 10 days, palpitations and anasarca. She reported 8-9 months of amenorrhea, anxiety, weight loss, and two months of watery diarrhea. In the ER she was in atrial fibrillation with right ventricular response and was started on diltiazem. Physical exam was significant for bilateral exophthalmos and an enlarged thyroid. Breath sounds were diminished at the right lung base, the heart rate was irregularly irregular, the abdomen was distended, and there was hepatomegaly, and anasarca. Thyroid panel revealed low TSH (0.01mIU/L), elevated free thyroxine (>7.77ng/mL). Significant labs include elevated alkaline phosphatase, total and direct bilirubin, and prolonged prothrombin time. Upon admission to ICU diltiazem was discontinued and the patient was started on propranolol. However, the patient became very hypotensive, bradycardic and hemodynamically unstable. She was given atropine, glucagon and started on a dopamine drip. Chest X-ray indicated right pleural effusion and thorcentesis confirmed a transudative fluid. Ultrasound of the abdomen showed ascites. Echocardiogram revealed normal LV function with ejection fraction of 64% and moderately enlarged right ventricle and right atrium, moderate to severe tricuspid regurgitation, and elevated right ventricular systolic pressure 40-50mmHg. Patient also had elevated central venous pressures ranging from 27-32cmH2O and elevated pulmonary arterial pressures; systolic readings in the upper 40вЂ™s, suggesting a diagnosis of isolated right heart failure. She had low systemic vascular resistance of 716, and elevated cardiac output pointing towards high-output cardiac failure due to the effects of high triiodothyronine (T3). Patient was started on potassium iodide, PTU, and decadron per endocrinology. With aggressive management the patient became hemodynamically stable with decreasing mean pulmonary arterial pressures and liver function tests. A follow-up chest X-ray demonstrated decreased pleural effusion. The patient was clinically improved and was discharged on methimazole and propranolol. Discussion: There are several differentials to consider when evaluating right heart failure; however, thyrotoxicosis is not commonly on that list. Frequent symptoms of thyrotoxicosis include palpitation, sinus tachycardia, atrial fibrillation, hypertension, left heart failure, and angina. Right heart failure is a rare manifestation of thyrotoxicosis and there are very few cases reported. Over the past 15 years there have been very few case reports of thyrotoxicosis causing isolated right heart failure. Here we present a case of a patient with severe thyrotoxicosis, causing elevated pulmonary artery pressures leading to right heart failure. Possible mechanisms of pulmonary hypertension induced by hyperthyroidism entail pulmonary vascular endothelial dysfunction or damage due to the autoimmune process, the high cardiac output state, or Heidi Fagerlund, MD; Jigar Patel, MD Case: A 33 year old male with a history of ankylosing spondylitis and coronary vasospasm presents with polyarthralgias, fever and chest pain. The patient was initially evaluated in an Urgent Care Center for a one week history of diffuse polyarthralgias and fevers. He was subsequently diagnosed with presumed influenza and discharged home. He returned for evaluation secondary to continued fever, new onset migraine symptoms, nausea and substernal chest pain with radiation to the neck. On evaluation his temperature was 39.5 C, blood pressure 115/72, heart rate 72 and oxygen saturation 99% on room air. He was slightly diaphoretic with a normal jugular venous pulsation. Cardiovascular and pulmonary examinations were normal, with no murmurs, rubs or gallops auscultated. There were no focal deficits or meningeal signs. Initial electrocardiogram (EKG) displayed diffuse ST elevations. Chest x-ray did not identify any acute cardiopulmonary process. Transthoracic echocardiogram was performed and revealed normal left ventricular (LV) size, normal LV thickness, and normal LV function, with a preserved LV ejection fraction. There was no evidence of pericardial effusion or wall motion abnormalities Review of previous coronary angiogram in 2005 was negative for obstructing lesions. Laboratory values were significant for sedimentation rate 6, AST 158, ALT 210. Serial cardiac markers revealed a peak Troponin-I of 9.08, total CK 484 and CK-MB fraction of 44.3. Cardiac MR illustrated a pattern of late enhancement consistent with myocarditis. CMV IgM serology returned positive at 1:10 with a negative IgG antibody. The presence of CMV was confirmed by PCR. Serologies for other viruses including HIV and EBV were negative. Prior to discharge our patient was started on Colchicine therapy with improved symptoms. Discussion: This case was unique in that although CMV is a ubiquitous organism, CMV myopericarditis is an unusual and relatively under-studied entity. Of particular interest is that almost all documented cases of CMV pericarditis have been in immunocompromised or transfused hosts whom are rarely seropositive. Ankylosing spondylitis portends additional importance, although unlikely related to acute myopericarditis, there is a higher incidence of Aortic Insufficiency and conduction disturbances in this population. This case highlights the importance of identifying atypical etiologies of a common complaint in the ever emerging field of science. Recurrent Pneumothoraces As a Complication in a Patient with Idiopathic Lipoid Pneumonia Farbod Farmand, DO; Castro, Robert DO; Chen Cynthia, MS3 23 Western University of Health Sciences Lipoid pneumonia is a very rare pulmonary disorder resulting from exogenous inhalation of lipid materials or endogenous deposition of fat in the lung tissue. Its diagnosis is usually difficult as there are no pathognomonic imaging studies or serological tests for it. Here we report the case of a patient with recurrent pneumothoraces and underlying lipoid pneumonia found on lung biopsy. Since there was no positive history of lipid exposure or endogenous mechanism, it was diagnosed to be idiopathic. To our knowledge, this is the first reported case of idiopathic lipoid pneumonia that presented with recurrent pneumothoraces. A Rare Cause of Headache and Gait Instability Charles Feng, MD; Pouya Afshar, MD; Charles Smith, MD Scripps Clinic/Green Hospital CreutzfeldtвЂ“Jakob Disease is a prion disorder that causes severe neurological dysfunction. It commonly presents with rapid-onset dementia, myoclonus, ataxia, and speech impairment. We report a 64-year-old female who came to clinic for a headache and difficulty ambulating. At the same time, she stated that her thought processes had "dulled," and that she had problems recalling facts. The patient had been well prior to the visit. Physical exam did not reveal any evidence of dementia. Aside from a mildly unsteady gait, there were no focal neurologic deficits. Further, a work-up for dementia, including CT of the head without contrast, B12, folate, ESR, CRP, and TSH, was negative. The patient was referred for physical therapy for her gait disturbance. Two months later, the patient came back for follow-up, and her condition had noticeably deteriorated. She had difficulties speaking and could not stand without the assistance of her husband. Physical exam showed a saccadic ocular pursuit, and unsustained upbeat nystagmus on upward gaze. An extensive work-up was initiated. The cerebrospinal fluid was acellular, with normal glucose and protein levels. A test for the anti-Yo antibody, which is specific for paraneoplastic cerebellar degeneration, was negative. However, EEG showed background slowing with periodic sharp waves and bursts of high-voltage delta waves. In addition, MRI showed bright cortical signaling over the right frontal lobe and the medial aspect of the right occipital lobe on diffusion-weighted imaging. Given the high clinical suspicion for CJD, the 14-3-3 protein, found in the CSF, was sent off and came back positive. The patient passed away one month after her last clinic visit. Our current knowledge of CJD is limited. A diagnostic test such as EEG has a sensitivity of 64 percent and a specificity of 91 percent, while an abnormal 14-3-3 protein has sensitivities and specificities ranging from 53 to 88 percent. Thus, definitive diagnosis can only be made with a brain biopsy. WhatвЂ™s more, there is no effective treatment for the disease. CJD occurs with a prevalence of 1 case in one million people, but the rapidity with which a patient declines after diagnosis makes CJD a devastating disease to endure. This case illustrates the importance of considering CJD in the initial differential of a patient with rapid-onset dementia and ataxia. Even though there are no treatments, early diagnosis has many practical benefits. The patient and her family, along with her physician, can properly manage expectations, and can mentally and physically prepare for what will happen as the disease progresses. Moreover, early diagnosis would allow for more clinical trials to be performed, so that, in time, a cure for CJD will eventually be found. CancerвЂ¦. Not Always the Answer Aimee French MD; Nader Kamangar MD Olive View- UCLA Medical Center Case: 32 year-old nulligravid heterosexual Hispanic female, diagnosed with HIV/AIDS presented with two weeks of pressure-like abdominal pain, increasing abdominal girth, nausea and vomiting. patient also endorsed subjective fevers, chills, and night sweats. She noted a 40lbs unintentional weight loss. She denied cough and hemoptysis. Her only medical history was HIV/AIDS. She was taking HAART therapy , TrimethoptimSulfamethoxazole, and Azithromycin. She was febrile to 39.7, BP 116/65, HR 80, RR 18, and oxygen saturation 100% on room air. She was ill-appearing and cachectic. She had bilateral post-cervical, submandibular and axillary lymphadenopathy all of which were firm, fixed, and non-tender. Pelvic exam revealed bilateral tenderness of the adenexa. No adenexal fullness was noted. The remainder of her exam was normal. Laboratory studies revealed a CD4 count of 60 cells/mL and an HIV RNA of 2970 copies/mL. CA-125 72 units/mL (normal 0-34). The following studies were (-): sputum AFB, fungal stains and cultures, Pneumocystis jiroveci pneumonia (PCP), cytomegalovirus (CMV), coccidioides, and cryptococcus; blood cultures for bacteria and fungus; urine for histoplasmosis and legionella. CT chest revealed scattered bilateral parenchymal nodular opacities with mediastinal and hilar lymphadenopathy suspicious for infectious or metastatic disease. Abdominal CT scan revealed omental caking, ascites, lymphadenopathy throughout the abdomen including the pelvic side walls, and cystic structures present on bilateral adenexa suspicious for ovarian malignancy. Fine needle aspiration of enlarged lymph nodes and interventional-radiology guided biopsies of the omental wall revealed necrotizing granulomas with extensive necrosis and visible acid fast bacilli. AFB stool was (+). Discussion: This case illustrates that without a high clinical suspicion the diagnosis of abdominal tuberculosis may be missed or delayed. Once thought to be rare in developed countries, the incidence of abdominal TB is 24 rising. With the recent HIV/AIDs pandemic the incidence of tuberculosis is growing along with the frequency of extrapulmonary disease. Abdominal TB is also more prevalent and may or may not include pulmonary manifestations. Without a high clinical suspicion diagnosis may be missed or delayed. Multiple case reports and journal articles site the repeated misdiagnosis of abdominal TB as malignancy specifically ovarian cancer and lymphoma. As with our patient, tissue biopsy made the diagnosis in these misdiagnosed cases. Without coexisting work-up for abdominal TB therapy in this patient therapy may have been delayed. Amyotrophic Lateral Sclerosis Presenting as Recurrent Pseudomonas Pneumonias Natalie Garcia, MD University of California, San Diego Case: A 56-year-old man was transferred from an outside hospital with pneumonia and weight loss. Repeat sputum cultures grew Pseudomonas, and the patient was treated with three courses of antibiotics during the two months prior to transfer. Review of the patientвЂ™s history revealed he had been admitted to various institutions a total of five times in the past two years for recurrent Pseudomonas pneumonias and had previously been diagnosed with obstructive lung disease based on hyperexpansion on chest x-ray, though no pulmonary function studies were available. In addition, though he had been obese, the patient was cachectic on presentation with a 97kg weight loss over the previous two years. He had a history of depression and aggressive dieting, and the weight loss was initially attributed to a combination of these compounded by his recent, prolonged illness. During the initial two weeks after transfer, the patient had recurrent decline in his respiratory status requiring mechanical ventilation. He would improve gradually with therapeutic bronchoscopy, aggressive pulmonary toilet and physical therapy only to worsen again. After two months of hospitalization, the patient required on-going, night-time mechanical ventilation to avoid hypercapnic respiratory failure. Neurology was consulted for muscle weakness and atrophy, which were initially thought to be secondary to his recurrent pneumonias and prolonged illness in combination with statin therapy. On repeat evaluations and exams, he was noted to have the onset of tongue fasciculations and hyperreflexia in his lower limbs. Given the progressive nature and the development of both upper and lower motor neuron signs, amyotrophic lateral sclerosis was diagnosed. Discussion: This case reiterates the potential variability in presentation of amyotrophic lateral sclerosis and emphasizes the need to pursue an underlying diagnosis rather than to simply treat the complications. Primary respiratory muscle weakness is the presenting symptom in only 1-3% of cases of this rare disease, and early diagnosis of hypoventilation followed by pulmonary function tests, demonstrating low forced vital capacity, and, possibly, phrenic nerve conduction studies may be useful. The ultimate recognition of this patientвЂ™s disease was integral in formulating a long-term treatment plan and in helping him cope with his illness. Thyroid in the Brain Vikram Garg, MD; Tanya-Sue Winey, MD Scripps Green Hospital Case Presentation An 81 year-old female presented symptoms of left sided weakness and ataxia for 3 weeks. The weakness was causing her difficulty with walking. She had a history significant for papillary thyroid carcinoma which was initially diagnosed in 2001 and treated with surgery and radiation. She suffered 2 recurrences in regional lymph nodes in 2004 and 2005 and she was treated at both times with surgery and radiation. Her current medications include Lipitor and Synthroid. She is a non-smoker, originally from Mexico, and moved to the United States 5 years ago. Family history was significant for 1 brother with leukemia. On exam she was afebrile and hemodynamically stable. Pertinent neurological findings included 3 beat nystagmus with leftward gaze, left facial droop, left sided lower and upper extremity strength 4/5 compared to 5/5 on the right, sensory deficits on the left compared to the right, and ataxia towards the right with ambulation. The remainder of the physical exam was unremarkable. Initial basic metabolic panel and liver function tests were unremarkable. Her CBC showed a white cell count of 7.0, hemoglobin of 13.2, hematocrit of 36.7, and a platelet count of 207. ESR was13 and her INR was 1.0. An MRI showed a 3.6 cm x 2.6 cm right posterior frontal lobe mass with surrounding edema and multiple hemorrhagic foci within the mass (Figures 1 and 2). Imaging evaluating for other possible metastatic sites was unrevealing. Neurosurgical consultation was obtained and the patient opted to have surgical removal of the mass. Biopsy results from the mass revealed papillary thyroid carcinoma. The patient recovered from the surgery quite well and was discharged in stable condition to a neurological rehabilitation Discussion Several studies have looked at the incidence of cerebral metastases of papillary thyroid carcinoma and the results range from 0.4% to 3% as the first site of distant metastatic involvement (1,3). However, when looking at second or subsequent distant metastatic sites approximately 1/3 involved the brain in one retrospective study (3). Patients with brain metastases almost always have complications with metastases at other sites such as liver, lung, or bone with the lung being one of the common distant metastatic sites in one study (3). Cerebral metastases suggest an aggressive disease and have an unfavorable prognosis. However, early detection and treatment can lead to long-term survival. Our patient had local lymph node recurrence but no distant metastatic organ involvement so this case was unique in that the brain seemed to be the first site of distant metastasis. 25 ICD Lead Thrombus Causing Multiple Pulmonary Emboli Paul Gerczuk, MD University of Southern California This is a 36-year-old Hispanic female with a known history of congenital long QT syndrome who was admitted to the hospital because of an implantable cardioverter defibrillator (ICD) lead thrombus, which was incidentally found on routine echocardiography. The patient was diagnosed with long QT syndrome in 1993 after surviving an episode of sudden cardiac death; an intra-abdominal ICD was subsequently placed. In 1996, the intraabdominal ICD was exchanged for a subclavian ICD. Three days prior to admission the patient underwent routine transthoracic echocardiography in preparation for a generator change that was scheduled for the following week. The echocardiogram revealed multiple mobile masses in the right atrium that appeared to be attached to the ICD lead. The patient was admitted to the hospital for further workup. She denied any recent difficulty breathing, chest discomfort, palpitations, cough or hemoptysis. She did admit to initiating oral contraceptive therapy 3 months prior to admission. Cardiopulmonary exam was unremarkable except for the ICD palpable below the left clavicle. Bilateral lower extremity exam revealed no swelling/edema and HomanвЂ™s sign was negative. Transesophageal echocardiogram performed on hospital day # 1 showed multiple mobile masses on the ICD lead in the right atrium, the largest measuring 1.6 cm x 1.0 cm. The septum was aneurismal and there was likely a patent foramen ovale. Left and right ventricular function appeared normal. CT pulmonary angiogram was performed on hospital day #2 and revealed multiple bilateral filling defects of the pulmonary arteries consistent with pulmonary emboli without filling defects in the lower extremities. Anticoagulation therapy with intravenous heparin was initiated on hospital day #2. Doppler ultrasound of the lower extremities and left subclavian and internal jugular vein were negative for thrombosis. Transesophageal echocardiogram was repeated on hospital day #12 and showed a decrease in thrombus size from 2.5cm2 to 2.2cm2 . The patient was discharged on hospital day #14 on oral anticoagulation therapy with coumadin. Transthoracic echocardiogram was done 1 year later, which showed complete resolution of all thrombi. The patient remained asymptomatic and is scheduled to undergo ICD generator change in the near future. Cytomegalovirus Infection Disguised as an Ulcerative Colitis Flare George M. Girgis, MD University of Southern California Introduction: Ulcerative colitis is a disease of the intestine characterized by bloody diarrhea and abdominal pain during periods of flare. On rare occasion, these symptoms can be caused by infection, subsequently misdiagnosed and treated as a flare of ulcerative colitis. Case: A 42 year old woman with a nine year history of ulcerative colitis presents to the emergency room with two weeks of progressive abdominal pain and bloody diarrhea. The pain was characterized as dull and pressurelike, radiating throughout her abdomen, and worse on the left side. She also complained of 8-10 loose bloody bowel movements daily. Other symptoms included generalized fatigue and weakness. Three weeks prior, the patient was treated for similar symptoms at an outside hospital. A flexible sigmoidoscopy at that time was consistent with significant colonic inflammation and ulceration. She was treated for an ulcerative colitis flare and discharged on prednisone, oral asacol, and steroid enemas, but the symptoms progressed. On admission the patient was afebrile, tachycardic and normotensive. She had pallor, abdominal pain in all quadrants, and gross blood on rectal examination. Laboratory values included a WBC of 9800, Hgb of 10.2 g/dL, platelet count of 502K, and an electrolyte panel within normal limits. Abdominal X-ray demonstrated colonic dilatation but no evidence of toxic megacolon. She was started on parenteral and topical steroid therapy, 5-ASA, and bowel rest. Despite aggressive treatment, her symptoms did not improve. A repeat flexible sigmoidoscopy with biopsy was performed. Biopsy results returned positive for cytomegalovirus infection and she was started on antiviral treatment with parenteral gancyclovir. The symptoms subsequently improved with antiviral therapy and the patient was discharged with Infectious Disease and Gastroenterology followup. Discussion: This case represents how CMV colitis can be mistaken for an ulcerative colitis flare. IBD patients are commonly immunosuppressed (particularly those on steroid or immunologic therapy), and therefore presumably at increased risk for opportunistic infections. Clinicians must keep a high index of suspicion for CMV superinfection in cases of IBD refractory to parenteral therapy. Sarcoid: The Textbook would be Proud! Thomas Griffiths, MD University of California, San Diego Sarcoidosis is a disease of unknown etiology, characterized by noncaseasting granulomas of affected organs. Known to most to be a cause of large hilar adenopathy, sarcoid can manifest in many other ways. A 40 year old African American male seen in a pulmonary clinic after a chest X-ray for Addiction Day Treatment Program screening demonstrated radiographic evidence of sarcoidosis. On further questioning the patient described symptoms such as yellowish green sputum in the a.m. for 3-4 years, worsening over the last year. He complained of skin lesions on his right hand and neck that were non tender and non pruritic and described a subcuticular mass the size of a grape in his left axilla. The patientвЂ™s physical exam was pertinent for findings of: stridor and diffuse wheezing bilaterally, subcuticular nodularity in the axilla, 26 non tender papular lesions that were non erythematous at the base of penis as well as similar nodules on the extensor surfaces of the handвЂ™s. The insidious nature in which the disease had progressed was objectively summarized with flow spirometery. The patient was subjectively asymptomatic of respiratory symptoms however the results demonstrated otherwise: FEV1/FVC 51.9% of predicted, consistent with severe obstructive disease. High Resolution CT demonstrated right middle lobe and right lower lobe atelectasis secondary to hilar adenopathy causing compression of bronchial tree. Sarcoidosis was confirmed by skin biopsy from a lesion on the back of the patientвЂ™s neck, which demonstrated classic non caseating granulomas associated with the disease. Treatment was initiated with a six week tapering course of high dose corticosteroids. The patientвЂ™s response to treatment was evaluated with repeat spirometry which improved dramatically after the steroid course. Unfortunately the steroid course proved too much for the patientвЂ™s already poor sugar control and he had to be started on oral therapy for type two diabetes. This case illustrates a classic presentation of a relatively common disease of which we do not fully understand. The insidious nature in which sarcoid can progress is illustrated by the patientвЂ™s subjective asymptomatic state even with his right middle and lower lobes being completely atelectatic. Further development of more specific treatment is essential if this disease is to be treated successfully without other metabolic complications of high steroid therapy. Making the Diagnosis: Paralyzed Legs and a Cardiac Mass Katherine Hartzell, BS; Dana Faw, MD; Rebecca Liddicoat Yamarik, MD, MPH University of Calinfornia, Irvine Case: A 71 y/o Filipino female presents with sudden onset severe burning abdominal pain and paraplegia of bilateral lower extremities. Her vital signs were within normal limits. PE revealed: mild epigastric pain, 0/5 strength in both lower extremities, absent DTRs, Babinskis neutral, and absent rectal tone. Brian MRI revealed one thalamic and two cerebellar lesions consistent with acute infarcts, and T-spine MRI revealed an enhancing T2 signal abnormality from T8 to T11. The patientвЂ™s presentation was consistent with anterior cord syndrome; differential diagnosis was transverse myelitis with unclear etiology vs. cord infarction vs. an autoimmune process. The patient was initially treated with high dose steroids and IVIG for 5 days, without change in her symptoms. A TTE was normal, but a TEE revealed a 1.1cm mass on the mitral valve. The differential included infective endocarditis manifesting with embolic spinal cord infarction and embolic strokes. The patient was started on antibiotics. Blood cultures, however, were negative x 5 and the patient remained afebrile. The location of the embolic events in the brain and spinal cord were not typical for emboli originating from the heart. Other etiologies were considered, including a Rheumatologic disease or a paraneoplastic process from a possible cardiac tumor. ESR and CRP were normal on admission, but rose to 67 and 18.5 respectively, on day 12. Lupus anticoagulant (antiHex) was positive, and a diagnosis of antiphospholipid antibody syndrome was entertained. However, the test was drawn when the patient had a supratherapeutic PTT secondary to a sensitivity to subcutaneous Heparin and was thought to be falsely positive. Temporal artery biopsy was also negative, making vasculitis or other Rheumatologic problem unlikely. Cardiac MRI demonstrated a benign calcification, an unlikely source of emboli. Repeat MRI of the T spine showed expansion of cord edema consistent with evolution of a previous infarction. Her history of PVD, and evidence of arterial calcification noted on previous CT made a diagnosis of anterior spinal cord infarction secondary to diffuse atherosclerotic disease the most likely cause of her symptoms of paralysis, with simultaneous CVAs due to PVD seen on brain MRI. Discussion: This case demonstrates the wide variety of disease processes that can cause nontraumatic spinal cord injury, how each of these different processes may manifest, how these conditions are worked up and the importance of keeping a wide differential in order to ultimately arrive at the correct diagnosis. An Incidental Finding of Metformin Associated Lactic Acidosis Aimee Hechanova MD; Maher Roman, MD, MBA Loma Linda University Case: A 76 year-old diabetic male came to clinic to establish care and get medication refills. Physical exam was unremarkable and the patient appeared well, though on review of systems endorsed fatigue, nausea, decreased appetite, and weakness. Per patient, outside labs drawn 3 weeks prior were all normal. He had also gotten a CT head with contrast one week prior and had resumed metformin immediately afterwards. New baseline labs were drawn, showing a creatinine of 1.8 and a wide anion gap acidosis (Na 142, Cl 100, CO2 19.4, AG 22). After reviewing the labs the following day, the patient was contacted immediately, told to stop metformin and come to the emergency department for further evaluation. Repeat BMP showed an improvement in renal function (Cr 1.4, Na 141, Cl 99, CO2 22.8, AG 19), and lactic acid 3.7. The patient received intravenous fluids, and the next day was feeling much better with improved mentation, decreased fatigue, and a good appetite. Repeat labs showed resolution of the patientвЂ™s contrast-induced acute kidney injury, resolution of the anion gap acidosis and normalized lactic acid levels (Cr 1.2, Na 140, Cl 107, CO2 23.0, Lactic acid 0.8). Discussion: Metformin associated lactic acidosis (MALA) is a very rare complication of metformin (3 cases per 100,000) which is crucial to detect because it carries a mortality rate of 48%. It most frequently occurs 27 after contrast imaging due to contrast induced acute kidney injury. The acute kidney injury allows metformin, which is renally excreted, to accumulate and cause lactic acid buildup. This case demonstrates the importance of having a high index of suspicion for MALA in patients on metformin with an acute impairment of renal function and an anion gap acidosis. It also underscores the importance of holding metformin immediately prior to any contrast study and checking the creatinine 2-3 days afterwards to ensure normal renal function before restarting metformin. vitamin A. The patient discontinued all nutritional supplementation while in hospital and was discharged home with the diagnosis of vitamin A induced intrahepatic cholestasis. At 2 months free of nutritional supplementation the patientвЂ™s bilirubin and alkaline phosphatase levels returned to normal. A review of the literature revealed one other case of vitamin A toxicity from long term consumption of вЂњtherapeuticвЂќ vitamin A supplementation. In intrahepatic cholestasis vitamin A toxicity should be considered even with long term low dose consumption of vitamin A. Vitamin A Toxicity from Long Term HerbalifeВ® Dietary Supplement Use Pheochromocytoma Presenting as Acute Congestive Heart Failure and Acute Renal Failure Gary Hensley, MD; Bim Pham St. Mary Medical Center Andrew Ho, MD; Run Yu, MD, PhD; Amanda Ewing, MD Cedars-Sinai Medical Center Nutritional supplementation has steadily increased over the past 20 years, however supplement use is not without risk. Vitamin A induced liver disease has been recognized for over 100 years. Liver hepatotoxicity as a result from low вЂњtherapeuticвЂќ doses of vitamin A has not been considered clinically significant. Here we present a case of intrahepatic cholestasis from prolonged ingestion of vitamin A from HerbalifeВ® dietary supplements. A 46year-old male presented to our ED with complaints of worsening jaundice and pruritis for 2 weeks. Upon admission to our ED the patient had normal vital signs; physical exam was significant for scleral icterus, jaundice, palpable liver at 2cm below the costal margin, and negative hepatitis and autoimmune serologies. An abdominal ultrasound showed a common bile duct of 4.7mm, no evidence of gallstones, no gallbladder wall thickening, and no intrahepatic biliary dilation. Initial liver serologies revealed a total bilirubin 10 times the upper limit of normal, a direct bilirubin 21 times the upper limit of normal, and an alkaline phosphatase 1.5 times the upper limit of normal. AST and ALT values were within normal limits. Total and direct bilirubin and alkaline phosphatase continued to increase daily and on hospital day 4 an ERCP was performed and a biliary stent was placed. A second ERCP was performed on hospital day 8, due to continued increasing bilirubin and alkaline phosphatase levels, which demonstrated a biliary stent that had good bile and sludge flow. Yet the stent was removed and replaced. On hospital day 13, a liver biopsy was performed for increasing total bilirubin 20 times the upper limit of normal, direct bilirubin 40 times the upper limit of normal and alkaline phosphatase 2.2 times the upper limit of normal despite 2 normal ERCPs. Pathology revealed prominent centrilobular cholestasis with activated stellate cells with increased vacuoles, intracellular necrosis with apoptotic bodies observed in KupfferвЂ™s cells, dilated bile ducts, and bile thrombi. Based upon these results a more detailed history was obtained and the patient had been consuming a HerbalifeВ® Healthy Meal Nutritional Shake and two HerbalifeВ® Multivitamin Complex pills daily for the past 12 years. This nutritional supplementation amounts to approximately 5000IU of vitamin A which is also the recommended daily dose of A 41-year-old male with a history of hypertension on no medications, presented to the emergency room complaining of one week of shortness of breath with exertion. Patient also noticed bilateral lower extremity swelling with a 15 pound weight gain. Pt denied chest pain, orthopnea, sick contacts, fevers, night sweats, or recent travel. Pt did report intractable headaches, exacerbated by occasional episodes of flushing over the past few years. While in the ER, patient's vitals were 98ЛљF, 98 beats/min, blood pressure 201/132 and O2 saturation of 99%. Physical exam revealed an obese man, with elevated jugular venous pressure, bilateral crackles at the lung bases with dullness to percussion on the right side. Patient's heart was tachycardic but regular, with bilateral lower extremity pitting edema. Labs revealed Cre 1.4, hemoglobin 11.9, BNP 478, troponin 0.03. EKG showed sinus tachycardia, poor R wave progression, low voltage and QTc472. A chest xray showed congestive heart failure (CHF) and a right pleural effusion. CT chest with contrast was performed because of suspicion for PE and was negative, but bilateral adrenal masses were incidentally found measuring 7cm in the left and 3cm in the right. Transthoracic echocardiogram revealed an ejection fraction of 55 to 60%, left atrium and ventricle dilation, moderate to severe mitral regurgitation, and diastolic dysfunction. Management of patientвЂ™s heart failure included diuresis and blood pressure control. Attention was given to not use beta blockers while waiting lab results given a high suspicion for pheochromocytoma. The patientвЂ™s clinical condition improved. Laboratories confirmed pheochromocytoma with urinary catecholamine metabolites markedly elevated 28 (metanephrine level of 12,600 Ојg/day; normetanephrine level of 5040 Ојg/day), and the fractionated plasma free metanephrines elevated at 22.3 and 17.9 for metanephrine and normetanephrines respectively. sis, fibrosing interstitial pneumonitis, and perivascular amyloid deposits consistent with amyloidosis. Tests for other sites of amyloid involvement, including fat pad biopsy, echocardiogram, and urine studies, were normal. Adrenal pheochromocytomas are neuroendocrine tumors that secrete high levels of both epinephrine and norepinephrine. Patients with a pheochromocytoma classically present with the triad of headaches, palpitations, and diaphoresis. However, patients can also present either asymptomatically or with chest pain, shortness of breath, flushing, or anxiety. It is also known that pheochromocytomas can present with refractory hypertension. It is unusual however, for a patient with a pheochromocytoma to present in acute decompensated CHF requiring hospitalization like our patient. Some have suggested that patients with an unknown etiology of CHF be screened for pheochromocytoma, but as of current practice, this is not widely done. In a patient with CHF where there is a suspicion of pheochromocytoma, caution should be used in initiating beta blockers in the treatment regimen to avoid unopposed alpha-adrenergic receptor stimulation and further blood pressure increases. In summary, we present a young patient with no history of previous myocardial damage, who was in florid CHF, found subsequently to have pheochromocytoma. The patientвЂ™s pulmonary symptoms improved with intravenous ceftriaxone and azithromycin. Her arthralgias were treated initially with ibuprofen, but after the severity worsened, she was given intravenous methylprednisolone. At discharge, she was given prednisone, azathioprine, and prophylactic trimethoprim-sulfamethoxazole. Not Your Average Pneumonia: Primary Pulmonary Amyloidosis Siwen Hu-Lieskovan, MD, PhD; Taib Rawi, MD; Andrew Hendifa, MD; Samina Ahmed, MD; Aarti Chawla, MD; Jose Gonzales, MD; Emanuel Voyiaziakis, MD; Howard Liebman, MD; Bharat Chaudry, MD George Holman, MD University of Southern California Case: A 61 year-old woman with prior history of trigeminal neuralgia was brought to our hospital after being treated for presumed pneumonia at another clinic. Two months prior to admission, she had acute onset of a dry cough and pleuritic chest pain. She went to her clinic after the symptoms persisted for four weeks and was initially given a week-long course of an unknown antibiotic. She improved while taking the antibiotic, but her symptoms returned after completing the regimen. She also developed fevers and severe arthralgias, mostly in her wrists and left hand. She returned to the same clinic and was given a different antibiotic for another week. Again, she improved while taking the antibiotic, but her symptoms returned after finishing it. On physical exam, she was found to be afebrile, tachypneic, and had an oxygen saturation of 94% on room air. She had bibasilar lung crackles. There was tenderness and swelling in both wrists and the joints of her left hand, but no erythema or warmth. Results of routine lab studies were normal except for albumin 3.1g/dL and magnesium 1.5mg/dL. ESR was 67mm/Hr and high-sensitivity CRP was 68mg/L. Later, she was found to have positive ANA, rheumatoid factor, pANCA, and anti-myeloperoxidase antibody. The chest x-ray showed a reticulonodular pattern in the lung bases, so a high-resolution CT was performed. It found idiopathic pulmonary fibrosis. A bronchoscopy was performed and found peribronchial fibro- Discussion: This patientвЂ™s presentation was unusual for amyloidosis because she was acutely symptomatic and amyloid was only found in the lungs. In general, isolated pulmonary amyloidosis is seen as a lung nodule and caused by abnormal B cell proliferation. Diffuse pulmonary amyloidosis is associated with systemic involvement and predominantly affects the kidneys and other organs before the lungs. As was done in this case, the diagnosis of amyloidosis requires further studies to determine the extent of the disease and to rule out inflammation, infection, neoplasm, or genetic disease as secondary causes. A Case of Warm-Antibody Autoimmune Hemolytic Anemia without Spherocytosis University of Southern California, Dept. of Medicine/Hematology Autoimmune disorders are often associated with secondary autoimmune hemolytic anemia (AIHA), mostly warm antibody (Ab) AIHA. Patients with this variant present with varying degrees of anemia, jaundice and hepatosplenomegaly, with lab values showing increased LDH, indirect bilirubinemia, decreased haptoglobin & reticulocytosis. One diagnostic hallmark of warm Ab AIHA (WAHA) is spherocytes in the peripheral smear, due to splenic sequestration and monocyte/macrophagemediated partial destruction of Ab-attached RBCs. We report a case of WAHA without spherocytosis. Patient is a 61 year-old Hispanic woman with hypothyroidism treated with Synthyroid for 3 years and interstitial cystitis treated with Elmiron for 2 years, who presented 2-months of fatigue, dizziness & jaundice. Hb was 4.5 gm%, with increased LDH (456), indirect bilirubin (3.5) and decreased haptoglobin (<20). Elmiron was held. Type & cross showed the patient was (+) for indirect CoombвЂ™s test with non-specific reactivity (NSR) but (-) for direct antiglobulin test (DAT). Patient denied past transfusions/pregnancies. Peripheral smear showed reticulocytosis but no spherocytes. Two units of matched pRBCs were transfused twice during the hospital stay. The patient initially responded, but in both instances Hb dropped over the next 3 days. Cold hemagglutinin test 29 was (-). Patient was also found to have autoimmune hepatitis with (+) titers for ANA & anti-smooth muscle Ab & elevated AST/ALT. Abdominal US showed cirrhosis & enlarged spleen (13cm). CoombвЂ™s-(-) hemolytic anemia work-up demonstrated a (+) DAT due to IgG (warm autoantibody of broad specificity) & complement-coated red cells. The patient's indirect antiglobulin test was also (+) with NSR. She responded well to Prednisone 50 mg po daily, with stabilized Hb. This patient has a classic presentation of secondary AIHA due to autoimmune disease with (+) warm antibodies on RBCs & in the peripheral blood but without peripheral spherocytes. One possible explanation is complete splenic sequestration of Abcoated RBCs by macrophages circulated out of the spleen. DAT-negative non-spherocytic AIHA is a known entity but DAT-positive non-spherocytic AIHA has rarely been reported in the literature (Pubmed search, 9/09). This case demonstrates that the diagnosis of AIHA can not be excluded in the absence of spherocytosis. Indinavir-Induced Nephrolithiasis after Cessation of Indinavir Therapy Julie Huynh, MD; John J. Sim, MD; and Aviv Hever, MD Kaiser Permanente Southern California Indinavir sulfate is a protease inhibitor used in the treatment of HIV by inhibiting the cleavage of precursor polyproteins into functional infectious proteins. Indinavir-induced nephrolithiasis is a well reported side effect of the medication; however, there is little literature regarding the occurrence of indinavir-induced nephrolithiasis after cessation of therapy. We present a patient with confirmed indinavir-induced nephrolithiasis 3.5 years after the cessation of indinavir therapy. A 49-year-old man with HIV was treated with a HAART regimen that included indinavir. After eight years of therapy, the patient presented with signs and symptoms of nephrolithiasis and indinavir had to be discontinued. A CT KUB at that time demonstrated mild bilateral hydronephrosis and right hydroureter consistent with obstruction although no radioopaque calculi were seen. The patientвЂ™s symptoms resolved with the discontinuation of indinavir and conservative management. A follow up renal furosemide scan performed showed no evidence of stone or obstruction. More than three years after the patient discontinued indinavir therapy, the patient again presented with gross hematuria and right flank pain. A CT KUB showed complete resolution of the previously mentioned left hydronephrosis. However, there was moderate dilatation of the right intrarenal collecting system and right ureter, again with no evidence of calculi. The patient eventually passed multiple stones with resolution of symptoms. Stone composition analysis revealed indinavir. This case shows that indinavir-induced nephrolithiasis can present many years after cessation of therapy. Indi- navir stones are not easily detectable because they are radiolucent and not identifiable on CT. In addition, because of their gelatinous consistency, indinavir stones can cause high grade obstruction and are not amenable to lithotripsy. Although conservative management may be effective, some cases may require ureteral stenting. Therefore, there must be a high clinical suspicion of indinavir-induced nephrolithiasis in patients with symptoms of flank pain or hematuria who have a history of indinavir use, no matter how remote. Lemierre's Syndrome - Forget Me Not Ngoezi Iroezi, MD; Jenny Shen, MD; Neveen El Farra, MD University of California, Los Angeles Introduction: This case displays the presentation and clinical features of LemierreвЂ™s syndrome & aids in broadening the differential for a young adult presenting with non-resolving fevers/pharyngitis. Case description: 19 year old male presented to the hospital with 2 weeks of persistent high fevers and nonresolving sore throat. In the week prior to admission, he was examined twice and found to have no lymphadenopathy or tonsillar exudates. He also had 2 (-) strep throat cultures and a (-) Monospot. He represented to the ER and in the interim had developed left upper quadrant abdominal pain with pleurisy, as well as numbness under his right jaw. Review of systems was notable for a mild HA, the absence of cough, and no signs of meningismus. He denied intravenous drug use or unprotected sexual contact. Physical exam was notable for a temperature of 38.7 Celsius, pulse of 126, BP 142/51, respiratory rate 20, & 95% oxygen saturation on room air. He had submandibular lymaphadenopathy, an erythematous posterior pharynx with no ulcerations and exudates, crackles at bilateral lung bases, and splenomegaly. Labs showed a mild leukocytosis, a positive Epstein Bar Virus EBNA antibody, indicating an infection of mononucleosis at least 6 weeks old and a negative rapid HIV. A CT of the chest revealed findings consistent with septic emboli. Echocardiogram showed no vegetations. An MRI of the neck, however, revealed an abscess in the medial margin of the right mandibular body. Blood cultures grew Fusobacterium Necrophorum. The patient clinically improved on meropenem and metronidazole finishing a 14 day course upon discharge. Discussion: LemierreвЂ™s syndrome involves a septic thrombophlebitis of the internal jugular vein caused by Fusobacterium necrophorum. F. necrophorum is an anerobic gram rod, a normal flora of the oral cavity, gastrointestinal and female genital tract. The pathogenesis is characterized by hematogenous spread from a peritonsilar abscess to the jugular vein. Classic signs and symptoms include neck pain and swelling, septic emboli to the lungs, fever, leukocytosis, and thrombocytopenia. It has been hypothesized that immunosuppresion from a preceeding infection such as mononucleosis may predispose 30 individuals to LemierreвЂ™s syndrome. It may lead to lifethreatening erosion of the vein or invasion into the adjacent carotid artery. It can be easily treated with antibiotics if caught early. It is known to occur in immunocompetent young adults. Thus a thorough evaluation of a nonresolving pharyngitis that includes an infectious workup and appropriate imaging will preclude missing the almost forgotten syndrome- Lemierre's. Non-Hodgkin Lymphoma Presenting as Pleural Effusion with Dermatomyositis Farokh Jamalyaria, MD; Michelle Phillips, MD; Enoch J. Wang, MD Scripps Green Hospital Introduction: An 84-year-old man presented with a three-week history of progressive weakness in his shoulders and hips and a progressively nonproductive cough. He complained of difficulty brushing his hair, unsteadiness on his feet, and had suffered a mechanical fall. Over the six months prior to admission, he had unintentionally lost 30 pounds. He denied pleuritic pain, fever, chills, rigors, night sweats, lymphadenopathy, or chest pain. He had a three-month history of the following: erythema and exfoliation over the forehead consistent with erythroderma, heliotrope rash under the eyes, poikilodermatous macules over the nape and shoulders consistent with a positive shawl sign, patchy redness over the knuckles bilaterally consistent with GottronвЂ™s sign, and periungual erythema over the right fifth digit. His left lung had markedly decreased breath sounds throughout, with dullness to percussion and decreased tactile fremitus, but without egophony. The patient had coronary artery disease and a smoking history of occasional cigar and pipe use over approximately twenty years, having quit ten years prior to admission. Case: Based upon the history and physical findings, a diagnosis of dermatomyositis was strongly suspected. Chest x-ray revealed a large left-sided pleural effusion. A diagnostic and therapeutic thoracentesis was performed; pleural fluid analysis revealed an exudative picture: cloudy yellow color, glucose less than 1 mg/dL, protein 4 g/dL, LDH 3983 U/L, WBC 24800/cumm, RBC 30000/cumm, 200 cells counted (1% segs, 5% lymphocytes, 94% mononuclear cells). A comprehensive rheumatologic panel was positive only for ANA panel with ANA Hep-2 cell titer of >= 640. The following rheumatologic labs were negative: anti-DS DNA, anti-Jo-1, Smith, RNP, SSA, SSB, Anti-Scl-70, and rheumatoid factor. Erythrocyte sedimentation rate, uric acid, Creactive protein, and peripheral LDH were elevated at 40 mm/hr [0-30], 14.9 mg/dL [3.5-7.2], > 90 mg/dL [0-10], and 484 units/L [100-190], respectively. UPEP revealed albuminuria; no monoclonal proteins identified. SPEP revealed hypoalbuminemia without monoclonal proteins. Pleural fluid cultures and blood cultures were negative. HIV antibody screen was negative. Flow cytometry of the pleural fluid revealed an aggressive non-Hodgkin lymphoma. CT of the chest, abdomen, and pelvis for staging was significant for no lymphadenopathy. Discussion: Non-Hodgkin lymphomas (NHLs) are a diverse group of lymphoproliferative disorders. Up to forty percent of patients with NHL present with вЂњBвЂќ symptoms (fever, weight loss, and night sweats). About 20% present with mediastinal lymphadenopathy, and abdominal / pelvic lymphadenopathy is common in most subtypes. Dermatomyositis is a known paraneoplastic syndrome: approximately 15% of patients with dermatomyositis have a neoplasm. Roughly 10% of patients with NHL present with pleural disease. However, it is very unusual for NHL to present primarily as a pleural effusion in an HIVnegative patient. It is more common in HIV-positive patients (HIV-associated pleural effusion lymphoma). Zollinger-Ellison Syndrome Aaron Jeng, MD University of Southern California Introduction: Zollinger-Ellison syndrome is characterized by hypersecretion of gastric acid, high serum gastrin concentrations, and non-beta islet cell tumors of the pancreas. It was initially estimated that the incidence in the United States ranged from 0.1 to 1 percent of patients with peptic ulcer disease. Gastrinomas are derived from multipotential stem cells of endodermal origin. Known as enteroendocrine cells, they arise mainly in the pancreas as well as the small intestine. Case Presentation: A 60 year-old woman presented with the chief complaint of coffee ground emesis. Pt endorses 11 months of inability to retain ingested food. Gradually during the course of her disease, pt symptoms worsened and was seen in the ER multiple times. ER visits consisted of treatment with fluids, antibiotics, and PPI. Pt finally admitted 1 month prior to admission for dysphagia. During ptвЂ™s first admission, EGD done and pt was found to have multiple esophageal and duodenal ulcers. Pt subsequently sent home with PPI therapy and GI cocktail. Pt now returns with 2 day history of coffee ground emesis. Pt exam is significant for conjunctivial palor and epigastric tenderness, otherwise no other findings. Labs were significant for H/H of 5.3/ 15.6, gastrin level of 886 pg/ml, and chromogranin level of 341.2. Imaging studies that were done include small bowel follow through with findings concerning for intestinal disease and a octreotide scan with findings consistent with gastrinoma. Pt also had multiple EGDs with multiple esopheal and duodenal ulcers visualized and an EUS attempted but failed because of a stricture. Pt subsequently diagnosed with gastrinoma and Zollinger-Ellison syndrome and started on PPI gtt and converted to PO PPI. Surgical service was consulted and decision was to schedule pt for outpatient surgical intervention. Discussion: Zollinger-Ellison syndrome, unlike typical peptic ulcer disease, is often progressive and persistent and can frequently be life-threatening if not diagnosed or treated appropriately. With current assays for serum gastrin and current imaging, the number of individuals that 31 are diagnosed with the Zollinger-Ellison syndrome have increased and the diagnosis is often established before development of the complications of peptic ulcer disease or spread of a malignant gastrinoma. Additionally, patient are given appropriate therapy that alters the course of the disease. cal history was significant only for Diabetes Mellitus type II and hyperlipidemia. Diagnostic paracentesis revealed a SAAG of 0.8 and atypical cells consistent with adenocarcinoma. In conjunction with CT findings and omental biopsy, the diagnosis of metastatic pancreatic carcinoma was made. ItвЂ™s an Ulcer, an Outpouching, No a Double Pylorus! Discussion: Ascites is a harbinger of the final stages of pancreatic cancer. The incidence of clinically detected ascites in pancreatic carcinoma is reported to be between 15.6% and 38.7% [10]. It occurs relatively infrequently as many patients do not survive long enough to manifest with it. Carcinomas of the body and tail of the pancreas are more likely to induce ascites rather than those of the head of the pancreas. Nonetheless, the cases of pancreatic carcinoma with ascites as the initial sign seem to be extremely rare; a mere handful of case reports have described this presentation in the past [10, 12]. Radha Juneja, MD; Eric Choi Scripps Green Hospital A double pylorus is an accessory pyloric channel that can be classified as congenital or acquired. The most common cause is a complication of an ulcer that leads to intramural erosion between the lesser curvature of the stomach and duodenal bulb. This case provides a possible interesting alternative to the etiology of this rare abnormality. A 76-year-old man presented with progressively worsening RUQ pain that began 2 days prior to admission after eating a large meal. The pain was constant, non-radiating, sharp, and 8/10 in intensity. He denied associated symptoms. He attempted to relieve his symptoms with TUMS, and positional changes without avail. His PMH was significant for osteoarthritis treated with diclofenac twice daily for 15 years. Abdominal exam revealed a soft, non-distended abdomen, tender in the right lower quadrant. The patientвЂ™s abdominal CT revealed inflammation stranding along the second portion of the duodenum. A 2.4 cm round posterior outpoutching was amidst this inflammation, suggesting the possibility of duodenal diverticulitis. The patient was admitted and treated with ciprofloxacin and flagyl. Additionally, the patient was started on a proton pump inhibitor and advised to stop taking diclofinac. The symptoms resolved within 48 hours and the patient was discharged in stable condition. Three weeks later, he underwent a follow up upper endoscopy. Gastric and duodenal mucosa showed no evidence of erythema or mucosal erosion. Insufflation of the stomach revealed 2 separate channels from the gastric lumen into the duodenal bulb. The endoscope could be passed through both ducts, and the findings were verified by infusing water through the ducts and into the lumen of the duodenum. This case suggests the possibility that a double pylorus may be acquired secondary to duodenal diverticulitis leading to inflammation, erosion, and intramural fusion. However, we cannot be sure that this case, like the majority of other cases of double pylorus, was not a complication of peptic ulcer disease. Although, there was no evidence of gastric ulcers on endoscopy, the patientвЂ™s long history of chronic NSAID use also makes this etiology quite possible. New Onset Ascites Resulting in Diagnosis of Metastatic Pancreatic Carcinoma Mary Kamel, MD Ceadrs-Sinai Medical Center Lost to Follow-up: Acute Paralysis and Cyanosis Sarah Kemble, MD; Jamil Aboulhosn, MD University of California, Los Angeles Case: A 20-year-old male developed sudden onset of confusion, slurred speech, and left-sided weakness. On arrival of paramedics, he was also noted to have perioral cyanosis. His mother reported he had always been blue, and had undergone heart surgery in childhood. He had been followed by a pediatric cardiologist, but at age seventeen, his father lost his job, and he lost the health insurance provided under his fatherвЂ™s plan. He continued to take aspirin, and seemed to be doing well, working full time and playing pick-up football on the weekends. However, over the last month, he had become increasingly short of breath with exertion. He had worsening cyanosis and was unable to work due to fatigue. His mother had also noticed snoring at night and pauses in his breathing which sometimes caused him to awaken from sleep. In the Emergency Department, a CT scan of the head showed no intracranial bleed. Oxygen saturation was 80%. Tissue plasminogen activator was administered, with some improvement in speech and strength. An MRI of the brain showed a right middle cerebral artery infarct. A bedside echocardiogram demonstrated a single right ventricle, D-transposition of the great arteries with likely Stansel anastamosis, hypoplastic left ventricle, mitral atresia, lateral tunnel Fontan with fenestration and right to left shunting, non-obstructive ASD, and non-restrictive VSD. Right ventricular ejection fraction was reduced at 20%. Cardiac catheterization was performed, and the patient underwent Amplatzer device closure of the Fontan fenestration. Significant innominate to coronary sinus venous collateral flow was noted and treated with coil embolization. The patient demonstrated improved oxygenation following these procedures, and was started on warfarin anticoagulation and afterload reduction. He was discharged to an acute rehabilitation facility for further functional recovery following his stroke. Case: 64 year old male was transferred to our facility due to new onset ascites of unknown etiology. His past medi- 32 Discussion: After Fontan repair, patients are at higher risk for thrombosis and arrhythmias, particularly atrial fibrillation. Fenestrations in the Fontan conduit, as well as arteriovenous collaterals or malformations which develop as a result of high Fontan pressures can cause shunting, chronic hypoxia, and predispose to embolic stroke. Anticoagulation may be considered routinely post-Fontan to prevent thrombus formation and reduce stroke risk. Survivors of congenital heart surgery must undergo routine follow-up, and should be screened for comorbid conditions that affect the hemodynamics of their repair. In this case, obstructive sleep apnea may have contributed to increased Fontan pressures, further predisposing to thrombosis and Fontan failure. As more adolescents age out of health insurance coverage with no affordable alternatives, inadequate access to care has become a critical comorbidity for survivors of chronic diseases of childhood. De Novo Epitheloid Angiosarcoma of the Urinary Bladder Michael Keng, MD; Aashini Master, DO University of Southern California Background: Angiosarcoma of the bladder is exceedingly rare and often fatal. Only 12 cases of primary angiosarcoma have been documented in the literature, and even fewer de novo cases reported. The following case presentation is a patient with de novo epithelioid angiosarcoma of the urinary bladder. Case Report: The patient was a 60-year-old Caucasian man with coronary artery disease, hypertension, and right knee arthritis, who presented with bilateral leg pain for nine months, worsening over the last two months. He also admitted to occasional fever, sore throat, and dysuria. He had recently visited Panama in May 2008. After returning, he developed right foot pain and lesions and bilateral lower extremity rash. Leg x-rays were normal. Biopsies of the thigh and feet revealed keratosis and leukocytoclastic angitis and keratin debris, respectively. The patientвЂ™s feet remained painful, and sudden onset of left foot numbness and cyanosis prompted hospitalization. Social history was remarkable for alcohol use, 20-packyear tobacco history, and remote history of heavy LSD use. He worked in the construction industry and had a history of unprotected sex. Initial physical examination revealed normal vital signs, bilateral leg edema up to the mid calf, and white-brown verrucous lesions on the plantar surface of the right foot. The toes of the left foot were cold, purple and insensate with weeping, scattered bullous lesions, necrosis and purple discoloration. Significant serological tests revealed: white blood count 17.3K/cumm, LDH 164units/L, ESR 112mm/hr, CRP 260.9mg/dL, and positive lupus anticoagulant. All other routine, infectious, and rheumatologic serologies were negative. Leg x-rays showed multiple lesions of both femurs with fracture of the right femur. MRI of the left foot showed dry gangrene and right femur showed a pathologic subtrochanteric fracture suspicious for telangiectatic osteosarcoma. CT scan showed scattered nodules in the lungs, lytic lesions of the right femur, multiple lytic lesions of the pelvis. Biopsy of the right femur revealed epithelioid angiosarcoma. Ultrasound showed a thrombus in the right superficial femoral vein. While in the hospital, he developed gross hematuria, which was thought to be secondary to anticoagulation. The patient received bilateral below-the-knee amputations for progressive gangrene. Unfortunately, shortly after, he expired from cardiopulmonary arrest. Postmortem examination revealed epithelioid angiosarcoma of the urinary bladder with metastases to the lungs, skin, prostate, scrotum, and bone. Additionally, three pulmonary emboli were noted. Conclusion: Angiosarcomas are rare mesenchymal neoplasms that most often involve the skin and less commonly the deep tissues. The head and neck region is the most common site of angiosarcomas, but reports have indicated the presence of tumor throughout the body, including the heart, thyroid, breast, ovary, retroperitoneum, and other sites. Angiosarcoma of the bladder is exceedingly rare and epithelioid appearance has been documented previously in only two cases. Hyperfibrinolytic Activity in Patients with Liver Cirrhosis Michael Keng, MD; Yee Lu, MD; Matthias Barden; Eric Hsieh, MD University of Southern California Background: Hyperfibrinolytic activity is a poorly understood complication of liver dysfunction, partially due to the diverse causes of liver disease, clinical presentations, and laboratory variability amongst studies. Patients have increased bleeding risks, such as soft tissue hematoma, intracranial bleed after trauma, esophageal variceal bleeding, and transfusion requirements in the perioperative period for transplant patients. All of which are associated with increased morbidity and mortality. While hyperfibrinolytic activity is present in thirty-percent of patients with end stage liver disease, it is still clinically under-diagnosed and usually diagnosed only after patients develop severe bleeding complications. This presentation is a review of three patients who developed the complication and the available data for their prognosis and treatment. Case Series: Case I: Fifty-year-old Mexican gentleman with alcoholic cirrhosis (Child-Pugh-Class-C/MELD-26), current alcohol abuse, and hypertension presented with acute decompensated cirrhosis with fluid retention (due to spontane- 33 ous bacterial peritonitis). Incidentally, patient was anemic with decreased fibrinogen and platelets and elevated DDimer and INR. Hematology was consulted and disseminated intravascular coagulopathy was ruled out due to a normal Factor-VIII level. Laboratory evaluation was consistent with hyperfibrinolytic activity and the patient was subsequently started on chronic epsilon-aminocaproic acid (EACA). Case II: Forty-nine-year-old Mexican gentleman with alcoholic cirrhosis (Child-Pugh-Class-C/MELD-34) and portal hypertension and history of alcohol hepatitis presented with bilateral lower extremity weakness and numbness. Admission labs revealed hyperfibrinolytic activity. Patient was started on EACA. Unfortunately, the patient developed a spontaneous retroperitoneal bleed and passed away. Case III: Fifty-year-old African-American lady with alcoholic cirrhosis (Child-Pugh-Class-B/MELD-21) presented with two-month history of prolonged epistaxis, three-week history of increasing ecchymosis without trauma of the right upper extremity, and two-week history of right wrist drop. Physical exam was consistent with radial nerve palsy secondary to extensive ecchymosis and hematoma. All laboratory results indicated an hyperfibrinolytic activity without evidence of acute decompensated cirrhosis. Conclusion Patients with cirrhosis who develop hyperfibrinolytic activity have worse morbidity and mortality. However, this conclusion is confounded by the association with higher Child-Pugh scores. More studies are needed to assess whether patients with early stage cirrhosis, as classified by Child-Pugh and MELD scores, should be evaluated routinely for this coagulopathy before developing a clinical complication. This would allow physicians to intervene earlier with treatments to prevent bleeding events known to be associated with worse outcomes. Current literature only supports the use of antifibrinolytic agents once patients fail conventional treatment for bleeding complications from hyperfibrinolytic activity. There is no data for treating this coagulopathy prior to clinically significant presentation or when patients are non-transplant candidates. Given the impact on patient survival, this disorder should be considered as an independent predictor of hepatic dysfunction and more studies are warranted to evaluate prophylaxis treatment. Inflammatory Bowel Disease in the Setting of Ankylosing Spondylitis Michael Keng, MD; Seint Yee, MD; Meera Malhotra, MD University of Southern California Background: Ankylosing spondylitis (AS) is a spondyloarthropathy, characterized by sacroillitis and chronic inflammatory lower back pain. The axial skeleton is mainly affected, but it is commonly associated with peripheral, asymmetric arthritis, enthesitis, and acute anterior uveitis. Inflammatory bowel disease (IBD), is a lesser known, but significant extra-articular manifestation of AS. While only occurring between 5 and 10% of AS cases, subclinical inflammation of the gastrointestinal tract is much more prevalent (25-60%). Case Presentation: J.G. is a 36-year-old Hispanic male who presented with an exacerbation of AS. Onset of his symptoms started four years prior to admission after a motor vehicle accident. Since then, the patient never had complete resolution of his low back pain and he was clinically diagnosed with AS. His pain is located in the lower thoracic and lumbar regions and is accompanied by right leg radiculopathy. His symptoms have been progressive and unresponsive to all conservative measures. A review of systems revealed concurrent tenesmus and weekly intermittent diarrhea, but no fevers, chills, melena, or hematochezia. Significant findings on physical exam included limited cervical spine extension, tenderness along the lower thoracic and sacral spinous processes, bilateral hip tenderness with decreased range of motion, positive WrightSchober test, and positive straight leg test on the right side. Neurologic exam was otherwise unremarkable. An ano-in-fistula was found on rectal examination. Significant laboratory findings included: hemoglobin 9.5g/dL, hematocrit 30.2%, platelets 729K/cumm, CRP 93.7mg/dL, ESR 100mm/hr, and RF 20IU/mL. Iron studies corresponded to anemia of chronic inflammation. Anti-smith antibody, RNP antibody, ANA, ds-DNA, HIV, and HLA-B27 were all negative. A MRI revealed sacroillitis, confirming the diagnosis of AS. It further showed bilateral hip arthritis and a moderate right hip effusion. The aspirate was negative for infection. High-dose indomethacin and steroids were initiated with improvement of back pain, though with concomitant worsening of rectal pain, requiring a seton placement. A colonoscopy with biopsy was consistent with CrohnвЂ™s disease. Conclusion: IBD is an important extra-articular manifestation of AS. The internist should be aware of this significant association during the diagnosis and work-up of spondyloarthopathies, as there are important implications for treatment when the diagnoses occur together. There is evidence of subclinical microscopic inflammation of the GI tract in up to 60% of AS patients, of which a fraction evolved into actual IBD over time. Furthermore, remission of joint inflammation is always linked to reduction of gut inflammation. Case series indicate that there is an additional decline in quality of life and overall worse prognosis due to the extra-articular burden of disease. Modification of treatment, namely avoiding non-steroidal anti-inflammatory medications, is paramount to avoid further injury to already inflamed bowel. Treatment should target the underlying inflammatory processes of both the AS and extra-articular symptoms. 34 Renal Failure Associated With Silicone-Induced Granulomas nificant history as an uncommon but serious cause of non-parathyroid hormone mediated hypercalcemia. Omer Khan, MD; John Sim, MD Kaiser Permanente Los Angeles Fabry Disease in a Young Active Duty Male with Asymptomatic Proteinuria The use of liquid silicone for soft tissue augmentation has been in practice over the last 40 years. Initially thought to be inert, liquid silicone was extensively used for cosmetic subcutaneous augmentation. With its rampant and unregulated use however this substance has become implicated in a variety of adverse reactions. We report a case of a male-to-female transsexual patient who had received liquid silicone injections into both hips for cosmetic augmentation. Years after receiving these injections she developed silicone-induced granulomas causing hypercalcemia and subsequent renal failure. We discuss the controversy over the use of liquid silicone for soft tissue augmentation. In addition, the pathophysiology of granulomatous disease causing hypercalcemia, and treatment options for silicone-induced granulomas is discussed. Christine Kim, MD; Shagun Chopra-Sonthalia, MD Naval Medical Center San Diego Liquid injectable silicone is commonly used to correct or augment facial and body contours. The proponents for its use argue that when used appropriately liquid silicone can provide predictable beneficial long-term results. Conversely, opponents condemn its use altogether because of its numerous complications including chronic cellulitis, nodule formation, foreign body reactions, and migration. The controversy over the use of liquid silicone is yet to be resolved. The mechanism for hypercalcemia of granulomatous disease involves activated mononuclear cells, which are responsible for excessive conversion of calcidiol to calcitriol. Calcitriol in turn promotes hypercalcemia through various metabolic pathways. One would then expect that a patient with hypercalcemia due to granulomatous disease would have decreased serum levels of calcidiol and increased levels of calcitriol. This may not always be the case as illustrated in our patient who had normal levels of calcitriol (37 pg/mL; normal: 15-55 pg/mL). However, even though the calcitriol level was within the normal range, it was inappropriately elevated in the setting of hypercalcemia (12.7 mg/dL in our patient; normal: 8.410.2 mg/dL). Thus the notion of having a вЂњnormalвЂќ level of calcitriol alone should not rule out hypercalcemia due to granulomatous disease. Although the mainstay of treatment for silicone-induced granulomas is glucocorticoids, there are many other treatment options currently being utilized. A key point is that the removal of liquid silicone, which is not encapsulated, is difficult to perform. Surgical excision may result in incomplete removal, deformities, fistulas, and abscesses. This underscores the potential permanence of liquid silicone injections with adverse reactions requiring chronic long-term treatment. There are many causes of hypercalcemia. We should consider silicone induced granulomas in patients with a sig- Introduction: Fabry disease is a rare lysosomal storage disorder caused by a deficiency of alpha-galactosidase A. Its clinical manifestations result from the accumulation of glubotriaosylceramide in cells of the brain, heart, and kidneys. Symtoms are nonspecific which may delay diagnosis and worsen prognosis. We present a case of a young active duty male who presented with unexplained proteinuria. Case: A 33-year-old active duty male with a history of proteinuria as a reservist five years ago presented to the ophthalmology clinic with a six month history of binocular diplopia and was found to have a left 4th nerve palsy and corneal ventricullata suggestive of Fabry disease. Upon initial evaluation, he was noted to have 3 grams of proteinuria, low levels of alpha galactosidase, and normal kidney function. Nephrology evaluation revealed mildly elevated blood pressure and multiple angiokeratoma on scrotum. Serologic workup for other etiologies of proteinuria and renal ultrasound were unremarkable. A renal biopsy was performed with glomeruli demonstrating large podocytes with clear foamy cytoplasm. Fabry disease was confirmed. The pt was started on therapy with Fabrazyme every two weeks with reduction of proteinuria, and has maintained preserved kidney function for past three years. Discussion: Our case demonstrates the importance of early diagnosis of FabryвЂ™s disease in the young patient who presents with proteinuria. Early recognition of this disease is essential as devastating long-term complications can be reduced with early treatment. Life without Fibrinogen: An Uncommon Hemophilia Treated with a Common Blood Product Jerome Kim, MD; Deepthi Karunasiri, MD; Leland Powell, MD, PhD Olive View-UCLA Medical Center While relatively uncommon, patients with hemophilia A or B are easy to identify but require specific factor products for treatment. We present a case of a much less common congenital bleeding disorder whose treatment requires a readily available blood product. Case: A 21 yo woman with a history of easy bruising and bleeding presented with a three day history of increasing right hip pain. She denied any history of trauma and stated that the pain increased with hip flexion. There were no other associated symptoms including menorrhagia or hematochezia. She was a poor historian, but her past 35 medical history was significant for prior hematomas and an intra-cranial hemorrhage 5 years ago with no additional details. She had no siblings and stated that her parents were healthy. Her physical exam demonstrated decreased range of motion in the right hip and increased pain with right hip flexion and rotation. There was no obvious swelling, purpura, or petechia. Plain films of the hip were negative for fracture or dislocation. A CT scan of the pelvis indicated an asymmetric enlargement of the right obturator internus muscle consistent with a hematoma. Laboratory studies included platelets = 366,000, PT > 100 seconds, PTT > 201 seconds, fibrinogen level < 20 mg/dL, and d-dimer = 128 ng/mL. A PT mixing study (1:1 mix) corrected to 14.8 seconds. She was treated with 20 units of cryoprecipitate, and follow up laboratory studies included PT = 14.7 seconds, PTT = 28.6 seconds, and fibrinogen = 187 mg/dL. Subsequent studies from an outside reference laboratory indicated normal levels of Factors II, V, VIII, and IX, but with thrombin and reptilase times both > 150 seconds. Discussion: The patient was diagnosed with a congenital hypofibrinogenemia. Given the severity of her disorder and the lack of symptoms in either of her parents, she most likely had a mixed heterozygous state. The presence of a measurable d-dimer on initial presentation, along with the relatively mild bleeding history, suggested that she had a level of fibrinogen below the level of detection of standard laboratory assays. The blood product cryoprecipitate is known to be a rich source of fibrinogen (250 mg per unit). With correction of her fibrinogen level with cryoprecipitate, her fibrinogen was measured to decay at a half-life of approximately 2 ВЅ days, consistent with published reports and indicating the lack of a circulating inhibitor. She was prescribed to receive 20 units of cryoprecipitate every two weeks, and remained without clinically significant bleeding episodes when compliant with therapy. Preemptive Prevention of Immune Reconstitution Inflammatory Syndrome (IRIS) in Cryptococcal Meningitis Jerome Kim, MD; Catherine Yim, MD; Suzanne Donovan, MD, MPH Olive View-UCLA Medical Center A patient with AIDS and severe cryptococcal meningitis was initiated on corticosteroids, in conjunction with highly active antiretroviral therapy (HAART), to prevent immune reconstitution inflammatory syndrome (IRIS). IRIS is a frequent and troublesome complication of HAART in patients with cryptococcal and other opportunistic infections. Case: A 49-year-old male presents with cryptococcal meningitis complicated by increased intracranial pressures. He was found to be HIV-infected with a viral load of >100,000 copies/mL and CD4 of 10/mm3. He was initially treated with Amphotericin and 5-FC, followed by high dose fluconazole. MRI demonstrated confluent gelatinous pseudocysts secondary to cryptococcal infection (Figure 1). Follow-up exams, lumbar punctures and fundoscopic exams demonstrated persistent headache, elevated opening pressures and papilledema despite aggressive antifungal treatment, acetazolamide and serial lumbar punctures to relieve elevated pressures. Results of serial lumbar punctures are shown in Table 1. Follow-up CSF fungal cultures were negative and cryptococcal antigen (CRAG) titers on subsequent lumbar punctures demonstrated a significant decrease. Patient refused intraventricular shunt placement. HAART was initiated with high dose prednisone (60 mg/day), because of concern for IRIS with potential worsening of his intracranial pressures, with a prednisone taper over 6 weeks. Symptoms of IRIS (headaches, vomiting and increased ICP) occurred 4 months later after prednisone taper during a time of immune reconstitution (HIV viral load 422 copies/mL , CD4 30/mm3). CSF parameters are shown in Table 1. Patient responded to reinstitution of a longer taper of corticosteroids and short course of acetazolamide. Follow-up MRI demonstrated resolution of gelatinous pseudocysts. Results of follow-up lumbar punctures normalized. Discussion: Immune Reconstitution Inflammatory Syndrome (IRIS) is an inflammatory response to a preexisting infection after initiation of highly active antiretroviral therapy (HAART) in patients with AIDS. It occurs in up to one third of AIDS patients starting HAART, and has a tendency to affect patients who have profound immune deficiency at baseline, and rapid viral suppression and immune recovery with HAART. The treatment for cryptococcal IRIS include serial lumbar punctures to relieve elevated intracranial pressure, decreasing fungal load with antimicrobial therapy (Amphotericin B and flucytosine) and the use of corticosteroids for anti-inflammatory effects. We present a case of a patient at high risk for IRIS, and at significant risk for neurologic complications, successfully treated with steroids at HAART initiation. A Case of Coloboma and Horseshoe Kidney Paul Korc, MD; Eunice Rios, MD University of Southern California Case: A 27 year old woman with no past medical history presented to our hospital with 4 days of cough, shortness of breath, and wheezing. In the emergency room she was hypoxic with an oxygen saturation of 82% on room air. She was treated for new onset asthma with bronchodilators and systemic steroids. On exam she was noted to have abnormally shaped and downwardly displaced pupils consistent with coloboma of the iris. She also had a secondary complaint of lower abdominal pain and underwent a CT abdomen/pelvis, which demonstrated an ovarian teratoma and, incidentally, horseshoe kidney. She denied a history of any visual complaints, and had normal visual acuity. Her renal function is normal as well. 36 Discussion: Coloboma results from a failure of the fetal or choroidal fissure to close during the 5th to 7th weeks of fetal life. It can involve almost any ocular structure. The Renal Coloboma Syndrome (or Papillorenal Syndrome) is an autosomal dominant disorder characterized by renal abnormalities and optic nerve defects. The gene that has been implicated is PAX2, which encodes the PAX2 developmental nuclear transcription factor. Renal abnormalities can include hypoplasia, early renal failure, and vesico-ureteral reflux. The degree of renal abnormality lies along a spectrum, and variable phenotypes have been reported, including rare cases of horseshoe kidney. Given the constellation of bilateral coloboma and horseshoe kidney in our patient, she may harbor the PAX2 mutation and a variant of the Renal Coloboma Syndrome. We are currently considering genetic testing. candidate for percutaneous closure of the ASD given his symptoms. Cardiac catheterization was performed. There was no evidence of coronary artery disease. Pulmonary artery pressure was 40/12. QP to QS ratio was 1.26. MRI of the lumbar spine revealed L4-L5 disk prolapse with impingement of the L5 nerve root. The patient underwent neurosurgery for the disc herniation. After recovery, he had percutaneous closure of the ASD, which improved his symptoms and oxygenation. This case illustrates the importance of detecting abnormalities on physical exam as well as clinical studies, which can clue the internist into completing a further workup. Congenital heart diseases vary in their age of presentation. It is important, not only for pediatricians, but also for internists to be able to diagnose these conditions. Ebstein's Anomaly Diagnosed in a 33 Year-Old Roshan Kotha, MD Scripps Mercy Hospital Congenital heart diseases vary widely in their severity and clinical presentations. Certain pathologic findings on physical exam and clinical studies can provide clues to the diagnosis. A 33-year-old Vietnamese male presented to the ED complaining of shortness of breath and right lower extremity pain. The patient endorsed limitations in physical activity due to dyspnea since childhood. He had been told he had a вЂњleaky valveвЂќ in childhood, however did not have further follow up for this. Over a few days prior to admission, he developed worsening dyspnea with minimal exertion. He also reported intermittent right lower extremity pain radiating from the gluteal area, to the ankle over the past 3 years. 2-3 days prior to admission, he developed weakness as well. On exam, he was a well appearing, well developed, male in no distress. His vitals were within normal limits with an oxygen saturation of 96% on room air. There was no evidence of cyanosis. Lungs were clear to auscultation. Cardiovascular exam revealed a regular rate and rhythm, with a fixed and split S1 and S2, and a 3/6 systolic murmur at the left lower sternal border. JVP revealed prominent a and v waves. Strength was 5/5 throughout, except for the right leg, which had 2-5/5 strength in various muscles. Sensation was decreased to light touch and pinprick along the L5 dermatome. Laboratory studies were negative for evidence of myocardial ischemia. EKG revealed sinus rhythm, right atrial enlargement, and a non-specific intraventricular conduction delay. Chest X ray revealed cardiomegaly, without pulmonary vascular prominence. A transthoracic echo revealed EbsteinвЂ™s anomaly, with atrialization of the right ventricle and apical displacement of the tricuspid valve, along with an atrial septal defect. A transesophageal echo revealed a bidirectional shunt, which was predominantly left to right along the ASD. The patient was felt to be a New Onset Reflux After Acute Gastroenteritis: A Case of Post-Infectious GERD David Kunkel, MD; Mark Pimentel, MD Cedars-Sinai Medical Center Exposure of the gut to infectious pathogens can alter native bowel flora and motility, triggering disorders such as Irritable Bowel Syndrome. Here we report the first case of GERD developing in an adult after an episode of acute gastroenteritis. A previously healthy 48 year old female presented to GI clinic for evaluation and treatment of belching and epigastric bloating. One year prior to presentation, the patient had traveled to a remote area of China and eaten at a local restaurant of questionable repute, subsequently developing nausea, vomiting, and diarrhea the following morning. While her acute symptoms resolved, the patient started to experience frequent belching associated with bloating and anorexia. She had resorted to eating small meals because large portions resulted in food regurgitation. She was not having any fevers, sweats, cough, blood per rectum, abdominal pain, or change in bowel habits. Short courses of probiotics, digestive enzymes, peppermint oil, and herbal medications had not relieved her symptoms. On physical exam, the patient had a temperature of 98 degrees. Auscultation of the abdomen revealed intermittent bursts of hyperactive bowel sounds over the epigastrium occurring once every 20 seconds. Her abdomen was otherwise soft, non-tender, with no masses or hepatosplenomegaly. Laboratory studies revealed a normal complete blood count. Her liver function tests, amylase and lipase were normal. Stool studies including fecal ova and parasite were negative for evidence of active infection. Upper endoscopy was performed which revealed normal gastric and duodenal mucosa. Biopsies were negative for H. pylori and celiac disease. Antroduodenal manometry disclosed high amplitude contractions of the pylorus up to 466 mm Hg. Twenty-four hour pH monitoring revealed markedly elevated acid exposure times in both the upright and recumbent positions of 20% and 14% respectively, for an acid reflux composite score of 79, consistent with a diagnosis of GERD. The patient was placed on a PPI and experi- 37 enced complete resolution of her symptoms. Exposure to infectious pathogens can lead to several complications ranging in severity from acute gastroenteritis to postinfectious IBS. This case illustrates the first report of reflux developing after an epsidoe of acute gastroenteritis. Ruptured Intracranial Mycotic Anuerysm in Infective Endocarditis Isabel Kuo; Theodore Long; Bharat Chaudry, MD; Michael Karp, MD; Nathan Nguygen, MD University of Southern California Mycotic aneurysms (MA) are intracranial aneurysms that develop in the presence of infections such as infective endocarditis (IE). They account for a small percentage of all intracranial aneurysms but carry a high mortality rate when they rupture. A high index of suspicion and early treatment can reduce the risk of rupture and improve outcome. Our case is that of a 54-year-old male with history of IE complicated by cerebrovascular accident 15 years ago without residual deficit who was in his usual state of health until 2 days prior to admission when presented to the emergency department complaining of slurred speech. His physical exam was significant for a temperature of 102.3 degrees Fahrenheit and a 3/6 holosystolic murmur most prominent at the left lower sternal border with radiation to the axilla. He also had poor dentition, marked clubbing of his fingers, left facial weakness, subtle slurring of speech, and diffuse hyperreflexia. The echocardiogram showed a large mobile mass on the mitral valve leaflet, and multiple sets of blood cultures grew Streptococcus viridans. An MRI of the brain showed an acute right frontal lobe infarct and old blood products. Intravenous Penicillin G and Gentamicin were started immediately; however, six days into his antibiotic course, he became obtunded. An emergent CT cerebral angiogram showed a large ruptured aneurysm at the distal segment of the anterior cerebral artery with adjacent intraparenchymal and intraventricular hemorrhage. He underwent emergent craniotomy and aneurysm clipping. His postoperative course was complicated by multiple arterial vasospasms treated with triple-H therapy. The patient achieved partial recovery and was able to follow basic commands at the time of discharge. Valve replacement surgery was deferred until resolution of his acute stroke and hemorrhage. After tracheostomy and gastric tube placement, he was transferred to a skilled nursing facility for a planned six-week course of IV antibiotics. This case emphasizes the importance of considering MA in the differential diagnosis when IE and intracranial septic embolization are present. MA should be investigated early with vascular angiography, and a low threshold of suspicion for subarachnoid hemorrhage should be maintained. Once the diagnosis has been made, a neurosurgical consult should be obtained early to determine the risk of rupture and the appropriate therapeutic intervention. Crystal Clear: A вЂњShockвЂќ-ing Case of Gout Brent W. Lacey, MD; James Prahl, MD; Scott Parrish, MD Naval Medical Center San Diego, CA Case: An 80 year-old male with chronic gout presented with severe left knee and lower back pain. A lumbar MRI showed findings consistent with L4/5 pyogenic discitis, and an intervertebral disc biopsy showed minimal granulomatous changes. All cultures were negative. The patientвЂ™s condition deteriorated despite broad spectrum antibiotics, and he developed polyarticular inflammation and delirium. He subsequently required intubation and vasopressor support. A left knee arthrocentesis revealed monosodium urate crystals, 32,000 neutrophils, and a negative gram stain. Repeat lumbar MRI showed changes consistent with gouty arthritis. After all cultures were confirmed to be negative, solu-medrol was started to treat systemic distributive shock presumed to be secondary to an acute gout flare. The patientвЂ™s fever defervesced, and vasopressor and ventilator support were discontinued the next day. The patient made a full recovery on a 3-week steroid taper and allopurinol. Discussion: 81 cases have been reported where gout was seen to mimic pyogenic discitis. Only one case has been associated with distributive shock. This case represents the first patient reported to have both conditions simultaneously. Gout is a monoarticular inflammatory condition most commonly affecting the knee or first metatarsophalangeal joints. The definitive diagnosis is incumbent on identification of either tophi or monosodium urate crystals. Gout affecting the spine, though rare, can pose a significant health risk, including spinal cord compression necessitating urgent surgical intervention. Spinal gout appears to respond to standard acute gout management, despite the unusual location. The development of distributive shock is the most significant feature of the case. Distributive shock results from an imbalance between the pro- and anti-inflammatory components of the hostвЂ™s immune system. This patient did not have an obvious predisposing feature for progression to shock. The pathogenesis of his condition should therefore prompt exploration into genetic or other pathophysiologic factors that would incline this patient to develop shock. Profound Lactic Acidosis Due to Hepatic Steatosis Mark Lee, MD; Carrie Chun, MD Scripps Green Hospital Lactic acid is a derivative of anaerobic metabolism and is the primary cause of metabolic acidosis. Early clearance of plasma lactate levels has been associated with improved survival in hospitalized patients. Identifying the source of lactic acidosis is critical so that appropriate treatment can be tailored to the underlying cause. 38 University of Southern California A 57 year-old morbidly obese woman with a history of gastric-banding surgery, right hemicolectomy secondary to colon cancer, and nephrolithiasis presented with a two day history of clear emesis and colicky, abdominal pain. Her husband noted a two month period of depression and poor oral intake with an associated 50-pound weight loss. On exam, the patient was normotensive and tachycardic, with exophthalmos, dry mucous membranes, decreased skin turgor and an obese but benign abdomen. Labs demonstrated: WBC 15.2 cells/ОјL, potassium 2.6 mmol/L, serum creatinine 0.8 mg/dL, serum bicarbonate 9 mEq/L, anion-gap 28, lactic acid 2.0 mmol/L , VBG pH 7.12, normal hepatic function and urine ketones. AbdomenPelvis CT revealed small renal stones with mild hydronephrosis. The patient was admitted to the ICU with starvation ketoacidosis. She received IV-fluid resuscitation and TPN with resolution of the acidosis. After early improvement, the patient developed hypotension initially responsive to fluid resuscitation, but eventually required vasopressor support. She became febrile with persistent leukocytosis, and broad-spectrum antibiotics were begun. Given her hemodynamic instability, a lactic acid was repeated and elevated at 7.8 mmol/L. Blood and urine cultures were negative. Repeat abdomenpelvis CT showed decreased hydronephrosis with no evidence of mesenteric ischemia or masses. Upper endoscopy revealed only mild gastritis. On hospital day 5, the patient was intubated for impending respiratory failure. Lactic acid was 14.7 mmol/L. Multiple blood, urine, and sputum cultures remained negative. Her medication profile was reconciled with her husband, and no lactic acid inducing medication was identified. Despite continuous bicarbonate infusion and initiation of CRRT, shock and lactic acidosis persisted. After several days of maximal aggressive care, the family requested transition to comfort care. The patient expired on hospital day 7. Autopsy toxicology results were unrevealing, but gross pathology revealed marked macrovesicular hepatic steatosis with near complete fatty replacement of the liver. This case demonstrates a rare case of hepatic steatosis induced lactic acidosis. All other workup for hyperlactatemia was negative. Lactic acidosis and hepatic steatosis may result from medications, but there are few reports of hyperlactatemia from hepatic steatosis alone. In advanced liver disease, lactic acidosis is thought to arise from tissue hypoxia that results from arteriovenous shunting. This finding is associated with high mortality, and care is supportive only. Pulmonary Mucormycosis in a Non-DKA Diabetic Patient Than Lin, DO; Kenneth Lue, DO; Sid Anand, MS IV, MBA; Christine Kim, MD; Shahrod Mokhtari, MD; Vonny Tunru-Dinh, MD; Michael Karp MD Introduction: Pulmonary mucormycosis, a subset of zygomycosis, occurs when spores are inhaled through the respiratory tract. Though uncommon in healthy individuals, patients with poorly controlled diabetes are at increased risk for the infection. Diabetic ketoacidosis (DKA) is a common underlying medical condition that is present when mucormycosis is detected. In those with DKA, the acidic environment of the serum causes dissociation of free iron from sequestering proteins, stimulating rapid fungal growth. Case: A 26 year old Hispanic female with newly diagnosed diabetes (HbA1c 13.2) presented with hemoptysis, night sweat, fever and unintentional weight loss. Chest radiograph showed right lower lobe consolidation suspicious for abscess formation. Sputum cultures were negative for tuberculosis. CT chest showed a necrotizing process in the right lower lobe and a second cavitary lesion in the right upper lobe. Bronchoscopy was performed with bronchoalveolar lavage resulting positive for Rhizopus species and biopsy revealed nonseptate hyphae consistent with invasive mucormycosis. The patient was immediately started on Amphotericin B, Deferasirox, tight glycemic control, and surgical consultation was sought for lobectomy. After several weeks of antimicrobial therapy, the patient underwent single lobectomy and was recovering from that procedure as of the writing of this abstract. Discussion: Pulmonary mucormycosis is a rapidly invasive pulmonary infection that usually affects immunocompromised patients with hematologic malignancies receiving chemotherapy or HIV. Diabetic patients have a distinct clinical syndrome as evidenced by the organism invading the airways and hilar vessels, resulting in atelectasis, abscess formation, or severe hemorrhage presenting as massive hemoptysis, usually with a predilection for the right lung. Radiographic features include nodular infiltrates, mediastinal widening, solitary nodules, cavitation or pleural effusion. Histologic characteristics will show nonseptate hyphae with right angle branching invading pulmonary tissue. Despite early surgical and medical therapy, literature suggests a poor prognosis for pulmonary mucormycosis. The mortality rate of patients solely treated with antifungal medication is 68% compared to 11% for patients treated with antifungal medication and surgical correction. Conclusion: Diabetic patients have been linked to Mucormycosis, but most cases presented with rhino-cerebral infection, and most have DKA. This case is unique in that our patient did not present with DKA, had pulmonary, not rhino-cerebral infection, and that she was not immunocompromised with malignancy or HIV. While literature suggests a mechanism for patients with DKA, there is limited information regarding why non-DKA diabetes are predisposed to infection. This case suggests the need for a proposed mechanism of action for nonDKA diabetic patients infected with Mucor. If the 39 mechanism of infection in non-DKA diabetics is not the creation of a high-iron environment through acidosis, perhaps there is an additional component of the pathophysiology of the disease that should be explored for optimal management. 28 Year-Old Female Requiring Frequent Therapeutic Whole-Lung Lavages Eduardo A. Lopez, MD; Nikhil Barot, MD; Dennis Yick, MD, FCCP; Nader Kamangar, MD, FACP, FCCP Olive View-UCLA Medical Center Case: 28-year-old African American woman consulted her doctor because of progressive dyspnea on exertion, non-exertional chest pain, and occasional hemoptysis. Her lung examination was normal despite grossly abnormal chest radiograph. She did not exhibit any evidence of heart failure, cyanosis, or clubbing. Her pulmonary function tests revealed a restrictive ventilatory defect and a moderate to severe reduction of her DLCO. Her arterial oxygen saturation was 94% while she was breathing ambient air and quickly desaturated after minimal exertion, and her chest radiograph showed bilateral alveolar and interstitial opacities. High-resolution computed tomography (CT) of the chest showed a bilateral ground-glass opacity with interlobular septal thickening, creating a mosaic, or "crazy paving," pattern in both lung fields. A very extensive work up failed to demonstrate an infectious origin of her symptoms. Treatment was initiated with high-dose corticosteroids and broad spectrum antibiotics. Despite this, her condition worsened. Bilateral WLL was performed under general anesthesia and single-lung ventilation. . We used 2 liters of Normal Saline in the left lung and 8 liters on the right side,1 week apart. The lavage fluid was initially turbid with sediment but gradually cleared. After the procedure, the symptoms diminished, with O2 saturation improving to 97% while the patient was breathing ambient air and 96% with exertion. The patient was asymptomatic at discharge. Discussion: Pulmonary alveolar proteinosis (PAP) is a rare disorder caused by abundant accumulation of surfactant-derived components in the lungs. Historically, there has been no effective pharmacotherapy for PAP, and sequential whole-lung lavage (WLL) under general anesthesia has become the mainstay of treatment. PAP should be considered an "orphan" lung disease. There are only 500 reported cases in the literature to date. Secondary PAP can be seen in hematologic cancers, pharmacologic immunosuppression, inhalation of toxins, and certain infections. Crazy-Paving is characteristic for PAP, yet many other diseases can show this appearance on CT; Crazy-paving is a nonspecific finding. This case illustrates that many issues remain unclear regarding the optimal therapy of patients with PAP. The Least Compatible Patient Dustin Lorentz, MD; Mark Tschanz, DO Cedars-Sinai Medical Center Autoimmune hemolytic anemia (AIHA) is caused by the presence of autoantibodies directed against antigens on the surface of red blood cells. The incidence is reported at one per 80,000 people, peaking in the first four years of life, with males and females affected equally. Most commonly idiopathic, predisposing conditions include infections, drugs, hematologic conditions, and/or immune disorders. Treatment is aimed at reducing the amount of antibody produced or decreasing the efficiency of the antibody in lysing cells, most commonly with corticosteroids or immunoglobulin. AIHA can be an emergency when patients become severely anemic, as they can lose the ability to sustain sufficient oxygenation to vital organs. In these patients, immediate transfusion of blood can be life-saving. Our patient is a 62 year-old female with a history of idiopathic thrombocytopenic purpura (ITP) who was admitted with generalized weakness and jaundice. Her baseline hemoglobin was 14.4 g/dl, and upon admission the hemoglobin of 7.4 g/dl dropped precipitously to 4.8 g/dl within 10 hours of presentation. All lab values including direct CoombвЂ™s test, haptoglobin, lactate dehydrogenase, and reticulocyte count were consistent with a diagnosis of AIHA. Orders were made to crossmatch blood, but all available units were deemed incompatible. The blood bank was contacted for stat retrieval of вЂњleast incompatibleвЂќ blood, and the patient was placed on high-dose corticosteroids prior to transfusion of blood in the intensive care unit. Her hemoglobin eventually stabilized, and she was discharged from the hospital on prednisone. Given her associated history of ITP, the patient was diagnosed with Evans Syndrome, a combination of Coombs-positive warm AIHA and ITP. Though people with this syndrome are historically more resistant to standard treatment than those with AIHA or ITP alone, the patient continues to have stable blood counts on follow-up. In AIHA, it is unlikely that fully compatible blood can be matched, since the autoantibody produced by the patient reacts with antigens present on cells of almost all individuals in the donor population. Thus, for patients in need of emergent transfusion, compatibility testing should not delay their possibly life-saving treatment. This often requires contacting the blood bank to minimize delays and avoid misunderstandings concerning the degree of compatibility of available blood. With appropriate and timely therapy, these incompatible patients can be successfully treated 95% of the time. An Incidental Finding of Coccidioidomycosis Osteomyelitis Monique Mahlum, MD University of California, San Diego Case: Mr. S is an 82-year-old African-American man originally from Arizona with history of diabetes mellitus who presented with microhematuria in routine screening labs from clinic. Ureterolithiasis was suspected as the source of microhematuria. An abdominal x-ray was nega- 40 tive for calcifications. A CT urogram was done and showed an incidental finding of inflammatory changes in the L1-2 region. After this finding, a focused history revealed 3 months of consistent low back pain. He denied fevers, weight loss, night sweats, cough, and shortness of breath. He had no history of IV drug use, previous back surgery, or trauma to the low back. On exam, he had no spinal tenderness or neurological signs. Chest x-ray was clear. ESR was elevated at 79. Blood cultures were negative. Bacterial, AFB, and mycology bone biopsy cultures were negative. Pathology showed non-necrotizing granulomas with foreign body type giant cells. GMS and PAS stains highlighted rare round, encapsulated organisms. A diagnosis of vertebral coccidioidomycosis was made. Serology showed cocci IgG positive, IgM negative. The patient was started on fluconazole 400 mg PO q12 hours with infectious disease follow up. Discussion: Coccidioidomycosis, also known as Valley Fever, is endemic in the southwestern United States, parts of Mexico and South America. Spores live in the soil and are spread airborne in dust. Of people who live in an endemic region, about 10-50% will have evidence of exposure to Coccidioides. The annual incidence of Coccidioidomycosis tripled in California between 2000 and 2006. Incidence rates in Arizona are similar to those in California. Arizona annually reports about 60% of all cases in the US. Less than 1% of patients infected with C. immitis progress to extrapulmonary disease, most commonly to the skin, bones, joints and CNS. Bone infections, specifically the vertebral column, account for about 20-50% of extrapulmonary cocci. An increased risk of disseminated disease is seen in men, African-Americans and Filipinos. Mechanism of spread is hematogenous after a primary pulmonary infection. By the time patients experience skeletal involvement, the pulmonary infection has resolved and findings on CXR are normal. This is the case with Mr. S as the IgG was positive with a clear CXR and no pulmonary symptoms. In an endemic Cocci region, clinicians must always have a high level of suspicion for this fungus, even with no overt pulmonary symptoms. Left Bundle Branch Block Induced by Near Tachycardia in a Patient without Ischemic Disease Nalin Mallik, MD; Reza Rafie, MD; Meera Malhotra, MD; Sharmineh Shams, MD University of Southern California Rate-related bundle branch block is a well established phenomenon. These patients have an increased refractoriness of the bundle and the block appears at tachycardic rates. They usually have underlying coronary disease. In this case report we present a patient with serial ECGs that demonstrate the induction of LBBB at near-tachycardia, with a return to a narrow QRS at slower rates. She has no underlying CAD. Case Description: A 65 year-old woman with a history of hypertension was admitted for chest pain. Her chest pain started three days prior to admission. She described the pain as pressure-like with radiation to the back and neck. The pain lasted for a few minutes, and occurred at both rest and during exertion. The patient also experienced associated shortness of breath with her chest pain. She had good exercise capacity and denied orthopnea, peripheral edema or paroxysmal nocturnal dyspnea. She was taking losartan for her hypertension. Physical exam was significant for a BP of 148/96 mmHg and heart rate of 93 bpm. Heart exam showed regular rhythm without murmur, rub or gallop. Her troponin levels were checked three times, all of which were in the normal range. The first ECG showed normal sinus rhythm with a left bundle branch block pattern. The QRS duration was 124 msec, and heart rate was 95 beats/minutes. Subsequent ECGs in the next few hours showed sinus rhythm with heart rates of 68 and 76 beats/minute. The QRS durations on these repeat ECGs were 84 msec and 82 msec, respectively, with no evidence of conduction abnormality. The patient was recognized as near tachycardia-induced LBBB. She subsequently underwent exercise cardiac thallium scan that showed a normal ejection fraction with normal cardiac perfusion results. She had no further chest pain and was discharged on losartan and amlodipine. Discussion: Rate-dependent LBBB uncommonly occurs in healthy adult patients with normal cardiac perfusion radionuclide scans, and heart rates exceeding 125 beats/minute. The left bundle branch has dual blood supply involving left anterior descending and posterior descending artery and the LBBB can be due to either coronary vasospasm versus a perfusion defect in the microcirculation. Rarely, exercise-induced LBBB has been associated with dilated cardiomyopathy. However, for the first time we present a case of near tachycardia-induced LBBB occurring in a patient with a normal cardiac perfusion radionuclide scan at a heart rate of 95 beats/minutes. This case report depicts that upper-limits of normal heart rate can induce LBBB in patients with normal cardiac perfusion. Although previous studies have shown exercise induced LBBB predicts a higher risk of cardiovascular events, more studies are needed to figure out the outcome in patients with LBBB induced by trivial elevation in heart rate and normal cardiac thallium scan. Acromegaly Uncovered by West Nile Meningoencephalitis Ryan Martin, BS; Nasser Mikhail, MD; Mark Richman, MD, MPH Olive View-UCLA Medical Center Case: A 63 year-old Hispanic male with a past medical history of hypertension, type II diabetes mellitus, and bilateral knee osteoarthritis presented with acute onset of severe headache, fatigue, fever, and light-headedness with a questionable episode of loss of consciousness one night prior to admission. Upon presentation, his blood pressure was 119/84, pulse 140, respiratory rate 23 with 41 normal oxygen saturation, and a temperature of 40o C. He was obese with oily, diaphoretic skin and coarse facial features, including frontal bossing, prognathism, and large, thick hands. He spoke with a deep, resonant voice. His mental status was intact and there were no focal neurological deficits. Kernig and Brudzinski signs were negative. Initial laboratory studies were normal except for an elevated lactic acid level of 2.5 mmol/L. Brain CT showed an enlarged 12 mm pituitary gland. Pituitary function tests were normal except for an elevated insulin-like growth factor-1 (IGF-1) of 650 ng/mL and low total testosterone of 103 ng/dl. A lumbar puncture elicited an opening pressure of 28 cm H20 with apparently normal CSF analysis; CSF analysis subsequently proved to be positive for the West Nile Virus. The patient responded well to conservative management and was discharged home. An outpatient MRI of the brain showed a large T1 hypointense, hypo-enhancing intra-sellar mass measuring 12.5 x 16 x 12.2 mm, consistent with a pituitary macroadenoma. Formal visual field evaluation elicited bilateral superior hemianopia. A diagnosis of acromegaly and hypogonadotropic hypogonadism was established and the patient was referred to neurosurgery for resection of the pituitary macroadenoma. Discussion: Acromegaly is a rare endocrine disorder, with over 90% of cases secondary to a benign monoclonal GHвЂ“secreting pituitary adenoma, leading to excessive production of IGF-1 by the liver. This causes excessive cell growth, multiplication, and inhibition of apoptosis in a variety of organ tissues. More than half the cases are diagnosed by subspecialists after evaluation for a variety of cardiovascular, endocrine, musculo-skeletal, neurologic, respiratory, and other manifestations resulting from either local pituitary mass or systemic growth hormone effects. The diagnosis has historically been made by combining a high index of suspicion with abnormal oral glucose tolerance testing. However, the latter has largely been replaced by direct assays of elevated serum IGF-1 levels, which correlates better with the signs and symptoms of acromegaly. Early diagnosis is important, as the morbidity and 3-fold increased mortality associated with acromegaly is largely related to the duration of exposure to elevated serum IGF-1 and GH levels; early detection and treatment can limit many of these deficits. Treatment goals of acromegaly include normalization of IGF-1 levels which can be obtained through surgical or external beam radiotherapy debulking of the pituitary adenoma. The somatostatin analogs, ocreotide and lancreotide, are options for conservative management. Ornithine Transcarbamylase Deficiency in Patients with Neuroendocrine Tumors Aashini Master, MD; Michael Keng, MD; Helen Gu, MD University of Southern California Background Urea cycle deficiencies are extremely rare disorders of nitrogen metabolism occurring in one out of eight thousand births. Most affected patients present as newborns, but those with partial enzyme deficiency may become symptomatic later in childhood or adulthood. The following cases presented are those with previously undiagnosed ornithine transcarbamylase deficiency (OTCD) unmasked by increasing hepatic tumor burden from neuroendocrine cancer. Case DESCRIPTION The following cases were patients with newly diagnosed neuroendocrine tumors presenting with altered mental status. Case I: A 28-year-old Chinese woman with a history of chronic Hepatitis B and newly diagnosed neuroendocrine tumor with liver metastasis presented with six months of abdominal pain. After consuming a colonoscopy preparation for a scheduled endoscopy to look for the primary tumor site, the patient presented to the ED with severe abdominal pain and nausea. In the ED, she became increasingly confused and progressed to becoming unresponsive to verbal and painful stimuli. Upon admission, she was found to have normal laboratory values other than a mild transaminitis and elevated ammonia level, which prompted an amino acid analysis panel since there was no evidence of portal hypertension or hepatic synthetic dysfunction. Case II: A 51-year-old Korean woman with a history of metatastic carcinoid tumor of the pancreatic tail presented to the ED for altered mentation. The patient had an elevated ammonia level without evidence of portal hypertension or hepatic synthetic dysfunction and was treated for presumed hepatic encephalopathy during prior hospitalizations. The patient returned with the same chief complaint despite medication compliance. Chemotherapy treatment was withheld secondary to her poor performance status. Despite implementation of aggressive treatment for hepatic encephalopathy, the patientвЂ™s mentation continued to decline, resulting in an amino acid evaluation. Conclusion The two cases discussed both illustrate examples of hyperammonemia in the setting of neuroendocrine tumor. It is important to underscore that urea cycle genetic disorders can manifest later in life. Although these disorders may not be the most common causes for hyperammonemia, it is important to consider their diagnoses during periods of acute stress such as malignancy, pregnancy, bleeding, sepsis, and fasting. Upon review of the literature specifically regarding malignancy, there is a well-known association of hyperammonemia with multiple myeloma, plasma cell leukemia, and leiomyosarcoma, or in patients who have recently received chemotherapy. To our knowledge, there has only been one published case report of hyperammonemia in the setting of neuroendocrine tumor. The cases presented demonstrate a further association between neuroendocrine tumors and urea cycle deficiencies. This highlights the necessity for a 42 complete urea cycle evaluation for patients diagnosed with neuroendocrine tumor and elevated ammonia levels. Spontaneous Rupture of the Spleen in a Patient with Diffuse Large B-Cell Lymphoma Aashini Master, MD; Michael Keng, MD; Roberto Contreras II, MD; Marcia A. Ciccone University of Southern California Introduction: Spontaneous splenic rupture (SSR) is a rare, but known complication of hematologic malignancies. It is defined as splenic rupture without antecedent trauma. This event is a true emergency, requiring prompt surgical intervention. Case Presentation: The patient was a 45-year-old Caucasian woman with no past medical history presenting with two weeks of fevers and left flank pain. The left flank pain was described as вЂњthrobbing and aching,вЂќ which progressively increased in intensity. The patient only admitted to having fevers and myalgias. She denied night sweats, chills, and weight loss. On admission, she appeared comfortable and in no acute distress. Vital signs: Temperature 102.3F, HR 103/min, and BP 100/63mmHg. Initial exam was significant for palpable, non-tender axillary and cervical lymphadenopathy and splenomegaly extending six-centimeters below the costal margin. She had diffuse abdominal tenderness to deep palpation, without rebound or guarding. Significant admission laboratory findings: hemogloblin 8.9g/dL, hematocrit 25.4%, platelets 41K/cumm, white cell count 5K/cumm, metamyelocyte 2%, myelocyte 2%, total protein 4.9g/dL, and albumin 2.6g/dL. The patient received an abdominal ultrasound in the ED, revealing splenomegaly approximately at 16cm in length. CT abdomen/pelvis confirmed the ultrasound finding. She was admitted to the medicine service for fever and splenomegaly work-up. She was found to be Hepatitis C positive; but all other infectious work-up including Brucella, CMV, EBV, Hepatitis B, Histoplasma, HIV, Parvovrius, West Nile Virus, fungal, and bacterial infections were negative. She was empirically placed on antibiotics for persistent fevers per Infectious-Disease consultation. Further laboratory evaluation revealed an LDH of 463U/L. Vitamin B12, folate, TSH, iron panel, and reticulocyte count were all within normal limits. Bone marrow biopsy subsequently revealed diffuse large B cell lymphoma (DLBCL). Unfortunately, prior to the results of the bone marrow biopsy, the patient complained of sudden increased severity of abdominal pain and was noted to have new abdominal distention. She quickly decompensated, and was unable to be resuscitated. Postmortem examination showed marked splenomegaly with splenic rupture and 3000cc blood within the abdominal cavity. Changes of DLBCL were seen involving the spleen, bone, and lymph nodes. Conclusion: SSR is an infrequent occurrence that is generally seen in the setting of infectious, neoplastic, hematologic diseases, or congenital splenic lesions. Risk factors associated with SSR are male sex, adulthood and massive splenomegaly. A review of the literature reveals approximately 150 cases of SSR in the setting of hematologic malignancy, most frequently seen with nonHodgkinвЂ™s lymphoma and acute myeloid leukemia. Splenectomy remains the mainstay of treatment for SSR and this case emphasizes the importance of prompt surgical evaluation for massive splenomegaly and the necessity for further evaluation of the risk factors associated with splenic rupture in the setting of hematologic malignancy. Hypercalcemia and Strongyloides Superinfection in a Patient with Adult T-Cell Lymphoma/leukemia (ATLL) Bradley Messenger, MD; James Yeh, MD; Maria Dungo, MD Harbor-UCLA Medical Center A 55 year-old black male from Belize 6 years ago was admitted for 1 week of alteration in mental status, decreased oral intake, and low back pain. He had 10 lb weight loss and night sweats for 2 months. 2 months ago he had abdominal pain and stools were positive for Strongyloides stercoralis. He was given oral antimicrobials, but was lost to follow-up. He was afebrile, BP 170/100, lethargic, oriented only to self, hypovolemic, and appeared wasted. He had inguinal lymphadenopathy. BUN 60 mg/dL, Cr 4.5mg/dL, Ca 18.2mg/dL, total protein 7.1g/dL, albumin 2.7 g/dL, alkaline phosphatase 309. Urinalysis had 1+ protein. CT scan showed multiple lytic lesions in ribs, pelvis and spine. Following IV hydration, furosemide, and calcitonin, the hypercalcemia improved and the patient became gradually alert. Workup for multiple myeloma, include serum and urine protein electrophoresis and serum light chain, was negative. He was started on hemodialysis for renal insufficiency, but subsequently developed worsening hypercalcemia and leukocytosis. Bone marrow aspirate revealed hypercellularity with involvement by adult T-cell leukemia and lymphoma, and peripheral blood smear demonstrated marked lymphocytosis with вЂњflower cells.вЂќ Due to refractory hypercalcemia and leukocytosis, patient was initiated on dexemethasone, but subsequently developed respiratory distress requiring monitoring in the intensive care unit. Bronchoscopy revealed Strongyloides hyperinfection. Stool also showed Strongyloides. Serology for HTLV-1 was positive, and he subsequently received combination chemotherapy with cyclophosphamide, vincristine, doxorubicin and prednisone. After an initial hematologic response with decrease in lymphoma cells in the peripheral blood over several days, patientвЂ™s lymphocytosis and hypercalcemia recurred. He developed fever, lactic acidosis, and myocardial infarction, and expired after being placed on comfort measures. Adult T-cell lymphoma/leukemia (ATLL) is a nonHodgkin's T-cell lymphoid neoplasm of varying clinical manifestations and prognoses. ATLL results from HTLV1 infection and its incidence strongly associates with areas endemic to the virus, such as Japan, the Caribbean, 43 western Africa and Peru. HTLV-1 may be transmitted by blood or sexual contact, but more often transmitted through breastfeeding, thereby infecting many at a young age. Pathogenesis of ATLL requires HTLV-1 infection, but only 4-5% of HTLV-1-infected individuals develop the malignancy, typically 10-30 years following infection. Diagnosis is made by serologic confirmation of HTLV-1 infection and identification of morphologically proven malignant cells with T-cell surface antigens. ATLL has 4 clinical variants: acute, lymphomatous, chronic, and smoldering, in descending order of incidence and worsening prognosis. Chronic and smoldering variants may be monitored without therapy, while acute and lymphomatous variants benefit from combination chemotherapy. Peritoneal Tuberculosis Associated with Tumor Necrosis Factor Alpha Inhibitor Therapy for Psoriasis LT Russell J Miller, MD; LT James C. Clifford, MD; LCDR Robert Carpenter, DO; CDR John S. Hammes, MD Department of Internal Medicine Naval Medical Center San Diego Introduction: Tumor necrosis factor alpha (TNF-О±) antagonists are effective tools in the treatment of rheumatic, dermatologic, and gastrointestinal inflammatory diseases. However, with increasing use of these drugs, adverse events have become more prevalent. One increasingly recognized complication is reactivation or development of Mycobacterium tuberculosis (TB) infections. We present a previously treated PPD-positive patient who developed TB peritonitis after receiving TNF-О± inhibitors. Case Presentation: A 57 year-old male with a history of longstanding psoriasis originally managed with topical agents, was given etanercept due to progressive skin lesions. The patient was a known PPD converter with a recent PPD in 2007 causing 20mm of induration. He denied history of active pulmonary TB, and reportedly received several months treatment for latent TB in 1975. During treatment with etanercept, chest radiography showed no evidence of active pulmonary TB. Three months after initial etanercept infusion, the patient presented with progressive fatigue, anorexia, and abdominal distention. A CT scan following admission revealed ascites and diffuse thickening of the greater omentum. Due to concern for malignancy a subsequent PET scan was obtained with showed multiple areas of increased uptake in the abdominal cavity consistent with tumor versus infection. Surgical biopsy was obtained for tissue histology, which demonstrated numerous non-caseating granulomatous foci in the omental tissue. Special stains were positive for acid-fast bacilli. The patient was started on combination drug therapy for treatment of TB peritonitis. Discussion: After numerous reports of re-activation and disseminated tuberculosis, the Food and Drug Administration issued a black box warning describing increased TB risk and need for screening prior to TNF-О± antagonists therapy. In addition, other serious adverse events including disseminated fungal infections and lymphoma were reported at rates exceeding the general population. However, much about the safety information of these agents is still unknown due to the increased rates of infection and malignancy associated with the underlying diseases as well as concomitant use of steroids and chemotherapeutic agents. Many dermatologist argue that the disease not the drug is responsible, resulting in poor compliance with universal screening recommendations. This case represents an increasing number of adverse events from using TNF-О± inhibitors in the treatment of psoriasis. Conclusion: Although TNF-О± antagonists are effective in the treatment of multiple inflammatory diseases, there is a growing recognition that these drugs do raise the risks of disseminated infection regardless of the underlying disease process. All physicians should understand the importance of both screening and surveillance before prescribing these drugs. Health care providers also must be aware that myocbaterial infections often present in atypical fashions in patients treated with these agents. Further review is necessary to develop a uniform policy to screen for both infectious and malignant complications of patients receiving biologic immune modulators. As well, we recommend initiation of a registry amongst all deployable personnel receiving these agents. Subcutaneous Panniculitis Like T-Cell Lymphoma Presenting as Optic Neuritis LT Russell J Miller, MD; CAPT Preston Gable, MD; George Luiken, MD Depts. of Internal Medicine and Hematology/Oncology Naval Medical Center San Diego Introduction: Optic neuritis is classically associated with multiple sclerosis (MS) with nearly half of MS patients having at least one episode in the course of disease. Despite this association, optic neuritis does not necessarily equate to a diagnosis of MS and can represent an isolated phenomenon, infection, connective tissue disease, or neoplasia. We present the first described case of optic neuritis as the presenting symptom of subcutaneous panniculitis like T-Cell lymphoma. Case Presentation: A 27 year old female presented to ophthalmology clinic with 5 days of right-sided central vision changes. Ophthalmological exam was consistent with optic neuritis without associated disk edema and the patient was admitted for IV steroids and MS work-up. Previous medical history was only remarkable for an incidental pulmonary nodule found on CT one month prior. Physical exam was notable for numerous non-tender subcutaneous nodules on all extremities measuring 0.51.5cm that had developed suddenly three weeks earlier. A brain MRI was unremarkable and CT chest showed resolution of previous pulmonary nodule with interval development of numerous new nodules. Biopsy of skin lesions, although non-diagnostic, showed features consistent with 44 subcutaneous T-cell lymphoma. Follow-up PET scan and bone marrow biopsy were negative. The patient chose to return to Texas to be close to family and for further diagnostic evaluation but was lost to follow-up. Five months later, the patient re-presented with bilateral visual changes progressing to complete blindness. Repeat MRI showed a large frontal brain mass with extensive edema. Craniotomy was performed for decompression and biopsy. Biopsy showed necrotic tissue with peripheral lymphocytic infiltration. The patientвЂ™s disease progressed and she died one month later. Discussion: Subcutaneous panniculitis like T-Cell lymphoma. is a rare type of non-HodgkinвЂ™s lymphoma which typically presents with deep subcutaneous nodules over the extremities and trunk. Often the diagnosis is missed because the lesions can resolve without treatment and reappear in other areas. Biopsy findings of skin lesions are usually non-specific and often have features which mimic benign panniculitis. We found only one similar case report of a 44 year-old man who presented with subcutaneous and pulmonary nodules initially responsive to steroids. In the course of his disease, he eventually developed neurological symptoms of mono-neuritis multiplex, but ocular nerve involvement has not previously been described. Conclusion: Although optic neuritis is thought to be associated with multiple sclerosis, other systemic processes should be considered in atypical cases including infectious, connective tissue, and malignant disorders. This case is the first one describing subcutaneous panniculitis like T-Cell lymphoma presenting as optic neuritis. A Caucasian Male with Multiple Syncopal Episodes: Sometimes the Hoofbeats are Zebras Laura Morgan, MD; Gregory Price, MD Naval Medical Center San Diego, CA Brugada Syndrome, also known as Sudden Unexpected Nocturnal Death syndrome, is a familial condition caused by dysfunctional cardiac sodium channels. It is responsible for nearly one-half of sudden deaths in healthly young individuals without structural heart disease in endemic areas. A 28-year-old active duty Caucasian male presented to the Emergency Department complaining of 11 days of pleuritic chest pain. EKG was noted to have features consistent with a Type II Brugada pattern (a вЂњsaddlebackвЂќ appearance of the ST segment with an ST elevation of 1 mm and incomplete right bundle branch block). Cardiology elicited a history of multiple unexplained syncopal episodes dating back to adolescence without associated prodrome or presyncope. At least one of these episodes had occurred during a shipboard deployment several years earlier resulting in facial trauma necessitating surgical intervention. Previous cardiovascular and neurological evaluations performed at an outlying facility revealed a normal echocardiogram, head CT, electrolytes, and thyroid stimulating hormone. The patient did not have a family history of sudden death, aborted sudden death, drownings, or dysrhythmias. He also denied any history of palpitations. During his initial NMCSD Cardiology consultation, the EKG demonstrated a Type II Brugada pattern in lead V2. Moving V1 and V2 up intercostal spaces did not result in a conversion to a Type I pattern. Review of his medical record from three years prior also demonstrated an EKG with a Type II Brugada pattern. Five days after consultation a Procainamide challenge was performed. At the conclusion of the 10 minute infusion (10mg/kg total dose), lead V1 demonstrated 2 mm of J-point elevation with a smooth, down-sloping ST segment which ended in a terminally negative T-wave (i.e. a Type I Brugada Pattern). Lead V2 had 2 mm of flat ST elevation. When these two leads were repositioned one intercostal space higher, both demonstrated Type I Brugada patterns, yielding a positive test. Based on his three presenting EKGs, multiple unexplained syncopal episodes, and a positive procainamide challenge test, Brugada syndrome was diagnosed. The patient subsequently underwent implantation of an automated implantable cardioverter defibrillator, in accordance with current American College of Cardiology guidelines. This case illustrates the need for clinicians to be familiar with specific EKG patterns which may be suggestive of an increased potential for malignant dysrhythmias, and guide specific therapeutic interventions. Although Brugada syndrome is thought to be a common cause of death in Asian men younger than 50 years, it can also affect males from other ethnic backgrounds. Bilateral Inguinal Masses in a 50 Year Old Female Margaret Moscato, MD; Christina Wang, MD Harbor-UCLA Medical Center A 50 y/o woman presented with a L inguinal mass that enlarged rapidly over the past 6 months. It was barely palpable and the same size as a mass in her R inguinal area for over 10 years. PMH: primary amenorrhea & lack of breast development. There was no facial hair, axillae were shaved, and there were bilateral breast implants. A 6 x 8 cm discrete, hard mass in the L upper inguinal area was mildly tender and a palpable mass deep in the R inguinal area was non-tender. There were labia majora & minora & clitoromegaly (1 cm in diameter at the base and 2 cm in length). The vaginal canal ended blindly (w/o cervix). CT showed 2 inguinal masses (R was 2.4 x 1 cm) & no uterus. She had mild anemia (Hgb 11.4 g/dl*), normal chemistry except LDH was slightly increased to 348 units (98-192), B-HCG increased to 54.4 mIU/ml (< 0.52.67), AFP-serum, 1.2 ng/ml (0-9.0). Serum gonadotropins were increased: LH 32.5 U/l (1.9-9.3), FSH 38.2 U/l (1.6-8.0). Serum steroid showed: increased androstenedione (A) 451 ng/dL (40-190), low testosterone (T) 96 ng/dL (250-1100), dihydrotestosterone (DHT) 20 ng/dL (25-75), increased testrone (E1) 154 pg/ml (< 68), low estradiol (E2)16 pg/mL (< 29); while DHEA 331 ng/dL (180-1250) DHEA-SO4 84 mcg/dL (25-240), 17 hydroxypregneolone alpha 33 ng/dL (20-450) were normal. Karyotype was 46, XY. Pathology: the L mass was a seminoma, stage T1NXMX, and the R was a testis with 45 severe tubular atrophy, loss of all germ cells & nodular hyperplasia of Leydig cells. Further genetic testing is pending. (*Male reference ranges are in parentheses.) The patient is a male pseudohermaphrodite (XY genotype, testes, & genital ducts or external genitalia not completely masculinized). Major categories of 46, XY disorders of sexual differentiation include: 1) androgen synthesis defects (affecting corticosteroids & androgens); 2) inability to convert T to DHT (5-alpha reductase 2 deficiency); and 3) androgen insensitivity (due to androgen receptor defects or post receptor signaling problems). T is synthesized from cholesterol via 5 main enzymatic steps. In the final step, A is converted to T by 17 betahydroxysteroid dehydrogenase 3 (HSD17B3) in the Leydig cells of the testes. increased levels of A and decreased levels of T support a Dx of HSD17B3 deficiency. Mean T:A ratio in one case series of patients with HSD17B3 deficiency was <0.4; this patientвЂ™s was 0.2. increased E1 compared to E2 levels further support the diagnosis since HSD17B3 also converts E1 to E2. Patients with HSD17B3 deficiency are born with ambiguous or female external genitalia. They may present because of primary amenorrhea, inguinal masses, or virilization during puberty. Some degree of virilization develops during puberty due to peripheral conversion of A to T by 17B-hydroxysteroid dehydrogenase-5. A similar phenotype may be seen with 5-alpha reductase deficiency; however, the T:DHT ratio is high (>20: 1). Males with complete androgen insensitivity do not virilize at puberty and develop female secondary characteristics from conversion of androgens to estrogens. The gene for HSD17B3 is on chromosome 9q22; 20 mutations have been reported. The deficiency is autosomal recessive; females are asymptomatic. Treatments at a younger age include estrogen therapy and/or corrective surgeries (clitoroplasty or vaginal dilation). Removal of the cryptorchid testes is done to avoid neoplastic transformation, as occurred in this patient. Endobronchial Hodgkin Lymphoma Presenting as a 3rd Cranial Nerve Palsy Ameer Mousssa, MD; Nadar Kamangar, MD, FACP; Nikhil Barot, MD Olive View-UCLA Medical Center Endobronchial Hodgkin Lymphoma is rare and should be part of the differential in any patient that presents with pulmonary mass and associated symptoms. What about those patients who present with seemingly unrelated symptoms? It is up to the clinician to determine the relevance of every aspect of the history and physical as they are presented and act accordingly. A 25 year-old woman presented to neurology clinic with history of severe headaches, which started 10 days prior & awakened her from sleep, as well as left eye swelling and diplopia that started the previous day. She was diagnosed with 3rd nerve cranial palsy and sent to the emergency room for further evaluation. On exam, there was a noticeable 3rd nerve cranial palsy with ptosis of the left eye, horizontal nystagmus, decreased air entry on the right lower lung fields in the mid-axillary region and fine crackles; there was no miosis, anhydrosis, or JVD. She denied SOB, dizziness, convulsions, fevers, nausea or vomiting, no recent travels nor a history of STDвЂ™s. An MRI/MRA of the head was done to rule out any obvious neurologic causes; which came back negative. A chest x-ray was done which showed a large mediastinal mass that infiltrated the right lung. On further investigation she was found to have a nonproductive cough and night sweats that had occurred for the past four to five months; which she thought was irrelevant. CT of the chest was completed and showed an extensive heterogeneous anterior mediastinal mass extending into the mid-mediastinum, paratracheal area and the right perihilar region with partial collapse of the RML and RLL, random lower lobe nodules, bilateral adrenal and renal masses. Brochoscopy showed a large exophytic lesion that obstructed the right middle bronchus. Biopsies were taken showing Hodgkin Lymphoma. Lumbar puncture showed no malignant cells, normal glucose and cell count. She was then sent home with follow up to the oncology clinic where the appropriate outpatient regimen would be started to treat the endobronchial Hodgkin lymphoma. This case shows the importance of a proper diagnosis and staging of mediastinal masses; as well as the importance of proper history taking. The patient was being evaluated for possible causes of a 3rd cranial nerve palsy with associated ptosis; was found to have pulmonary symptoms and a large mediastinal mass that infiltrated the right lung. Endobronchial Hodgkin lymphoma was found; which is rare and could be mistaken for more worrisome lesions that have worse outcomes. With proper diagnosis, an appropriate treatment plan can be devised and instituted with good prognosis. Ribavirin and Peginterferon in a DRESS Sheila Naghshineh, MD; Habibollah Alamdari, BA; Pooya Bokhoor, MD; Sammy Saab, MD, MPH; Delphine J. Lee, MD, PhD University California, Los Angeles DRESS syndrome (drug reaction with eosinophilia and systemic symptoms), is a serious idiosyncratic druginduced skin eruption presenting with fever & lifethreatening organ involvement. The delayed onset & severity of the reaction makes its diagnosis critical in preventing patient morbidity & mortality. A 64 year old man with hepatitis C, HTN, type 2 DM, and stage III CKD presented with malaise, fevers to 39В°C, and a worsening diffuse pruritic rash for 16 days. He had nonpurulent, nontender confluent erythematous, scaly plaques with scattered papules predominantly on the trunk and extremities. Labs revealed 33% peripheral eosinophilia & serum Cr 5.2 mg/dL (GFR 11, a 63% reduction from baseline). Skin punch biopsy showed mixed interface & spongiotic dermatitis accompanied by a superficial and deep perivascular mixed inflammatory infiltrate with eosinophils. There was no evidence of vasculitis, malignancy, or infection. His symptoms began 10 weeks after 46 starting ribavirin and peginterferon alfa-2a treatment for hepatitis C. The patient had no known drug allergies. The clinical presentation & pathology results supported a diagnosis of DRESS syndrome associated with acute tubular necrosis. Treatment began with a corticosteroid cream and antihistamines for the rash. The offending drugs had been discontinued 2 weeks prior to presentation. Since the rash was not significantly improving, IV corticosteroids were administered. He had a rapid response with the IV steroids. His rash began to resolve, his peripheral eosinophilia approached normal levels, and his renal function improved. IV corticosteroids were discontinued after 3 days and topical corticosteroid cream and antihistamines were continued. Within 10 days, the rash resolved and his renal function returned to baseline. This case highlights the potential association of ribavirin and peginterferon alfa-2a treatment with DRESS syndrome. Although these adverse effects are rare, early recognition is essential to formulate definitive therapy and prevent severe organ damage. Leather Fever Sheila Naghshineh, MD; Lobna Shahatto, BA; Suzanne Donovan, MD, MPH University of California, Los Angeles Coxiella burnetii, the strictly intracellular, gram-negative bacterium causing Q fever, is often latent in animals, primary reservoirs being species of mammals, birds, and ticks, with persistent shedding of bacteria. Humans are usually infected by contaminated aerosols from domestic animals, such as cattle, sheep, goats, and pets. We present a case of a 60 year-old gentleman, in the processed leather repair industry, who presented with 3 weeks of generalized malaise, daily fevers up to 40Вє C, chills, non-pruritic migrating erythematous rash, nonproductive cough, and a 5 lb weight loss. On physical examination, the lesions consisted of well-circumscribed erythematous macules and papules, ranging from 1-10 cm in diameter, waxing and waning in size and color. Labs were significant for elevated liver enzymes, with Aspartate Aminotransferase (AST) 155 and Alanine Aminotransferase (ALT) 147. Alkaline Phosphatase and Total Bilirubin were within normal limits. Studies for Histoplasma, Coccidioides, Cryptococcus, Acute Viral Hepatitis, Rocky Mountain Spotted Fever, Brucella, and HIV were all negative. Right upper quadrant ultrasound was unremarkable. The patient was initially placed on vancomycin. His lower extremity rash showed some improvement; however, he continued to have daily fevers. The patient subsequently underwent a bone marrow biopsy, which showed no evidence of infection or infiltrative disease. Rheumatologic work-up for Still's disease, Lupus, and Rheumatoid Arthritis were also negative. Given his persistent fevers and transaminitis, the patient was started on doxycycline four days after presentation, and he subsequently defervesced in two days. Later, his Coxiella burnetii serology was consistent with an acute infection, as both the phase I and Phase II titers were positive, with the Phase II titers being greater than Phase I. The patient was discharged with a ten day course of doxycycline. At his three-week follow-up appointment, he was asymptomatic with resolution of his rash and normalization of his liver enzymes. The case illustrates the value of taking a complete employment history and the connection between handling processed leather and Q fever. His clinical presentation also highlights the dermatologic manifestations of Q fever, which resemble rheumatologic, malignant and other infectious etiologies. Clues of the Brugada Syndrome Elisa Ng, MD University of California, San Diego Brugada syndrome is characterized by a high incidence of sudden death and one of several electrocardiogram (EKG) patterns with an incomplete right bundle branch block and ST-segment elevations in the anterior precordial leads. The typical patient with Brugada syndrome is a young, otherwise healthy male with normal physical exam findings. A 30 year-old Caucasian male with depression on citalopram and attention-deficit hyperactivity disorder presented to the emergency department shortly after an episode of generalized weakness. At the time of the incident, the patient was pushing shopping carts at work when he experienced palpitations associated with diffuse involuntary muscle relaxation, followed by 10-15 minutes of profound weakness. He had experienced similar episodes on two other occasions in the past 5 months. His presenting vital signs and physical examination were unremarkable. EKG showed normal sinus rhythm at 75 beats per minute, with a large RвЂ™, 1mm ST-segment elevation, and upright T wave in V2. Labs were unremarkable including negative cardiac markers. The patient was referred to Cardiology for further evaluation. Repeat EKGs showed resolution of the prior changes. Work-up is ongoing, and the patient is scheduled to undergo a flecainide challenge and holter monitoring to confirm Brugada syndrome. This case illustrates helpful clues in recognizing the Brugada syndrome. Although syncope is a common manifestation, some patients are asymptomatic and the diagnosis is made based on routine EKG. The classic Type 1 pattern consists of an accentuated J wave, coved-type STsegment elevation, and negative T wave in V1-V3. The Type 2 pattern consists of right precordial ST-segment elevation в‰Ґ1mm and upright T wave forming a saddleback configuration. Type 3 is similar to Type 2 but with <1mm ST-segment elevation. Our patientвЂ™s EKG was most consistent with the Type 2 pattern. These EKG patterns can be intermittent, making diagnosis more difficult. The pathophysiology of Brugada syndrome involves a defect in myocyte voltage-gated sodium channels that alters sodium flux in early repolarization, thereby increasing risk of ventricular arrhythmias. Acquired Brugada 47 syndrome may be triggered by medications such as antiarrhythmics or psychotropic drugs (including selective serotonin reuptake inhibitors) that inhibit sodium channels. Suspicion of Brugada syndrome in this patient was based on his history of weakness and palpitations, SSRI administration for depression, and transient Type 2 EKG findings. Administration of sodium channel blocking agents can help induce Brugada patterns and arrhythmias on cardiac monitoring. Because Brugada syndrome is one of the leading causes of sudden death in men under age 40, patients found with a Brugada EKG pattern should be referred immediately for further electrophysiology studies and considered for an implantable cardiac defibrillator when the diagnosis is confirmed. Gurgling in the Chest: An Unexpected Finding of BochdalekвЂ™s Hernia Visal Nga, DO; James Keefe, MD; Ann Marie Levan, MD; Patrick Cahill, MD; Jasminka Criley, MD St. Mary Medical Center and David Geffen SOM at UCLA Case Description: A 70 year old Caucasian female with past medical history of hypertension, and GERD presented with coffee ground emesis. She was on her annual cruise ship enjoying breakfast when she started having nausea and vomited blood. She denied abdominal pain, difficulty or pain with swallowing, shortness of breath, chest pain, or cough. On physical examination, T 98oF, RR 20, Blood pressure 102/51, Heart rate 114, Saturation 89 % on room air. There was dried coffee ground blood at the corner of her mouth. Cardiovascular and left lung examinations were normal. There was absence of breath sounds over mid and lower parts of the right hemithorax, with tympanic percussion and prominent gurgling sounds over the same area. The abdominal exam was normal. Admitting Laboratories were significant for hemoglobin 10.6, hematocrit 32, BUN 61 and creatinine 1.7. A chest x-ray revealed a right base infiltrate and hernia. Patient was started on Ceftriaxone, levaquine and clindamycin for possible aspiration pneumonia. She was given Protonix 40 mg IV bid for gastrointestinal bleeding and was admitted to the Intensive Care Unit. An upper endoscopy revealed diffuse esophageal ulcerations, which were the source of her bleeding, giant hiatal hernia with free reflux of gastrointestinal content, J-shaped stomach and edematous mucosa of duodenal bulb. Subsequently, we ordered an abdominal CT scan with oral contrast to better visualize the anatomy, which revealed a Giant Hernia extending in the right thoracic cavity. Her gastrointestinal bleeding stopped and she was transferred out of the ICU. During the rest of the hospital course, she remained asymptomatic. After considering surgical vs. non-surgical treatment, it was decided that the best option for her at this point was observation rather than surgical intervention. She was discharged to a boarding care home on Omeprazole and Ranitidine. Discussion: Our patient had a congenital defect in the diaphragm, i.e BochdalekвЂ™s hernia with the entire stomach herniating into the right thoracic cavity. Diaphragmatic hernia can often be missed. A correct diagnosis remains difficult and is usually made late. Our patient had long standing history of GERD. Her BochdalekвЂ™s hernia was diagnosed only after she developed severe ulcerative esophagitis and gastrointestinal bleeding. Diagnosis of diaphragmatic hernia may not have been entertained earlier because it is rare and may have misleading clinical features. Possibility of BochdalekвЂ™s hernia with delayed presentation should always be kept in mind in the differential diagnosis of adult with GERD. Earlier recognition of the BochdalekвЂ™s hernia may lead to earlier surgical intervention and/or more aggressive medical management of GERD; thus potentially slowing the progression of ulcerative esophagitis and other complications. PandoraвЂ™s Box: HIV, Disseminated KS, MDS and Gangrene-Complication on Complication Visal Nga, DO; Howard Van Gelder, MD; Benjamin Montoya, MD; James Kefee, MD; Joyti Datta, MD; Chester Choi, MD; Jasminka Criley, MD St. Mary Medical Center and David Geffen SOM at UCLA Case Presentation: PandoraвЂ™s Box, a gift from the Greek God Zeus, once opened will unleash evil on mankind. HIV, once controlled with HAART, but when stopped, can progress beyond control. We present a case in which stopping HAART led to Disseminated Kaposi Sarcoma (KS), Myelodysplastic Syndrome (MDS), and Severe Gangrene leading to the demise of our patient. A 45 year old Caucasian man with HIV/AIDS for 20 years, presented with generalized weakness and pain in his feet. Two months prior, he was diagnosed with AIDS related KS. He had discontinued HAART about 2-years ago. His skin lesions were violaceus, but started turning black after he received a one time dose of radiation for KS in his feet. He had dark tarry stools with intermittent diarrhea for several months. On admission he was pale, febrile, with diffuse purple-nodular lesions on the skin of upper and lower extremities, with swollen feet and edema to the ankles. The toes had multiple black ulcerations; yellow, purple discoloration; nails covered with white plaque, and serosangineous fluid from the ulcerations consistent with acute gangrene. LABS: WBC 26,000, Hb 4, platelet 25, BUN 112, Cr 2.25 and INR 1.81. EGD revealed bleeding gastric mucosal ulceration and KS lesions in duodenum that required multiple transfusions. Persistent thrombo-cytopenia led to a bone marrow biopsy which revealed MDS. Blood sepsis from MSSA, VRE, & E. coli was treated with antibiotics. MRI showed soft tissue swelling without osteomyelitis. The patient became afebrile and was restarted on HAART therapy. In addition to superficial debridement, amputation was recommended for definitive treatment of his gangrene, which the patient declined. Chemotherapy could not be instituted due to active infection and low platelets. Due to his poor prognosis, the patient decided on comfort care 48 only and subsequently died from respiratory failure due to pneumonia and pulmonary KS. Discussion: KS is a spindle-cell tumor likely derived from endothelial cell lineage seen most often in patients with HIV/AIDS. Current frequency in the United States is listed at 2500 cases/year. Our patient was initially controlled with HARRT, but developed aggressive disseminated KS after stopping HAART. His foot lesions, likely gangrene, were amongst the most serious complications, leading to sepsis. Complicating matters is the patientвЂ™s statement that his foot lesions did not worsen until initial treatment with local radiation, even after restarting HAART. Is it possible that the patient had a paradoxical reaction to radiation therapy? KS can cause gangrene, but there is no substantial literature that gangrene caused from low radiation doses typically used for treating KS lesions. His rapid decline and worsening gangrene were likely also attributable to his severe preexisting conditions (MDS, high HIV viral load, extensive tumor burden). Though rare, epidemic KS is still present in the HAART era. The case shows that patients with advanced KS can be a true вЂњPandoraвЂ™s Box,вЂќ вЂ“once opened, the possible complications and organ systems involved may be limitless. An Atypical Form of Lung Cancer: Intrapulmonary Schwannoma Jennifer Le Nguyen, MD; Jennifer I. Chang, MD; Grant Lee, MD Kaiser Permanente Los Angeles Case: A 70 year old gentleman was admitted to the hospital with a chief complaint of orthopnea, several week history of shortness of breath and mild, productive cough. His past medical history is significant for chronic pulmonary obstructive disease (COPD). He had been in his usual state of health until he returned home from a vacation to Cabo San Lucas, Mexico. Upon return from his trip, he was with subjective fever, cough, and mild production of white sputum alleviated with home nebulizer therapy with bronchodilators. Due to persistence of symptoms, he presented to his primary care physician and was prescribed an outpatient therapy of bronchodilators, a short course of prednisone, and oral antibiotics. His symptoms initially improved but recurred and persisted intermittently for an additional two weeks. This patient ultimately presented to the emergency department after experiencing new onset orthopnea in association with his previous two week history of cough and shortness of breath. He was initially diagnosed with an acute COPD exacerbation and treated with IV steroids, antibiotics, and nebulized bronchodilators. His workup for cardiac etiology and infectious precipitants was unremarkable. The patient was further evaluated with computed tomography (CT) scan of the thorax. CT was remarkable for a distal endotracheal tumor approximately 2.2 by 1.4 centimeters in size with near obstruction of the lumen. He was taken to the bronchoscopy suite for biopsy. During the procedure he developed respiratory distress with near obstruction of the airway. He was brought emergently to the operating room for rigid bronchoscopy. The majority of the tumor was removed with the rigid bronchoscope. Remainder of hospital course was unremarkable and patient was discharged to home. Discussion: Primary pulmonary schwannoma is an extremely rare intrapulmonary, neurogenic tumor. This rare condition was first reported in 1940 and subsequently, less than several hundred cases have been identified. The symptoms and radiographic manifestations may be variable and nonspecific. Patients with history of underlying pulmonary disorders such as asthma or chronic pulmonary obstructive disease (COPD) may obscure the clinical manifestations of this rare condition, leading to delayed diagnosis. In this clinical vignette, we present a 60 year old gentleman with a history of COPD presenting with a several week history of shortness of breath and stridor. He subsequently underwent bronchoscopy and diagnosed with primary pulmonary schwannoma confirmed on pathology obtained from transbronchial biopsy. In this clinical vignette, we provide a clinical analysis and review of the current literature on this rare condition. Nodular Regenerative Hyperplasia: A Rare and Silent Cause of Noncirrhotic Portal Hypertension Jennifer Le Nguyen, MD; Brandon Beaber, MD; Theodore Sy, MD Kaiser Permanente Los Angeles CaseS: In this case series report we present two patients with a history of HIV presenting with progressive abdominal distension. The first patient is a 63 year old gentleman with a two month history of progressively worsening abdominal distension. His past medical history is significant for a history of HIV in which he had previously been compliant with anti-retroviral treatment: Kaletra, Didanosine, and Atazanavir. The second patient is a 42 year old female presenting with a two to three month history of progressive, painless abdominal distension. Her medications included Didanosine, Truvada, and Kaletra. Workup for both patients was extensive in evaluating for etiology of portal hypertension and included evaluation of viral and autoimmune hepatitis etiologies, liver transaminases, and evaluating for malignancy with imaging studies such as ultrasound and computed tomography. Management included diagnostic and therapeutic paracentesis, liver biopsy and esophagogastroduodenoscopy. Liver biopsy with reticulin staining was significant for findings consistent with nodular regenerative hyperplasia in both patients. Following diagnosis via liver biopsy and alterations in anti-retroviral treatment, including cessation of didanosine, there has been an interval improvement of ascites and abdominal distension in both patients. Discussion: Nodular regenerative hyperplasia (NRH) of the liver is a rare cause of noncirrhotic portal hypertension in adults and children. This uncommon condition is characterized by diffuse transformation of normal hepatic parenchyma into small, regenerative nodules. Although it 49 is a rare cause of portal hypertension, it is one of the main causes of noncirrhotic portal hypertension in the Western world. NRH has been reported to occur in association with certain medications, rheumatologic disorders, vascular disorders, and myeloproliferative disorders. In this clinical vignette, we present two patients and their initial presentations leading to the diagnosis of NRH in both patients. The first is a 63 year old gentleman presenting with a two month history of progressively worsening abdominal distension and bloating and diagnosed with NRH. The second patient is a 42 year old female presenting also with a two to three month history of progressive, painless abdominal distension. We also review the hypothesized pathogenesis, histopathology, current literature, and varying management options in a rare condition that can lead to noncirrhotic portal hypertension and associated serious and longterm sequelae such as variceal bleeding, ascites, and splenomegaly. Anemia, Epigastric Pain, and Itching in an HIV+ Patient Sean Novak, BS University of California, Los Angeles Introduction: Itching is an unpleasant sensory experience which elicits a desire to scratch. Unfortunately, because itching is a non-specific and seemingly benign symptom, it is often dismissed as secondary to dry skin or a reaction to laundry detergent and treated with diphenhydramine or a dermatology followup. However, itching can be an essential clue to a number of dangerous and occult conditions. Case: A 42-year-old man with history of HIV infection presented with a 5-6 month history of fatigue, epigastric and pleuritic pain, night sweats, and generalized itching. Additional history revealed an episode of dark, sticky stool approximately two months previously, and an episode of Pneumocystis jiroveci pneumonia one year earlier. On physical exam, he was febrile to 100.6 and tachycardic, with orthostatic hypotension, pale conjunctivae, and lymphadenopathy of posterior auricular and axillary nodes. He had bibasilar pulmonary crackles, tenderness over the epigastric region without rebound or guarding, and a rectal exam negative for gross blood. Labs revealed hemoglobin 7.4 g/dL (normal 13.5-17.5) with inadequate reticulocyte compensation, CD4 count of 18 (normal 500-1600), and occult blood tests negative times three. Creatinine and blood urea nitrogen were elevated. Liver function tests were within normal limits. A chest xray was clear. Two units of blood were transfused with an appropriate hemoglobin increase. Diphenhydramine was administered in hopes of alleviating the patient's itching, but had only a minor effect. Lymph node biopsies were considered but deferred until the patient was hemodynamically stable. Gastroenterology was consulted and performed an upper endoscopy which revealed multiple gastric ulcers. Biopsies were taken and sent for analysis, with high suspicion for cytomegalovirus gastritis given the immunocompromised state. A proton-pump inhibitor was started. Ulcer biopsies were found to be positive for lymphoma, diffuse large B cell type. PET-CT imaging for staging revealed uptake in the stomach and focal areas in the lung and bowel, as well as diffuse lymph nodes in the axillary, cervical, and inguinal regions. A bone marrow biopsy was performed and confirmed marrow involvement. Oncology soon began treatment with R-CHOP chemotherapy. Discussion: This case demonstrates how seemingly minor complaints can be clues to major illness. In regards to this patient with a GI bleed and anemia, it would have been easy to treat the acute issues, and dismiss the itching to a primary care physician followup visit. Doing so neglects the patient's comfort level as well as the underlying condition. While this patient's itching, epigastric pain, and anemia were all related, in another patient each problem could easily be due to a separate pathologic process. Itching can be due to malignancies, renal disease (uremia), cholestasis, infections (dermatophytes, scabies), as well as the more well known causes such as dry skin, contact/atopic dermatitis, urticaria, and psoriasis. A Case of Amiodarone-Induced Thyrotoxicosis with Severe Myopathy and Dysphagia Ashley Ono, MD Scripps Mercy Hospital Case report: A 69 year old Caucasian male with a history of non-ischemic cardiomyopathy (EF:20%) s/p biventricular AICD placement in 2005 presented with severe weakness, lightheadedness, significant weight loss and dysphagia. Due to repeated AICD firing from recurrent VT, he was placed on amiodarone 3 years prior. On initial evaluation, TSH was undetectable, FT4 and FT3 were markedly elevated and CK was normal. Discussion: Amiodarone is a widely used, potent antiarrhythmic drug with significant side effects on non-cardiac organs of which thyroid dysfunction is a frequent manifestation. Although most commonly associated with hypothyroidism, amiodarone-induced thyrotoxicosis (AIT) occurs in 3% of patients and is associated with adverse cardiac events. Amiodarone induced thyroid dysfunction occurs as a result of combined intrinsic and iodineinduced effects. Type 1 AIT is an iodine-induced hyperthyroidism that occurs in patients with underlying thyroid disease (i.e. GravesвЂ™ or multinodular goiter) as a result of increased substrate delivery and unregulated hormone synthesis. Type 2 AIT, is a drug-induced destructive thyroiditis that occurs due to release of pre-formed thyroid hormone. Differentiation of AIT, although often difficult, is essential to initiating appropriate therapy as Type 1 AIT requires anti-thyroid medication while Type 2 AIT is responsive to steroids. Frequently, thyroid ultrasound 50 with Doppler flow is used to distinguish type of AIT, however, TSI, IL6, and radio-iodine uptake scans can also be used. Upon diagnosis of AIT, continuation of amiodarone therapy must be carefully considered. While therapy may be continued in Type 2 AIT, current recommendations support the cessation of therapy, if possible, for those with Type 1 AIT. Ultimately, the risk of unstable, fatal arrhythmias must be weighed against those of thyrotoxicosis. described. The use of furosemide could not account for each episode of hypokalemia in this patient. While elevated aldosterone levels can cause hypokalemia in cirrhotics, her serum sodium remained in the low normal rang. She did not respond to spironolactone. She eventually developed a distal Type 1 RTA during the final hospitalization for end stage liver disease. Additionally, thyrotoxicosis is also associated with neurologic manifestations, including myopathy. Approximately 60-80% of patients with untreated hyperthyroidism experience myopathy manifested as proximal muscle weakness. In rare cases, those suffering from acute thyrotoxic myopathy may present with severe proximal and distal weakness and bulbar dysfunction. While features including increased cellular metabolism, increased protein degradation and decreased muscle carnitine contribute to myopathy, the pathogenesis remains unknown. In this case, cardiopulmonary exercise testing, EMG and nerve conduction studies were used to elucidate the cause of our patientвЂ™s myopathy. The cause of our patientвЂ™s myopathy was confounded by side effects of his concurrent medical therapy with amiodarone and simvastatin. Winnie Pang, MD Kaiser Permanente Southern California Recurrent Symptomatic Hypokalemia in an Outpatient with Cirrhosis Katie OвЂ™Sullivan, MD; Susan E. Trompeter, MD, FACP University of California, San Diego Furosemide has been used frequently in the treatment of ascites. Hypokalemia may subsequently occur as an adverse reaction. In addition, hypokalemia presumably due to an increase in aldosterone can be seen in cirrhotics with ascites. In 2004, a 47-year-old woman presented with encephalopathy, jaundice, and ascites and was diagnosed with end stage liver disease secondary to alcohol abuse. Liver biopsy showed chronic hepatitis with severe fibrosis. The patient recovered clinically and was placed on a transplant list. She, however, was lost to follow up and continued to drink. In 2008, she presented to the GI clinic at our institution with jaundice, elevated INR, and thrombocytopenia. Abdominal CT scan revealed a markedly cirrhotic liver. She returned with ascites and weight gain and was placed on spironolactone with the addition of furosemide. Furosemide was subsequently discontinued due to presyncopal events. Over the course of the next 12 months, the patient had recurrent hypokalemia ranging from 2.8 to 3.0 presenting clinically as muscle cramping despite 20 - 30 MEQ of KCL daily. Spironolactone between 50 вЂ“ 100 mg daily was also prescribed during this time. The use of furosemide 10 mg was infrequent and did not account for episodes of hypokalemia. She presented with jaundice and acute renal failure and was diagnosed with potassium-wasting renal tubular acidosis as an inpatient prior to her death. This case describes frequent episodes of symptomatic hypokalemia in a woman with cirrhosis, a treatment challenge not well Central Pontine Myelinolysis: Not Just about Sodium Introduction: Central pontine myelinolysis (CPM) is a rapidly progressive neurological syndrome characterized by symmetrical, non-inflammatory demyelinization in the central base of the pons. CPM is often associated with rapid correction of hyponatremia but is also seen in many other conditions. Case: A 64 year old African American male with a past medical history of COPD and a 25 pack year history of smoking presented to the emergency department with gait instability, dysarthria, and bilateral upper and lower extremity tremors for 1 week. Review of systems was positive for fatigue, anorexia, unintentional weight loss, dry cough, and hallucinations. On admission, the patient was afebrile and vital signs were stable. PE was remarkable for clubbing. Neurological exam revealed dysarthria, truncal and appendicular ataxia, left upper extremity weakness, and absent reflexes throughout. Laboratory data was significant for a sodium level of 129 mEq/L, compared to 133 mEq/L 3 months prior, and an albumin of 2.8 g/dL. Urine osmolality was increased at 562 mOsm/L and serum osmolality low at 258 mOsm/kg. After excluding other causes of hyponatremia, the patient was felt to have euvolemic hyponatremia due to SIADH, which was treated with fluid restriction. An MRI of the brain was performed, revealing increased FLAIR and T2 signal intensity in the bilateral pons suggestive of central pontine myelinolysis. CT chest showed a 5.1 x 2.7 cm subcarinal soft tissue density. Transbronchial biopsy of the subcarinal mass was consistent with small cell lung carcinoma. During hospitalization, the patient showed no clinical improvement. He was scheduled to start chemotherapy for small cell carcinoma as an outpatient. Discussion: In this case, the patient presented with a myriad of neurological and constitutional symptoms, along with hyponatremia likely due to SIADH. These findings were highly suggestive of a paraneoplastic syndrome. Interestingly, CPM was discovered through neuroimaging and, later, small cell lung carcinoma was confirmed on biopsy. This case is unusual in that CPM developed without the rapid correction of hyponatremia and was likely multifactorial, occurring in the setting of occult malignancy, hyponatremia from SIADH, and malnutrition. It highlights the importance of considering lesser known etiologies for CPM. 51 CPM was originally described in 1959 by Adams et al. in patients with chronic alcoholism and malnutrition. However, it is now typically linked with the correction of severe, chronic hyponatremia. Other conditions associated with CPM include liver failure, organ transplant, burns, and chronic kidney disease. CPM is hypothesized to be related to changes in osmotic forces causing cellular edema and degeneration of myelin sheaths. Manifestations may vary, with symptoms including pseudobulbar palsy, ataxia, spastic para- or quadraparesis, seizures, diminished reflexes, lethargy, delirium, and a вЂњlocked-in syndrome.вЂќ Outcome is variable, from complete resolution of symptoms to no improvement and death. Treatment is supportive. A Rare Case of an Inflammatory Myofibroblastic Tumor of the Bladder Masquerading as Everything But Christopher Parrish, MD; Elaine Ku, MD; Vanita Chiang, MD; Brian Jason Brotherton, MD; Michael Welker, MD University of Southern California acid-fast bacilli. Concern regarding the potential for malignant conversion of urachal remnant tumors prompted elevective surgical excision of the mass with bladder preservation. On gross examination and intraoperative frozen section no malignancy was identified and a preliminary diagnosis of urachal cyst was made. However, the final pathologic diagnosis was inflammatory myofibroblastic tumor. Discussion: Inflammatory myofibroblastic tumors (IMTs) are rare tumors known to occur in the lungs, intestines, breast, kidneys, and rarely, the bladder. IMTs are commonly mistaken for other entities due to their underrecognition amongst surgeons, radiologists, pathologists, and internists. A history of bladder instrumentation and recurrent urinary tract infections have been linked to the development of IMTs. Urinary tract infections in males over one year of age are rare and should always prompt further workup in a man with no other predisposing factors. Heightened awareness of the existence and varied presentations of IMTs acan lead to prompt diagnosis and proper treatment. 5150 HOLD - Not Always Psychiatric Introduction: Inflammatory myofibroblastic tumors (IMTs), also known as plasma cell granulomas, are rare tumors of unknown etiology. IMTs represent a diagnostic challenge because of their infrequent occurrence and potential to mimic numerous malignancies. Case: An 18-year-old man with no significant past medical history presented with a 1-year history of a lower abdominal mass. He initially sought care for new-onset dysuria and lower abdominal pain associated with a palpable mass below the umbilicus. Computerized tomography (CT) of the abdomen and pelvis showed a mass above the urinary bladder, adjacent to and involving the rectus muscles, felt to be a hematoma. A urinary tract infection was diagnosed clinically, and the patient reported resolution of all symptoms after antibiotics. Two months prior to admission, the expanding abdominal mass recurred, accompanied by dull lower abdominal pain. No history of weight loss, fatigue, fevers, hematuria, change in bowel patterns or family history of cancer was elicited. The patient consulted his physician again and was referred for imaging. An umbilical hernia and an irregular mass superior to the bladder that appeared to be a hematoma involving the rectus muscles was seen on MRI. The patient was referred for surgical and hematological evaluation and presented to a tertiary care center for an alternate opinion. Vital signs were normal. Physical examination was unremarkable except for a 6-cm nontender mass palpable in the midline of the abdomen inferior to the umbilicus. No lymphadenopathy was noted. All laboratories, including serum creatinine and hemoglobin, were normal. Urinalysis was also unremarkable. Repeat MRI showed a complex mass anterior to the bladder. Differential diagnosis was considered to favor urachal remnant tumor versus primitive neuroectodermal tumor. CT-guided biopsy of the mass showed only granulation tissue formation and chronic inflammation, with negative stains for Kalpesh Patel, MD; Marian Kaldas, MD Olive View-UCLA Medical Center Learning Objective: Important differential to consider in acute psychosis-- Infectious vs. Metabolic vs. Autoimmune vs. Psychiatric vs. Neurological diseases vs. Malignancy vs. substance abuse Case Information: 23 y/o Hispanic male, no PMH, presented to psych ER with 3 days history of sudden change in behavior, delusional thoughts, generalized weakness and headache. He was alert and oriented x 2 and placed on 5150 hold for danger to self and others with combative behavior. Medicine consult was requested and patient admitted to medicine service to rule out other causes of acute psychosis. He was born in Mexico, vaccinated up to date, intermittently visiting Mexico every 6 months and admits having stressful relations with wife and at job, no family history of medical, psychiatric problems. Patient continues to be altered and delusional on floor so 5150 hold was continued and placed on 2+ intermittent soft restraints. Patient had a normal vital signs except tachycardia, normal PE findings.Chemistry, CBC, LFTs, TSH were normal. Serum and urine toxicology, CXR, urine analysis, troponin, CT chest angiogram, blood/urine cultures (-). EKG showed sinus tachycardia. LP was done prior to antibiotics that revealed no WBC, normal glucose and slightly elevated protein and negative infectious work up including gram stain & culture, viral culture, CSF VDRL, Indian ink, HSV DNA PCR, and WNV. MRI brain and MRA brain and neck were negative. RPR, HIV, Hepatitis panel was negative. On recommendations of Neurology and ID services, further work up including Antithyroglobulin Abs, Urine porphyrins, ceruloplasmin, Testicular exam, ASO titer, mononucleosis Ab, Lyme antibody, EBV serum titer, CSF EBV PCR, CSF cysti- 52 cercosis Ab, Urine heavy metal screen, CD4 count, ACE levelвЂ“all were negative. ESR 47(slightly elevated), EEG was normal. Repeat Lumbar puncture and Brain imaging negative. Autoimmune work up including ANA, Anti Smith Abs and Anti SSA were positive with low C3, C4 and Total Complements. Patient was diagnosed with lupus cerebritis with neuropsychiatric manifestations and placed on pulse dose of steroids; all other antibiotics and antiviral discontinued. Patient did not respond to pulse dose steroids, started on IV soulmedrol high dose вЂ“ followed by IV cyclophoshamide (CYTOXAN) one dose with no improvement in mental status. Later on he was treated with IV Immunoglobulin over a period of 4 days. Patient responded to IVIG very well and mental status improved. His Psychiatric hold was discontinued and discharged home with subsequent follow up in primary care and rheumatology. On subsequent follow up visit in outpatient clinic he was back to his normal behavior and normal life. Discussion: SLE is a chronic inflammatory disease of unknown cause which commonly affects skin, joints, kidneys, lungs, serous membranes. It is predominantly common in female in their 20s and 30s than men. It is usually presents with systemic signs of inflammation and clinical findings. First presentation of acute severe psychosis secondary to lupus cerebritis is very rare. This case highlights the unusual findings of Lupus and one important diagnosis to consider in the differential diagnosis of acute psychosis since we, as an internist/primary care physician, are getting so many psychiatric patients admitted to medicine service to rule out medical causes of psychiatric problems. A Case of the Iron Heart Jonathan A. Peng, MD; David Chang, MD; Arnold Baas, MD University of California, Los Angeles Case: A 48 year old female with end stage liver failure from underlying hepatitis C complicated by refractory ascites and severe encephalopathy received a liver transplant and within two days developed extensive hypotension and renal failure. She was started on vasopressors however eventually required an intraaortic balloon pump (IABP) to maintain blood pressure. The initial differential included sepsis, cardiac damage from vasopressors, or a direct cardiac effect by the hepatitis C virus. Echocardiography done prior to the transplant was within normal limits, however at that time revealed a left ventricular ejection fraction (LVEF) of 13%, right ventricular ejection fraction (RVEF) of 30%, and biventricular dysfunction consistent with dilated cardiomyopathy. In the next few days, the patient continued to be hypotensive and was found to be in pulseless electrical activity (PEA) requiring compressions multiple times. The patient expired after asystole within the week after transplant. An autopsy was done which revealed extensive hemosiderin deposition throughout the cardiac myocytes, pancreas, kidneys, adrenal glands, and small bowel consistent with hemochromatosis. Interestingly enough, the pretransplant and post-transplant liver did not contain the amount of hemosiderin found in hemachromatosis. Discussion: Hemachromatosis is a multifactorial genetic syndrome characterized by excess circulating iron which leads to parenchymal iron deposition in numerous organs throughout the body. The underlying mechanism is due to defective synthesis or decreased activity of hepcidin, a peptide hormone produced by hepatocytes in response to elevated serum iron. Genetic mutations, direct destruction of hepatocytes, and hepcidin resistance can lead to iron overload. The most common mutation is a homozygous defect of HFE (which was not found on PCR in the patient post-mortem). There are other rare causes of hemochromatosis which were not investigated and may be the cause of this patientвЂ™s presentation of primarily cardiac hemochromatosis, which has been documented. Interestingly enough, of patients receiving liver transplants, those with hemochromatosis are disproportionately more likely to develop cardiac complications particularly dilated cardiomyopathy with conduction abnormalities. Lymphandenopathy and Fevers that Progressed to a Skull Lesion as a Presentation of Langerhans Cell Histiocytosis Duc Phan, MD; Jeffrey Chen, MD; Katherine Lin, MD; Kathyn Lee Serwoka, BE; Eric Finlayson, MD; Jeffrey Canceko, MD University of Southern California Case: Mr. KM is a 30 year-old Caucasian male with history of diffuse lymphadenopathy for 5 months, presented to the emergency room for severe left-sided headache, photophobia, and hyperacusis for two months. Five months prior to admission, he presented to an outside hospital with pain and swelling of left axilla mass. He was initially treated with bactrim for two weeks without much improvement. The patient has had multiple axillary biopsies at an outside hospital, however all the biopsies were non-diagnostic. He was then referred to USC for further evaluation. Infectious etiologies were ruled out. Excisional biopsy performed 2 months prior showed mantle cell hyperplasia; however immunohistochemistry was inconclusive. He started having severe left-sided frontal headache with nausea, photophobia and left ear hyperacusis and came to the ED. He had no other neurological deficits. CT and MRI scans of the head showed a destructive lesion involving the left frontal calvarium with indistinct borders extending into the scalp with scalp swelling progressing into the extra-axial space. The lesion measured 16 mm x 27 mm x 17 mm. A left fontal craniotomy with an excisional biopsy was preformed. Pathology of the left frontal bone mass showed Langerhans cell hyperplasia. PatientвЂ™s symptoms resolved after the surgery and he was discharged home to be followed by Hematology, Neurosurgery, and his primary care physician for further treatment. The plan is to assess for other lesions and if amenable surgical resection and to followed with chemotherapy. 53 Discussion: Langerhans Cell Histiocytosis (LCH), formerly known as Histiocytosis X, is a rare disease. It is characterized by abnormal proliferation of cells found in epidermal histiocytes (Langerhan Cells). LCH can present as lesions in the bone (most common), skin, liver spleen, bone marrow, lymph nodes, hypothalamicpituitary region and lung. LCH is more commonly seen in children ages 0-15. However, reports in young adults have been documented. Diagnosis is made by cellular morphology and the presence of CD1a and CD207. Birbeck granules are classically found in LCH lesions by electron microscopy. Because LCH is more commonly seen in children, diagnosis is commonly delayed in young adults. LCH in our patientвЂ™s age group is rare. Note: bony erosive skull lesions are a common presentation. He has multisystem LCH given his lymph node involvement. The rarity and indolent manner of LCH in adults makes its incidence difficult to assess and diagnosis. This case stresses the importance for a proper tissue biopsy and the need for multiple biopsies, in addition to close monitoring for progression of disease. Since LCH can be a systemic disease affecting so many areas of the body a multidiscipline approach should be instituted. The internist should pursue consults from various specialists such as hematologists, oncologists, radiologists, surgeons, pulmonologists, and/or dermatologists for evaluation depending on the patientвЂ™s unique presentation and for assistance on diagnosis and treatment. The treatment for this rare condition is not well established. Treatment for multisystem LCH would include excision of the bony lesions, oral steroids and chemotherapy, namely, single agent vincristine. Cladribine appears to have some activity, as do vinblastine with steroids and anti-inflammatory agents. There are open protocols at some institutions. This being the case surveillance is important, for if there is no active disease simple observation is an option. Otherwise treatment plans consist of some combination of surgical intervention and chemotherapy. TB or not TB: A Patient with Abdominal Imaging Suggestive of Peritoneal Carcinomatosis, Proven on Biopsy To Be Mycobacterium tuberculosis Duc Phan, MD; Lusine Soghbatyan, MD; Nazanin Azadi, MD; Jeffrey Canceko, MD University of Southern California In 2006 the CDC had 139 documented cases of abdominal tuberculosis (ATB). ATB signs and symptoms are wide and varied. For tuberculosis peritonitis there are three forms: the вЂњwetвЂќ (ascites), the вЂњdry-plasticвЂќ (peritoneal involvement without ascites) and the вЂњfibroid-fixedвЂќ type (omental thickening and extensive adhesion formation). The outcomes can be severe even though it is readily treatable, mainly due to a delay in diagnosis. Mr. JZ is a 67-year-old male with a history of benign prostrate hypertrophy and depression complaining of abdominal pain. He has had mild symptoms for the past two years, but worsening in the last three months. The pain was diffuse, constant, and cramping; and worse after eating. He had symptoms of feeling feverish with chills, but only one episode of nausea and vomiting. He denied any hematemesis. He also denied any respiratory problems in the past or on this admission. He noted a weight loss of about 30 pounds in the last two months. CT of the abdomen and pelvis showed diffuse peritoneal carcinomatosis, retroperitoneal and mesenteric nodules, and cardiophrenic adenopathy. He was admitted to Medicine for workup and treatment. He continued to spike fevers despite being on broad-spectrum antibiotics. His pan-cultures were negative. Infectious Diseases, Gastroenterology, and Surgery services were consulted. The patient had an esophagogastroduodenoscopy (EGD), colonoscopy, and an EGD with push enteroscopy which all showed no evidence of disease. The patient's CA19-9, CEA, ACE, and LDH levels were normal. A PPD was placed and proved positive. A laparoscopic biopsy of his omentum and mesentery was performed. Preliminary evaluation of the biopsies showed evidence of TB. The patient was started on rifampin, INH, pyrazinamide, and ethambutol (RIPE) therapy. Final results showed the omental and mesentery biopsies had necrotizing granulomatous inflammation and were positive for AFB on special stains. Cultures from the biopsies grew M. tuberculosis complex, with a high probability for M. tuberculosis. On RIPE his symptoms improved; he was discharged to be followed by his primary physician and Public Health. Peritoneal TB should be considered in the differential diagnosis when presented with symptoms of abdominal pain, fever, and weight loss. Especially in a patient with peritoneal carcinomatosis as Mr. JZ. An awareness of this diagnosis can expedite the necessary procedures for diagnosis and early treatment. Procedures such as endoscopy or paracentesis for fluid assessment are valuable tools. In addition PCR testing may be beneficial with a suggestive history, but negative special stains for acid-fast bacilli on tissue sections. Laparoscopy, when indicated, should not be delayed for it can provide visual inspection to aid early assessment and intraoperative frozen sections to provide definitive diagnosis. Early Myocardial Infarction Reveals a Triplet of Prothrombotic States Michelle Phillips, MD; Robert Russo, Jeffrey Andrey Scripps Green Hospital A 45 year old woman presented with persistent left-sided chest and arm pain, increased cardiac markers, and inferior T wave inversions. Cardiac catheterization showed 80-90% occlusion of LAD and 40-50% occlusion of the first diagonal coronary artery. Her early onset coronary heart disease (CHD) prompted consideration of a prothrombotic or hypercoagulable disorder. Her PMH included hypothyroidism, HTN, and 4 normal pregnancies. There was no history of miscarriage, venous or arte- 54 rial clots prior to her presentation with acute myocardial infarction (AMI). Family history was likewise (-) for early CHD or evidence of hypercoagulable states. PE showed a BMI of 28 and was otherwise (-). Basic lab studies and fasting lipid panel were within normal limits. Protein S function, Protein C activity, and Factor V activity were normal. Prothrombin gene mutation was (-). The patient had the following unusual combination of defects: R506Q Factor V Leiden mutation (linked with activated protein C resistance), (+) for antiphosphatidylserine IgM and beta-2 glycoprotein I IgG antibodies (indicating antiphospholipid syndrome), and the methylenetetrahydrofolate reductase (MTHFR) 677; T homozygous вЂњTTвЂќ genotype. Activated protein C resistance confers a 3-to-8fold increased risk for venous thrombosis; conflicting evidence exists regarding an association with arterial thrombosis and acute MI, but a modest association has been reported in young females. Antiphospholipid syndrome is associated with recurrent venous and arterial thrombosis & recurrent fetal loss. MTHFR is involved in the conversion of homocysteine to methionine in a folatedependent process. The TT genotype predisposes to increased serum homocysteine, which correlate with risk of CHD, especially in the presence of low folate levels. Hyperhomocysteinemia has been the subject of much interest and research in the past decade. The in-vivo effects of hyperhomocysteinemia on the coagulation cascade, endothelial function, atherosclerotic lesion formation, and platelet aggregation are well-established; however, studies of the administration of folate, B6 and B12 to reduce homocysteine levels demonstrates no efficacy in secondary prevention of CHD. Careful review of the most recent evidence demonstrates a likely significance in early CHD in young female patients with the MTHFR 677 TT genotype especially in the setting of folate deficiency, but the value of screening or treatment for this state in the general population or for secondary prevention remains in question. Lifelong full-dose aspirin and anticoagulation with warfarin were recommended for this patient with multiple hypercoagulable disorders. When the Solution is the Problem: Polypharmacy of Supplements and a Case of Myopathy Michelle Phillips, MD; Nitasha Bakhru, MD; Enoch Wang, MD; Laura Nicholson, MD Scripps Green Hospital Patients commonly present with a list of nutritional supplements, over the counter medications, and traditional remedies used in addition to their prescribed medications. Clinicians must persist in obtaining this history, and selfeducate regarding the effects of unfamiliar agents on the health of our patients. This is a case of an elderly woman presenting with a constellation of complaints and a myopathy attributable to one home remedy among a lengthy list of supplements. A 74 year old woman with history of dyslipidemia, migraine, and hypothyroidism presented with progressive weakness over three months. The patient had previously been able to perform aerobics for an hour at a time, and now could not walk further than the length of a room before feeling weak and stopping to rest. The patient also reported sores on her tongue, constipation, pustules on her face, right lower extremity tingling sensations, excessive salivation, weight gain, and hair loss. She admitted to using multiple вЂњmedicationsвЂќ she ordered over the Internet, and had stopped using prescribed medications for dyslipidemia and hypothyroidism. The patientвЂ™s daughter produced a list of supplements including L-carnitine, selenium, zinc, lutein, iron, folic acid, vinpocetine, milk thistle, choline, inositol, calcium, potassium, taurine, biotin, magnesium, carnosine, 5hydroxytryptophan, L-lysine, methylsulfonylmethane, gamma-aminobutyric acid, vitamin B6, alpha-lipoic acid, malic acid, beta carotene, methylcobalamin, fish oil, flax seed oil, castor oil, molybdenum, LugolвЂ™s solution, riboflavin, idebenone, valerian root, chlorella, and CardioPeptase. Physical exam revealed a well-nourished woman with normal vital signs. There were scaling, excoriated pustules on the scalp and face as well as shallow ulcerations on the tongue. Neurologic exam revealed no focal deficits, but motor retardation and easy fatigability with ambulation. Laboratory studies demonstrated creatine kinase (CK) of 5,110 u/L [21-215] and CK-MB of 95 ng/mL [0.6-3.6] with normal EKG, troponin, urinalysis, and erythrocyte sedimentation rate (ESR). Thyroid stimulating hormone was 77.7 mcIU/mL [0.358-3.8] and free T4 was 0.25 ng/dL [0.76-1.46]. The clinical picture was consistent with hypothyroid myopathy secondary to iodine toxicity from chronic ingestion of LugolвЂ™s solution. LugolвЂ™s solution is a 130 mg/ml blend of elemental iodine and potassium iodide used to treat thyroid storm, with historical use in severe hypothyroidism. Ingesting supratherapeutic doses of iodine in the setting of autoimmune thyroiditis caused prolonged, severe hypothyroidism via the Wolff-Chaikoff effect. Hypersalivation and acneiform skin eruptions are signs of iodine toxicity. Patients are treated with thyroid hormone supplementation and withdrawal of iodine-containing agents. This case highlights the importance of careful history-taking and awareness of the toxicities and possible adverse events associated with supplements and traditional remedies. LГ¶fgren Syndrome: A 41 Year-Old Hispanic Man with Acute Sarcoidosis Donald Phillips, MD; Frank Degregorio, MD; Thomas Zung, MD; Leland Powell, MD, PhD Olive View-UCLA Medical Center Most physicians in the United States know sarcoidosis as a chronic, often disseminated disease with long-term disability. However, an acute form of sarcoidosis, most prevalent in Northern Europe, often permanently remits with no lasting morbidity. Its relative scarcity in the US makes LГ¶fgren Syndrome a diagnostic challenge. 55 A previously healthy 41 year-old Hispanic man presented to a community clinic with fevers, chills, a painful, symmetric polyarthritis first appearing in the ankles, and erythema nodosum. He was found to have an elevated ESR and was prescribed a seven-day steroid taper, which temporarily improved his symptoms. A few weeks later he presented to the emergency department with similar symptoms, plus increasing fatigue, malaise, night sweats, and a thirty-pound unintentional weight loss. His ESR remained elevated, but autoimmune serologies were negative. He had a normal white blood count with a normal differential. A chest x-ray revealed symmetric hilar lymphadenopathy. He had no respiratory complaints, a normal ACE level, a non-reactive PPD, and negative coccidioidomycosis antibody titers. A CT scan revealed enlarged and confluent mediastinal, hilar, celiac, and porta hepatic lymph nodes, concerning for lymphoma. He received an extensive work-up, including bronchoalveolar lavage negative for signs of infection or neoplasic cells, as well as two non-diagnostic trans-bronchial biopsies. Finally, pathology obtained from mediastinoscopy revealed non-caseating granulomatous inflammation consistent with sarcoidosis. When rechecked, his ACE level was high. The patient has since recovered, his chest radiograph normalized, and is currently asymptomatic without further treatment. This case illustrates the classic presentation of LГ¶fgren Syndrome, typified by an acute onset, a symmetric polyarthritis involving the ankles, erythema nodosum, and hilar adenopathy. The diagnosis was made difficult, however, by geography. LГ¶fgren Syndrome patients in Northern Europe are often diagnosed clinically, with no biopsy needed to provide histologic evidence. The lesson of this case is that LГ¶fgren Syndrome occurs in the United States probably more often than currently recognized, and prompt recognition of this variant may spare patients from an invasive work-up and the psychological stress of possibly having cancer. Lastly, understanding the usual course of LГ¶fgren Syndrome may prevent unnecessary treatment given its favorable prognosis. A Case of Solitary Fibrous Tumor of the Pleura Shahriar Pirouz, MD; Alison Kole, MD; Nader Kamangar, MD, FCCP Olive View-UCLA Medical Center Case: A 61 year-old white male presented to the ER with complaints of lower extremity swelling and shortness of breath. His symptoms began 4 months prior to admission, when he noticed a gradual decrease in appetite, 24 lb weight loss, chronic cough, progressive weakness, and fatigue. He also noted a palpable mass in his right lower quadrant, with subsequent right lower extremity swelling, which gradually progressed bilaterally. Review of systems was notable for worsening constipation, though otherwise was negative. Patient had history of lumbar spine osteosarcoma with resection at the age of 19. On exam, the patient was afebrile; heart rate 92; blood pressure 143/82; respiratory rate of 18, with 98% oxygen saturation on room air. He was cachectic, speaking in full sentences. Notable physical exam findings include decreased breath sounds over the right lung with increased tactile fremitus and increased dullness to percussion; a firm right upper quadrant without hepatosplenomegaly; and bilateral pitting edema. Chest x-ray revealed subtotal opacification of the right hemithorax with contralateral mediastinal shift. CT Chest with contrast revealed a 20.6 cm x 20cm large heterogeneous noncalcified and noncavitary mass abutting the chest wall without invasion. The patient underwent a percutaneous CT guided needle biopsy of the mass. Pathology revealed a CD34 positive, S-100 negative spindle cell lesion, consistent with a solitary fibrous tumor. The patient was referred for possible resection. Discussion: Solitary fibrous tumors (SFTs) of the pleura are rare neoplasms of mesenchymal origin. The initial presentation typically is a large incidental asymptomatic mass, with concomitant ipsilateral pleural effusion. Respiratory symptoms occur in approximately a third of patients and include cough, dyspnea, pleuritic chest pain, and hemoptysis. Extrapulmonary symptoms include hypoglycemia and hypertrophic osteoarthropathy, thought to be related to increased production of hyaluronic acid by tumor cells. Additional manifestations include weight loss, galactorrhea, and arthritic pain. Histologically, SFTs are characterized by fibroblast-like cells and connective tissue in varying proportions, often arranged in a "patternless pattern". Histochemically, SFTs are CD34 positive, and are keratin and S-100 negative. Benign SFTs are curable with resection. Malignant SFTs may be curable provided complete resection is possible. Local recurrence is common (up to 63%), thus close follow-up of highrisks patients is recommended. There is little data on the efficacy of chemotherapy or radiation in high-risk patients. The Pulsating Sternal Mass Shahriar Pirouz, MD; Jonathan Neyer, MD; Henry Honda, MD Olive View-UCLA Medical Center, UCLA Medical Center Case: Patient is a 59 year old male with h/o multiple cardiac surgeries who presented with a large protruding mass at the superior aspect of his sternum. His cardiac history includes VSD repair and aortic valvuloplasty as a teenager. At age 53, he developed atrial fibrillation, severe mitral stenosis and underwent mitral valve replacement. He eventually developed congestive heart failure with aortic insufficiency and underwent a porcine aortic valve replacement and Bentall procedure in April 2009. The patient did well for a month after the Bentall procedure but soon developed decreasing exercise tolerance and night sweats, which were reminiscent of his symptoms at the time of his endocarditis episode. He was admitted and found to have coag-negative staph and bioprosthetic aortic valve endocarditis. While receiving anti- 56 biotics, he developed the enlarging pulsatile chest mass. The mass proved to be a hematoma which was contiguous with a large anterior mediastinal and pericardial soft tissue collection; it was the product of leakage from the proximal and distal Bentall anastomosis sites. He was treated with tight blood pressure control and serial aspiration of the mass. The patient has been referred for surgical repair of the leak. Discussion: Periprosthetic leakage (PPL) is a frequent cause of reoperation after aortic valve replacement (AVR), occurring in 1% to 3.5% of patients after AVR. This represents the second most frequent indication for repeated surgery in this patient population, second only to degeneration of biologic prosthetic valves. PPL occurs due to incomplete apposition of the sewing ring to the native tissue. Prior or active infection increases the risk of PPL. Factors predisposing to PPL have not been fully identified, although prosthetic endocarditis, Marfan syndrome, bicuspid aorta, and severely calcified aortic annulus may be predisposing factors. Detection has increased as a result of improved techniques, particularly transesophageal echocardiography. Most PPLs are asymptomatic and benign. Clinically significant PPLвЂ™s may be due to regurgitation, with reduced effective cardiac output, or congestive failure, resulting in dyspnea on excursion. Large PPLвЂ™s are generally associated with a murmur, though occasionally an eccentric jet may be relatively inaudible. PPLвЂ™s may lead to hemodynamic, hematologic, or other clinical problems. More than mild periprosthetic regurgitation, presence of hemolysis, and decreased ejection fraction are standard indications for surgical reintervention. Operative risk is higher than firsttime surgery with recent published reports indicating a hospital mortality ranging from 5.4% to 16%. Disseminated Cryptococcosis in a Renal Transplant Patient Donald J Portocarrero, DO; Juan C Barrio, MD; Byron K Williams, MD White Memorial Medical Center Case: A 75 year-old Hispanic male with a PMH of HTN, DM II, ESRD s/p cadaver transplant presented with generalized weakness for 2-3 weeks. Patient had been on Rapamune for 4 years, but due to recent papilloma virus infection, it was switched to Leflunomide 6 months prior to admission. PatientвЂ™s baseline creatinine was 1.5. The patient admitted to decreased urine output. Review of systems was essentially negative, including headache, fever, chills, neck stiffness, chest pain, cough, shortness of breath, purulent sputum. The patient was afebrile with stable vital signs. He was awake and alert but disoriented. There was no neck stiffness or photophobia. Cardiovascular, respiratory, and gastroenterological evaluations were negative. KernigвЂ™s and Brudzinski's signs were negative. The patient had a left arm AV shunt with an eschar distal to it. The neurological exam was nonfocal. Initial labs revealed a mild leukocytosis of 11.9 with neutrophils of 89%, lymphocytes 6%, no bands. Chemistry panel: CO2 level of 18, BUN of 129, and Cre of 2.6. CT of the head was negative. The patient was admitted with initial diagnoses of acute renal failure and rule out sepsis. IV fluids and empiric antibiotics, featuring Vancomycin and Zosyn, were started. The immunosuppressants were discontinued. Blood Cultures were positive for Cryptococcus Neoformans on two sets and Amphotericin B was started. Given the patientвЂ™s renal insufficiency, Amphotericin B was switched to its lipid formulation Abelcet on Day 2. Flucytosine was held due to renal failure. The initial cryptococcal serum antigen titer was 1:4096. Biopsies of the eschar distal to the AV shunt revealed Cryptococcus neoformans infection with extensive necrosis and severe acute inflammatory reaction. Due to deteriorating mental status, a brain MRI was performed. This was essentially negative. Lumbar puncture revealed yeast in the spinal fluid consistent with Cryptococcus Neoformans meningitis. The HIV screen was negative. The patient had a hospital course complicated by NSTEMI, shock liver, septic shock, and DIC. Supportive and aggressive medical management saved the patientвЂ™s life. However, his renal function never recovered, necessitating resumption of hemodyalisis. The patient was subsequently transferred to a skilled nursing facility. After 40 days of systemic Abelcet, the serum cryptococcal Ag titer was repeated and remained elevated at 1:4096. Discussion: Mortality rates of Disseminated Cryptococcus are 70-80% in untreated patients compared to those treated with antifungal medications. The Crypto A/D Study concluded that initial dissemination, high serum Ag titer (>1:512), and lack of flucytosine during induction therapy were associated with treatment failure at two weeks independent of HIV status. This case not only validates those results, but also further extends the time limit of mycologic failure to greater than 5 weeks. Given the patientвЂ™s poor renal condition, two important management decisions were made (1) Flucytosine was not used for induction treatment; and (2) Abelcet was used instead of regular Amphotericin B for treatment. Nevertheless, our patientвЂ™s clinical status remained poor and the serum cryptococcal Ag titer was still elevated despite medical treatment. One should consider flucytosine induction with or without lipid formulations of Amphotericin B regardless of renal function. Transient Apical/Mid Left Ventricular Ballooning in a Woman with Severe Asthma Exacerbation Vinoy Prasad, MD; Kenneth Narahara, MD Harbor-UCLA Medical Center A 73-year-old Hispanic woman with a history of hyperthyroidism treated with propylthiouracil and moderate persistent asthma, but no cardiac history, presented with shortness of breath and diffuse wheezing concerning for asthmatic exacerbation. She had frequent hospitalizations and ED visits in the past for asthmatic exacerbations. She had a non-productive cough and shortness of breath for 2 days requiring increasing usage of her rescue inhaler, but denied any fever or chest pain and her initial cardiac 57 biomarkers were negative. Symptoms improved with nebulizers, and she was admitted for steroid treatment and observation. Overnight, she acutely decompensated and was noted to be in severe respiratory distress, appearing cyanotic with increased accessory muscle use and pursed lip breathing, with an arterial blood gas supporting the diagnosis of hypercarbic respiratory failure. The patient subsequently had substernal, squeezing chest pain. Cardiac biomarkers drawn showed an elevated troponin-I of 4.54 with a creatine kinase MB fraction of 21.2; thyroid hormone levels were normal. ECG showed Q-waves and ST-elevations in leads V2-V6 concerning for acute anterolateral infarction. She was emergently taken for cardiac catheterization, where she was noted to have angiographically normal epicardial coronary arteries. A 2-D echocardiogram performed thereafter demonstrated a depressed LV EF of 30-35% with an akinetic apical wall and hypokinetic mid-septal, anterior-inferior, and lateral walls. All basal segments contracted normally. The patient was treated supportively, and her cardiac biomarkers trended down after a troponin-I peak of 9.45. A follow-up 2-D echocardiogram 11 weeks later showed normal LV size and function (EF 65-70%) and no regional wall motion abnormalities. Transient apical/mid-ventricular ballooning, also known as вЂњTakotsubo cardiomyopathy,вЂќ is a syndrome which mimics acute myocardial infarction, but without angiographic evidence of coronary artery stenosis. For unclear reasons, post-menopausal women, such as our patient, are predominantly affected. Wall motion abnormalities typically involve the apical one-half to two-thirds of the left ventricle, with sparing or hypercontractility of basal segments. Echocardiographic and ECG findings are characteristically out of proportion to the degree of elevation of cardiac biomarkers. This transient cardiomyopathy is thought to be triggered by intense emotional or physical stress, possibly through the release of supraphysiologic levels of plasma catecholamines and stressrelated neuropeptides resulting in direct myocyte injury or coronary arterial or microvascular spasm. Supportive therapy and removal of the stress resolves the cardiac dysfunction, as it did with our patient. This case study illustrates an increasingly reported but peculiar transient cardiomyopathy, induced by the stress of a severe asthmatic exacerbation requiring intubation. Acute Changes in Renal Function in Severe Pulmonary Hypertension Reza Rafie, MD; Ali Farvid, MD; Ashkan Attaran, MD; Nima Taha, MD; Sharmineh Shams, MD; Mehdi Manzari, MD; Mohammad Pashmforoush, MD University of Southern California Introduction: Pulmonary hypertension affects right ventricular function that may cause venous congestion. Previous studies showed the effect of venous congestion on glomerular filtration in patients with heart failure. In this case report we present a patient with severe pulmonary hypertension that had elevated creatinin on admission. Her creatinin decreased to baseline with sildenafil and lasix that was resumed to treat her elevated pulmonary pressure Case: 32 year-old woman with history of idiopathic pulmonary hypertension since 2 years ago. She was admitted with shortness of breath after running out of her medications. She was diagnosed initially with elevated pulmonary pressure by transthoracic echocardiography that was confirmed later by catheterization. She had ventilationperfusion scan that showed normal results. She was started on sildenafil, lasix and home oxygen. She ran out of her medication 2 months prior to admission. She had progressive shortness of breath associated with swelling in her lower extremities. Her vital sign on admission showed blood pressure 98/60 mmHg, Heart rate 93 bpm, respiratory rate 26 per minute with oxygen saturation 98% on 6 liter oxygen. Her cardiac exam showed 3/6 systolic murmur in left sternal border, 3/6 diastolic murmur in pulmonic site and loud P2. She was started on lasix and sildenafil. Her creatinin on admission was 2.3 mg/dl that increased from her baseline values 0.7 mg/dl. She denied previous history of kidney disease. She had work up for her acute kidney dysfunction that all results came back normal. Meanwhile she had transthoracic echocardiography that showed severely reduced right ventricular systolic pressure with estimated pressure at 110-120 mmHg. Estimated pulmonary artery pressure was at least 40 mmHg and right atrial pressure was 15-20 mmHg. There was severe pulmonary and tricuspid regurgitation. She was continued on sildenafil and low dose of lasix. Her symptoms improved after 2 days. Her creatinin gradually declined and after 3 days returned to her baseline level. She was a candidate for combination therapy with bosentan and sildenafil due to her severe pulmonary hypertension. Discussion: Previous studies showed patients with advanced heart failure have increased incidence of concurrent renal function impairment but low cardiac output alone could not explain the deterioration in renal function. Recent study that published on May 2009 showed that venous congestion is the most important factor in worsening renal function in heart failure patients. In this case report, for the first time we present a patient with severe pulmonary hypertension with normal left ventricular ejection fraction and severely impaired right ventricular function that her acute renal dysfunction improved after treatment with sildenafil and lasix. Future studies can show the beneficial effect of decreasing venous congestion on renal function in severe pulmonary hypertension. Electric Dissociation within Left Atrial Appendage Reza Rafie, MD, David Cesario MD,PhD, Tasneem Z Naqvi, MD University of Southern California 58 Background: Electrical cardioversion is one of the modalities that has been used to treat atrial fibrillation. Before proceeding to cardioversion , transesophageal echocardiography (TEE) is performed to rule out thrombus within the left atrial appendage. We describe a patient who was admitted for cardioversion with underlying refractory atrial fibrillation, who was found during TEE to have an accessory lobe within the left atrial appendage that was electrically dissociated from the remaining of the body of left atrial appendage and left and right atria. Case: An 79-year-old man with history of severe mitral regurgitation, atrial fibrillation was admitted with refractory atrial fibrillation for cardioversion. He had history of mitral valve and tricuspid valve repair and Maze procedure on 2008. 3 months later he developed transient complete heart block that was recognized incidentally during his admission for a right groin abscess. He underwent dual chamber pacemaker placement. He was admitted on May 2009 to have ablation for persistent atrial arrhythmias. He underwent TEE that showed an elongated accessory lobe within left atrial appendage. During evaluation by Doppler imaging the accessory lobe was found to be in sinus rhythm that was electrically dissociated from the body of left atrial appendage, left atria and right atria with atrial fibrillation rhythm. He subsequently underwent electrophysiology study to have ablation. Activation map and bioscience intracardiac 3 dimensional mapping confirmed electrical dissociation data obtained by echocardiography. Discussion: The accessory lobule of left atrial appendage has been recognized if itвЂ™s length exceeds than that of the entrance lumen. Previous studies have reported coexistence of sinus rhythm and atrial fibrillation after Maze procedure however the lines of incision used during Maze procedure in our patient, do not anatomically explain the electric dissociation within the left atrial appendage. Several case reports previously described the electrical dissociation between the atrial appendage and rest of the body of ipsilateral arium but to our best knowledge there is only one description on electric dissociation within the left atrial appendage previously reported by our group. In that case report, Doppler echocardiography showed sinus rhythm within the main body of left atrial appendage whereas left atrial appendage accessory lobe had rapid atrial waveforms of atrial fibrillation. Our current patient had normal sinus rhythm in left atrial appendage accessory lobe on Doppler echocardiography while the main body of left atrial appendage as well as the left and right atria were in atrial fibrillation. Our TEE findings were confirmed during electrophysiologic study. These results emphasize the need to carefully evaluate the left atrium and its appendage by Doppler echocardiography. Atrial dissociation phenomenon may also explain occurrence of appendage thrombi with or without stroke in some patients in whom surface ECG may show sinus rhythm. Fentanyl Induced Atrioventricular Nodal Block Reza Rafie, MD; Ashkan Attaran, MD; Sharmineh Shams, MD; Ali Farvid, MD; Nima Taha, MD; Mehdi Manzari, MD; Mohammad Pashmforoush, MD University of Southern California Background: Several studies in dogs showed the effect of fentanyl on prolongation of sinoatrial and atrioventricular conduction time. There are several case reports about the atrioventricular nodal block by fentanylpropofol combination that are used for anesthesia. In this case report, we describe a patient with unknown cause of AV nodal block that fully recovered after stopping fentanyl. Case: 51 year-old man with history of intravenous drug abuse, bilateral upper extremity cellulites, infective endocarditis and was admitted with falling episode on the day of admission. In emergency room, he had CT scan of the neck that showed no fracture but there was possible phlegma and discitis between C2-C7 so his C-collar was kept in place. He subsequently developed respiratory distress and was intubated for airway protection with possible diagnosis of acute respiratory distress. He was transferred to medical intensive care unit. He had bilateral upper extremity cellulites and 1/6 systolic murmur at cardiac apex. His initial EKG showed sinus tachycardia. Result of his blood culture was positive for oxacillin resistant staphylococcus aureus. He was started on empiric antiobiotic treatment with vancomycin. He underwent transthoracic echocardiography that showed thickened mitral leaflet with possible healed endocarditis. A vegetation on mitral valve could not be excluded. It was recommended to have transesophageal echocardiography(TEE) to rule out vegetation on mitral valve but TEE could not be performed at that point due to cervical spine phlegma and the high risk for complication with removing C-collar. On day three of admission, he suddenly developed 2 episodes of atriventricular nodal block that each last 5 seconds. External pacemaker was placed. He was intubated and was treated with fentanyl at that time for sedation. Fentanyl was considered as the main culprit, although there was concern about suspected endocarditis and its complications. He was considered high risk for possible cardiac operation. His fentanyl was discontinued and his heart rhythm returned to normal. He never had repeated episode of atrioventricular nodal block. His condition improved and he was extubated successfully. His TEE after temporary removing his C-collar showed no vegetation on mitral, aortic and tricuspid valves. Conclusion: Fentanyl can cause prolongation of cycle length and antegrade effective refractory periods of the A-V node and ventricles in dogs. These effects can be due to vagus nerve stimulation or the direct effect on sinoatrial and atrioventricular (AV) nodes. Although several previous case reports showed the AV nodal blocking effect by combination of fentanyl with other medications such as propofol, in the patient that we described here it appears that the sole effect of fentanyl caused reversible AV block. With extensive use of fentanyl in intensive care setting, its cardiac effect should be evaluated more carefully. 59 Massive Right Sided Cardiac Thrombus in Patient with Dilated Cardiomyopathy and Hyperhomocysteinemia Reza Rafie, MD; Ashkan Attaran, MD; Sharmineh Shams, MD; Ali Farvid, MD; Nima Taha, MD; Mehdi Manzari, MD; Mohammad Pashmforoush, MD University of Southern California Introduction: Homocysteine has been recognized as a risk factor for arterial and venous thrombotic events. Previous studies showed the association of hyperhomocysteinemia with intracardiac thrombus especially left atrial thrombus in stroke patients. In this case report we describe a patient with elevated homocystein level and ischemic cardiomyopathy that was admitted with massive thrombus in right side of the heart expanding from inferior vena cava entrance to right ventricle. Case: 68 year-old woman with history of myocardial infarction, was admitted with acute onset shortness of breath and exacerbation of peripheral edema. Before admission to emergency room, she developed pressure like chest pain, lasting 30 minutes that was relieved by nitroglycerin given in emergency room. Her vital signs were stable on admission. On physical exam she had crackles in lower part of both lungs. Her cardiac exam showed regular rhythm with 2/6 systolic murmur in xephoid area. Her ECG showed sinus rhythm with Q waves in leads II, III, AVF and V4-V6 but without ST-T changes. She had normal troponin levels. She underwent transthorasic echocardiography that showed dilated cardiomyopathy with severely reduced left ventricular function and elevated right ventricular pressure. There was a mass in right atrium that appeared to be attached at the level of Eustachian valve, between the right atrium and inferior vena cava. This mass prolapsed into the right atrium, right ventricle, and inferior vena cava. The largest diameter of mass was 5.8 cm. She also had CTPA that showed thrombus in right and left pulmonary arteries, she was started on heparin. She also had cardiac catheterization that showed severe 3 vessel disease. Results of her anticoagulation work up only revealed elevated homocysteine level ( 20.6 Вµmol/L with normal level < 11.5 Вµmol/L). She was considered a poor surgical candidate due to her physical condition at that point. She was treated with lovenox with close follow up in cardiothoracic surgery to have CABG after improve her general condition. Discussion: Homocysteine level has an established effect on coronary thrombotic events but there is limited data about the effect of hyperhomocysteinemia on intracardiac thrombi formation. Several studies also described large intracardiac thrombi in patients with dilated cardiomyopathy. The patient that we described in this case report had huge intracardiac thrombi in right atrium and right ventricle. Her underlying dilated cardiomyopathy predisposed her to have thrombus formation in her heart, but the additive role of elevated homocysteine level in thrombi formation in right side of heart needs more evaluation. In patients with dilated cardiomyopathy, the intracardiac thrombus formation in the context of hyperhomocysteinemia can be a target for treatment in the future. Possible Faces of Patent Foramen Ovale (PFO) Reza Rafie, MD; Samuel Daneshvar, MD; Tasneem Naqvi, MD University of Southern California Introduction: In approximately 27% of the population, the communication that provides oxygenated placental blood flow to the fetus remains patent as a patent foramen ovale (PFO). In this report we describe unusual manifestations of PFO. Case 1: A 59 year-old man with history of diabetes presented with progressive weakness in his upper and lower extremities that started 1 week prior to admission. He was bed bound since admission. MRI of the spine showed a large expansile intramedullary lesion from C3 to C7. He underwent midline myelotomy at the level of C3-C4 and C6-C7 levels and also found to have an epidural abscess with pus. Culture was positive for streptococcus viridians. He had a transesophageal echocardiography(TEE) that showed a 4 mm PFO. He completed the antibiotic treatment after the surgery and was transferred to rehabilitation center to consider PFO closure after improvement in his medical condition. Case 2: A 35-year-old man with h/o severe scoliosis since birth and frequent migraine HA complained of severe HA and slurred speech 1 month prior to admission. MRI of brain at the time was normal and MRI of the spine showed rotoscoliosis. He underwent transcranial Doppler that was (+) for bubbles. He was referred for further evaluation. Before admission he developed another episode of severe HA with transient R-sided weakness. TEE showed moderate right to left shunting, which was exacerbated by body habitus with extrinsic compression of right atrium by the liver. Resting hypoxemia of 87% on room air was noted during TEE. Pulmonary artery pressure was normal. The patient underwent percutaneous closure of the PFO. At 2 months follow-up after PFO closure, he denied further TIA and also mentioned improved HA. Discussion: Our first patient describes a patient with cryptogenic spinal abscess in association with PFO. Multiple case reports describe the association between cryptogenic cerebral abscess with PFO but to the best of our knowledge there is only one case report within a case series that describes spinal abscess in a patient with underlying PFO. Howevert in that case there was no evidence of right-to-left shunting despite Valsalva maneuver and cough. In patients with possible cardiac source of septic emboli, PFO should be evaluated as a potential source of cardioembolic disease. 60 Second patient presented with a history of multiple abnormalities of the spine, migraine headaches, and TIA. In this patient, abnormalities of the spine caused right atrial compression by the right hemidiaphragm. This in turn resulted in opening up of a normally closed PFO channel resulting right-to-left shunting across PFO. Cardiac and spinal anomalies are both part of the VACTERL syndrome. Our report describes occurrence of hypoxemia at rest and of right to left shunting across PFO resulting from extrinsic right atrial compression from an elevated diaphgram. More studies are required to evaluate the PFO incidence in patients with other congenital anomalies including congenital anomalies of the spine. HaNDL with Care Santhi Raja, MD, FJFICM; Jasminka Criley, MD, FACP, FHM; Ali Khoiny, MD; Chester Choi, MD, MACP St. Mary Medical Center Introduction: Pseudomigraine or HaNDL (Headache with transient Neurologic Deficits and Lymphocytic pleocytosis) is a self limited syndrome of unclear etiology. The clinical picture includes episodes of severe bilateral headaches, often without meningismus, accompanied by transient neurological deficits. CSF shows a lymphocytic pleocytosis with negative etiological results and neuroradiological studies are normal. Case History: This 26-year-old Hispanic male, previously in good health, presented with recurrent episodes of headaches associated with transient severely altered mentation. His neurological exam was otherwise normal. His hemogram, serum chemistry, toxicology screen and chest x-ray were normal. He had a total of 6 episodes in a span of 8 weeks and 2 of the episodes were associated with severe agitation. All episodes were associated with CSF lymphocytosis (WBCвЂ™s of 166 to318), elevated protein but normal glucose and negative etiological results. Repeated neuroimaging was also normal. He was asymptomatic between the episodes. Each episode lasted for 2-6 days. Encephalitic antibody panel, CSF VDRL,cryptococcal antigen,cocci antibody and India ink preparation were all-negative. CSF culture was negative for fungi, bacteria including TB. CSF PCRs for HSV, CMV, HIV, and AFB were also negative. Antibodies for toxoplasmosis, leptospirosis, and borreliosis were all negative in CSF. Autoimmune work up was negative. The patient was treated empirically with antibiotics and acyclovir in the initial 2 admissions and improved rapidly each time with discharge to home within 4-6 days of admission. Discussion: Our patient appears to have had Pseudomigraine or HaNDL, which is defined by the International Headache Society as a self-limited syndrome of unknown origin characterized by recurrent severe bilateral headache accompanied by transient neurologic symptoms and CSF lymphocytosis. These patients are usually between 15 and 40 years of age. It is more frequent in men. Lym- phocytic pleocytosis (10 to 760 cells mm3) and increased CSF protein are found with negative microbiological and immunologic studies. CT scan and MRI are normal, but an electroencephalogram frequently shows focal slowing. It is possible that pseudomigraine could result from an activation of the immune system secondary to a recent viral infection, which would produce antibodies against neuronal or vascular antigens. This syndrome is not well known as a cause of recurrent aseptic meningitis and can be confused with MollaretвЂ™s syndrome. The later usually recurs over a period of years while Pseudomigraine is self-limited over a period of few weeks to months and no residual sequlae, although the neurological deficits such as sensory symptoms (78%), aphasia (66%) and motor deficits (56%) may be quite marked. Acute confusional state and agitation are less common, but have been previously described. Recognizing this syndrome may help to avoid repetitive investigations, offer a better prognosis, and eventually lead to an improved understanding of the etiology and pathophysiology involved. From 'Black & White' to Red All Over - How a Straightforward Case of Streptococcal Pharyngitis Evolved Into Guttate Psoriasis Sangeetha Reddy, BA; Jason Napolitano, MD University of California, Los Angeles Case: A 32 year old female presented to the emergency department with a painful generalized erythematous rash, arthralgias, and low grade fever of 100 F. Eight weeks prior, patient was treated for streptococcal pharyngitis with 10 day course of amoxicillin, of which she completed 3 days. Two days after stopping antibiotics, she developed an erythematous rash in her intertriginous areas. Patient began treatment with topical antifungals with no improvement. Over the next several weeks, the rash worsened with development of bilateral arthralgias of her knees and ankles. Four days prior to presentation, the rash spread diffusely to her chest, face, and extremities. Physical exam was notable for multiple well defined erythematous papules, with central scaling, on her entire body, with palmoplantar sparing. The papules coalesced into large plaques on the face and chest. Laboratory investigations were significant for white count of 10.3, ESR of 53, and ASO titer of 541. Of note, HIV antibodies and pharyngeal swab were negative. A punch biopsy of the skin was consistent with psoriasis. Treatment with topical steroids and systemic therapy with infliximab was initiated, and the rash improved. Discussion: Streptococcal pharyngitis has been known to cause several post-infectious skin syndromes. This case illustrates that in addition to the more commonly considered scarlet fever, antibiotic drug reactions, rheumatic fever associated erythema marginatum, and TSS, it is important to keep psoriasis on our differential. Approximately two thirds of first presentations of guttate psoriasis are preceded by streptococcal pharyngitis. One pro- 61 posed mechanism suggests that streptococcus superantigens induce the expression of skin homing receptors in T cells, which then migrate to the skin and cause the pathological characteristics of psoriasis. Guttate psoriasis can resolve on its own, beginning several weeks to months after the rash begins. Otherwise, treatment is similar to other types of psoriasis, which can include topical steroids, UV therapy, and systemic anti-inflammatory medications. Antibiotic treatment for the rash is not recommended based on randomized controlled trials. Several case reports have shown improvement of skin lesions after tonsillectomy, but this has not yet been studied in trials. In addition to streptococcus, other infectious triggers of psoriasis include HIV, candida, malassezia, and staph aureus. The Disease of KingsвЂ¦With a Twist Eugene Richardson, MD Stanford University Medical Center Case: A 62 year-old obese Tongan male with a history of type II diabetes, hypertension, and gout presented to the emergency department with two days of severe left knee swelling and pain. He was admitted to the short stay unit with the diagnosis of gouty arthritis and was given fluids, prednisone, and hydromorphone. The patient continued to require high doses of hydromorphone, and medicine was consulted. A joint aspiration was performed which demonstrated a neutrophil count of 52,000, negatively birefringent crystals, and gram-positive cocci growing in pairs and chains. Blood cultures drawn previously in the ED demonstrated similar gram-positive organisms which were later speciated as group B streptococcus (Streptococcus agalactiae). The patient was started on 5 million units penicillin IV q4hours (he weighed 325 pounds) and was taken for surgical irrigation and debridement. His pain improved considerably after the operation requiring only infrequent Percocet. The patient was discharged to a skilled nursing facility for four weeks of IV antibiotics via PICC as well as physical therapy. Follow-up blood cultures performed weekly were negative for growth. Discussion: Gout was known throughout history as the вЂњdisease of kingsвЂќ due to its association with overindulgence in food and wine. Our patient was a pastor and shunned alcohol, but did report a hearty dinner at the local Chinese restaurant the night before onset of symptoms. What complicated this case was the concomitant joint infection and bacteremia. A retrospective case series of 265 patients found the incidence of simultaneous gout and septic arthritis to be 1.5%. One theory, known as вЂњcrystal shedding and strip mining,вЂќ posits that inflammation induced by infection releases crystals from the synovial membrane. Alternatively, gouty erosion may provide a substrate for bacterial seeding. GBS is an uncommon cause of septic arthritis, causing 2.9% of cases in one series. The incidence of invasive disease in non-pregnant adults is on the rise, however, with reports of a two to four-fold increase over the past two decades. Clinicians should thus be aware of this trend as well as the association invasive GBS with serious underlying conditionsвЂ” namely diabetes, malignancy, neurologic disease, and cirrhosis. Postpartum Diabetes Insipidus: An Interesting Path to a Rare Diagnosis Lisa Riley, MS4, DMA; Nardine Saad, MD; Hilary K. Seligman, MD, MAS University of California, San Francisco Case: A 31-year-old woman presented with 4 days of worsening thirst and increased urination 7 months after normal delivery of a healthy baby girl. Her symptoms began acutely during a cross-country airplane flight 5 weeks prior to her presentation and had progressively worsened, particularly over the previous 4 days. She reported daily fluid intake of about 4 gallons, associated with hourly urination during the day and frequent nocturia. Her review of systems was significant for increased fatigue and a mild, diffuse headache that was partially relieved by acetaminophen. She continued to exclusively breastfeed her infant without a noticeable change in her milk supply. The infantвЂ™s growth and development were reportedly normal. The patientвЂ™s VS and PE were normal. Serum sodium (141mmol/L), Ca (9.4 mg/dL), Cr (0.72mg/dL), and osmolality (288mOsm/ kg) were normal. Her urinalysis showed a specific gravity of 1.002, sodium of 28mmol/L, potassium of 17mmol/L, chloride of 32mmol/L, and osmolality of 124mOsm/kg. A urine pregnancy test was negative. With 24 hours of water deprivation, her urine specific gravity increased from 1.002 to 1.021 and urine osmolality increased from 124 to 664mOsm/kg. Her serum osmolality and serum sodium remained steady. Two hours after intranasal desmopressin (dDAVP) was administered, her urine osmolality increased to 842mOsm/kg without any change in her serum osmolality, indicating a partial central diabetes insipidus. Over the subsequent weeks, dDAVP was titrated to achieve relief of her thirst symptoms. Discussion: Diabetes insipidus (DI) presents with polydipsia and polyuria. The source of DI (central or nephrogenic) can be assessed by responses to a water deprivation test and a dDAVP challenge. Central DI is caused by inadequate anti-diuretic hormone (ADH) production by the pituitary gland. Generally in central DI, urine osmolality increases somewhat with water deprivation, but increases dramatically with dDAVP, a synthetic analogue of ADH. In nephrogenic DI, ADH production is intact but kidney response to ADH is impaired. Therefore, urine osmolality increases only mildly (if at all) with both water deprivation and dDAVP administration. With water deprivation, our patient's urine osmolality rose from 124 to 664mOsm/kg, considered a submaximal response. After dDAVP, the patient's urine osmolality 62 rose significantly higher, from 664 to 842. This 27% increase confirms that the patient was producing inadequate ADH to maintain homeostasis. This submaximal increase indicates a partial central DI, as opposed to a complete central DI which would show an increase of 100% or more. Springing a Leak: Pancreatic Duct Disruption from Acute Pancreatitis Michelle Rios, MD University of California, Los Angeles Case: A 34-year-old man with a history of alcohol dependence presented to the emergency room with complaints of epigastric abdominal pain and increased abdominal girth over the previous two months. Physical examination was notable for significant cachexia, abdominal distention with shifting dullness, and dullness to percussion over the right lung field. He had no stigmata of chronic liver disease. Laboratory studies revealed an elevated serum lipase. His serum albumin was low, but coagulation studies and transaminase levels were within normal limits. A CT of the abdomen showed massive ascites, peripancreatic fluid collections, and fat stranding consistent with acute pancreatitis. Chest imaging showed a large right pleural effusion. Thoracentesis and paracentesis were performed, and pleural fluid analysis was consistent with an exudate with an elevated amylase level. Ascitic fluid analysis revealed a low serum-albumin ascites gradient, elevated total protein, and elevated amylase level. After therapeutic paracentesis and thoracentesis, the fluid rapidly re-accumulated despite treatment with fluid restriction, sodium restriction, furosemide, and octreotide. ERCP showed extravasation of contrast into a three-centimeter cystic structure in the pancreatic head. A plastic stent was placed in the area of extravasation. Dietary restrictions were continued along with furosemide and octreotide. After repeat paracentesis the patient did not re-accumulate fluid and was discharged from the hospital. Discussion: This case illustrates the need for a thorough evaluation of ascitic fluid, particularly on the initial presentation of ascites. Given this patientвЂ™s drinking history a preliminary diagnosis of cirrhotic ascites could have been entertained. The lack of evidence of severe liver disease and the presence of a low serum-albumin ascites gradient warranted further investigation, ultimately revealing a pancreatic source. As pancreatic ascites does not often respond to medical therapy alone, it is important to diagnose to allow appropriate endoscopic or surgical intervention. A Fine Balance Kristen Rogers, MD; Payam Afshar, MD Scripps Green Hospital Case Presentation A 53-year-old male with cirrhosis secondary to HCV-1 and known esophageal varices presented with a one week history of intermittent cramping pain in the lower abdomen. The pain increased from mild to severe one day prior to admission. Associated factors included loose stools for three days and an isolated fever of 102.2 one week prior to admission. Physical exam revealed diffuse abdominal tenderness to palpation and was otherwise normal. CT scan of the abdomen and pelvis with IV contrast demonstrated splenomegaly, varices, superior mesenteric vein (SMV) filling defect and mesenteric stranding. MRI with MRA/V revealed a patent left portal and splenic vein, SMV thrombus with extension into the main and right portal vein, and subtotal thrombus of the SMV branches. Of note, the patient had an ultrasound of the abdomen one month prior to admission positive for portal hypertension but with no evidence of portal vein thrombosis. The patientвЂ™s Activated Protein C Resistance/ Factor V Leiden ratio was abnormal at 1.2 suggesting Factor V Leiden mutation. The patient was discharged home on Lovenox and Coumadin. Discussion: Mesenteric ischemia is a relatively rare disorder characterized by decreased intestinal blood flow resulting in ischemia and subsequent damage. Early recognition is imperative secondary to its high morbidity and mortality rate. The most common presenting symptom is severe abdominal pain out of proportion to physical exam findings. Associated symptoms include nausea and vomiting (50%), diarrhea (50%), and melena or hematochezia (15%). Mesenteric ischemia can be caused by an arterial or venous etiology and can be either hemodynamically occlusive or non-occlusive. Approximately 5-10% of mesenteric ischemia cases are due to mesenteric vein thrombosis of which 80% has an identifiable cause. Known risk factors include inherited and acquired hypercoagulable states, infection, inflammation, portal hypertension and trauma. 75% of patients with SMV thrombosis have an underlying hypercoagulable state. Hence, all patients with SMV thrombosis should be tested for a hypercoagulable state even if they have another known risk factor for SMV thrombosis. Our patient was found to have Factor V Leiden mutation which is only found in about 5% of the population. Factor V Leiden is a codominantly inherited disease due to substitution of glutamine for arginine at position 506 in factor Va. This position is necessary for Activated Protein C (APC) binding and subsequent Factor V degredation. However, the binding site is altered by the mutation resulting in ongoing thrombin production and a hypercoagulable state. Even though our patient has known portal hypertension, his abdominal ultrasound immediately prior to presentation revealed a patent portal vein with no evidence of thrombus. This suggests that the thrombus originated in the SMV with extension to the portal vein. In comparison, a mesenteric thrombus secondary to portal hypertension typically originates in the portal vein and may extend to the SMV. It is important to distinguish that this patientвЂ™s SMV thrombosis occurred as a probable independent cause from his portal hypertension because the 63 former requires anticoagulation but the latter does not always. All patients with SMV thrombosis should receive lifelong anticoagulation therapy in the presence of a hypercoagulable state unless contraindicated. Our patient will need close follow-up and continual assessment of the risk-to-benefit ratio regarding anticoagulation therapy in the face of progressive liver disease. son who has one or more of the following cardinal symptoms: (1) dermal macule or plaque with sensory loss, (2) nerve enlargement, or (3) skin smear positive for acidfast bacilli. Although leprosy can have a protracted onset and be difficult to recognize, cure is achievable with appropriate multidrug therapy. A Clinic Presentation of Borderline Lepromatous Leprosy Radiation-Related Left Main Coronary Artery Disease and Subclavian Artery Stenosis in a Patient Previously Treated for Non-Hodgkin Lymphoma Yasmin Sacro, MD; Stanley Amundson, MD Scripps Mercy Hospital Arash M. Saemi, MD; Dylan Wessman, MD Naval Medical Center San Diego Case: An 82-year-old Cuban male presented to a community clinic with complaints of a left wrist dermal plaque and multiple maculopapular pruritic lesions on his extremities present for approximately 3 months. Patient also complained of longstanding right hand paresthesias and weakness. Anti-histamine blockers provided mild relief of pruritic symptoms, yet persistence of lesions and paresthesias remained. Past medical history included moderate-severe aortic insufficiency, coronary artery disease, and chronic renal insufficiency. He had no new medications. Patient had immigrated from Cuba 20 years prior and had no return travel to his country of origin. He had no recent foreign travel nor sexual activity. On physical exam pertinent findings of the skin revealed a left dorsal wrist 3 x 5 cm plaque with normal coloration and central anesthesia, right extensor olecranon 1.2 x 1.3 cm plaque also with central anesthesia and ulceration, as well as multiple maculopapular erythematous lesions measuring 0.5-1cm scattered on bilateral posterior calves. Neurological exam revealed an enlarged, palpable right ulnar nerve and positive right TinelвЂ™s sign. Atrophic muscle loss of the right thenar eminence as well as bilateral intraosseous muscle atrophy were also noted. Deep tendon reflexes and sensation were notably decreased in the distal extremities. Labs revealed lymphopenia with WBC of 4.0 and 22% lymphocytes, and microcytic anemia with hemoglobin 10.4 and MCV 78. Complete metabolic panel, TSH, B12, glucose, folate and treponema antibody were within normal limits. HIV was found to be negative with a CD4 count of 270. Skin biopsy was performed which revealed AFB positive smear (3+) with perineural granuloma formation seen. Given the patientвЂ™s multiple, asymmetric lesions with diminished sensation and many AFB seen on skin biopsy, a diagnosis of borderline lepromatous leprosy was made. Case: A 38 year-old male was diagnosed with Stage IIB NHL and treated with CHOP-R (Cyclophosphamide, Doxorubicin, Vincristine, Prednisone, Rituxan) and mantle field irradiation. Four years later, he developed dyspnea on exertion and exercise intolerance. An exercise treadmill test was positive for ischemia, and he underwent coronary angiography revealing severe stenosis of the left main coronary artery. Initially, this was treated percutaneously, but he twice developed in-stent restenosis and subsequently underwent 2-vessel CABG surgery. Six months later, the patient developed recurrent anginal symptoms, and an exercise stress echocardiogram was concerning for ischemia of the anteroseptal wall. Angiography revealed patent bypass grafts and severe stenosis of the left subclavian artery, which was treated successfully by balloon angioplasty and an 8 x 18 mm stent. The patientвЂ™s anginal symptoms resolved, and a repeat exercise stress echocardiogram was negative for inducible ischemia. Discussion: Hansen Disease, commonly known as leprosy, is uncommon in the U.S.and poses difficult diagnostic and treatment challenges. Leprosy has a range of clinical manifestations which are determined by the hostвЂ™s cell-mediated immune response. The route of transmission for leprosy remains unclear, but evidence suggests respiratory transmission via droplets. It is also one of the most common causes of peripheral neuropathy in developing countries. Perineural granuloma formation and nonspecific inflammatory infiltrate/fibrosis can result in neuropathic symptoms. Leprosy is diagnosed in a per- Discussion: Patients with lymphoma potentially can be cured by combination chemotherapy and radiation therapy. However, long-term survivors are at increased risk for developing cardiac and vascular disease from their prior treatments. Radiation therapy to the chest can cause accelerated atherosclerosis involving the ostial and proximal segments of large arteries. We present a unique case of a patient successfully treated for NHL with combination chemotherapy and mantle field radiation, who subsequently developed both left main coronary artery disease and left subclavian artery stenosis requiring percutaneous and surgical interventions. This case highlights the increased risk among long-term lymphoma survivors for accelerated atherosclerosis related to prior radiation therapy, as well as the need for physicians to maintain a high level of clinical suspicion whenever these patients present with anginal symptoms. Fulminant Liver Failure as Initial Presentation of Disseminated Strongyloides Hyperinfection in a HIVNegative Alcoholic Patient Neha Sampath, MD; Steve Yung; Sally Stipho Scripps Mercy Hospital Parasitic infection with Strongyloides sp. is a rare and unusual cause of liver failure in developed countries. If 64 left untreated or misdiagnosed, mortality with disseminated Strongyloides hyperinfection is almost 100%. Timely diagnosis, is thus, of critical importance in suspected cases. Case: 69 year old Laotian male with a history of noninsulin dependent diabetes, alcoholism and variceal bleeding presented with acute onset of hematemesis and melena. Patient was also found to be in fulminant hepatitic liver failure associated with a severe coagulopathy, thought initially to be secondary to alcoholic liver disease. He also had an unusual rash on his torso and bilateral lower extremities. After the patientвЂ™s recurrent variceal bleed had been stabilized with emergent endoscopic treatment and blood product transfusions, he continued to have non-bloody diarrhea and abdominal distention. He underwent imaging studies which revealed diffuse colonic wall thickening, severe circumferential gall bladder wall thickening in absence of cholecystitis, and severe liver heterogeniety indicative of innumerable masses or microabscesses. He also had bilateral patchy infiltrates on chest xray. Stool studies came back positive for Strongyloides stercoralis larvae as well as Clostridium difficile toxin. Lumbar puncture was performed and CSF studies were negative for CNS involvement. Patient was treated with Metronidazole and five day course of Ivermectin. His abdominal distention and diarrhea improved. However, he had a complicated hospital course with respiratory failure requiring tracheostomy, and renal failure requiring dialysis. He also had persistent direct hyperbilirubinemia, and fever concerning for cholangitis, for which he underwent percutaneous cholecystostomy. The procedure was complicated by hemoperitoneum. He was eventually made comfort care by his family and passed shortly thereafter. Discussion: Disseminated strongyliadisis in a patient without evidence of immunosuppresion is an unsual cause of multi-organ failure, but should be considered in a patient with risk factor such as previous residence in or travel to endemic areas. No consensus exists on exact duration of treatment with anti-parasitic medicines, and further clinical trials are needed to determine this. Canned Tuna: A Biological Weapon? Pallabi Sanyal, MD; Kandan Baban, DO; Kenneth Grudko, MD Scripps Green Hospital Introduction: An 85 year old retired fisherman with emphysema was admitted with 2 days of non-bloody emesis, diplopia, bilateral ptosis, and orpharyngeal secretion pooling. Extraocular movements were slow and he had limited tongue protrusion. Strength and sensation were otherwise grossly intact. On day 3, the patient's ability to clear secretion worsened and he was transferred to ICU for ventilatory support. On Day 4, the patient's 91 year old wife (pt#2) came to visit him, and was not feeling well. She awoke in the AM with profound weakness, ataxia, dysarthria, diplopia, and dysphagia. Her physical exam was essentially identical to her husbandвЂ™s except for 3/5 right upper extremity proximal strength. Case: Pt#1's initial CBC and chemistry were normal. Head CT and MRI/MRA did not reveal any evidence of an acute stroke. Lumbar puncture showed 1 white cell, red cells, 31 mg/dl protein, and 99 mg/dl glucose. The gram stain and the acid-fast bacilli stain were negative. Nerve conduction study and electromyogram showed non-specific mild slowing of motor and sensory velocities. Repetitive stimulation testing was not obtainable. Acetylcholine receptor binding and blocking antibodies were negative. Pt#2 also had a negative head Ct scan. A niece accompanying pt#2 informed staff that the wife has been canning food for years, particularly tuna. Blood,stool cultures, serum and food samples from home were collected from both patients, and were sent to the Public Health Department for indentification of botulinum spores and biotoxin assay. Given the high suspicion of botulism, with state approval, equine antitoxin flown in from Los angeles was administered. Serum samples from both patients returned positive for botulinum toxin A; stool was negative. Pt#1 showed mild improvement on repeat EMG days later. Pt#2 exhibited mild improvement, but neither pt fully recovered. Discussion: The clinical presentation of food borne botulism (eight strains) occurs within 12 to 36 hours of ingestion. Both patients in this case unfortunately presented much later. Our differential initially included brainstem stroke, myasthenia gravis, and guillain barre syndrome. Arrival of pt#2 in addition to the added canning information solidified our diagnosis. The botulism toxin itself binds to the presynaptic site, and causes irreversible damage to acetylcholine release. Plasticity of these sites requires at least six months. Treatment, in addition to supportive care, consists of an equine serum antitoxin. Duration of mechanical ventilation and decreased fatality have been loosely associated with treatment. C.Botulism is the deadliest bacterial toxin known and has been developed and employed as a bio-weapon. It is important for physicians to be able to diagnosis and treat this rare form of poisoning. Lithium-Induced Thyroiditis: A Rare Case of Thyrotoxicosis Maria Scremin, MD; Daniel Peng, MD; Anna Milanesi, MD Cedars-Sinai Medical Center Lithium has been recognized for many years to have inhibitory effects on the thyroid, most commonly hypothyroidism and goiter. In the setting of lithiumвЂ™s anti-thyroid effects, lithium-associated thyrotoxicosis is unexpected and critical to recognize given the potential to worsen mania in bipolar patients. Rarely, lithium has been associated with thyroiditis, an inflammation of the thyroid gland. In such cases, rather than a hypothyroid state, the patient experiences high circulating levels of thyroid hormone. A 23 year old man with bipolar disorder on 65 chronic lithium therapy was admitted to the hospital with confusion, tachycardia, and acute mania. On physical exam, patient was acutely manic, diaphoretic and tachycardic with heart rate of 130 bpm. There was no apparent goiter or neck pain. Initial serum lithium levels were elevated well into the toxic range. Laboratory studies were significant for a suppressed TSH and elevated T4. The patient had no personal or family history of thyroid dysfunction. Radioactive uptake scan was virtually undetectable at less than 1%, consistent with a diagnosis of thyroiditis. Lithium was discontinued and the patient was started on atenolol for his tachycardia. Three weeks later, thyroid studies showed adjusted T4 levels trending downward, while TSH remained suppressed at < 0.02. Lithium levels decreased from 2.3 on presentation to below toxic range. The patientвЂ™s tachycardia and diaphoresis resolved with b-blocker therapy, although his mania persisted. The patient was transferred to the psychiatric ward where he continued to be treated for his manic state. This case illustrates the potential for acute thyroiditis causing thyrotoxicosis in a patient treated with lithium carbonate. Almost exclusively, lithium causes hypothyroidism due to interference with synthesis and release of thyroid hormone. Although hyperthyroidism is rarely seen while on lithium, it is an important side effect to consider given itвЂ™s potential to worsen or incite mania. Correct diagnosis of the etiology for the thryrotoxicosis is also vital, as the course and treatment of thyroiditis differs significantly from that of hyperthyroidism due to more common causes such as GraveвЂ™s disease or toxic nodular goiter. Huge Benign Mass Leading to Shock Roshni Shah, DO; Babak Eshaghian, MD, MS; Michael Hochman, MD; Babak Saedi, MD; Aarti Chawla, DO; Justin Lee University of Southern California Case: A 52 year old G2P2 Hispanic female with significant history of menororrhagia presented with the complaint of abdominal pain, 1 year of increasing abdominal mass, nausea, vomiting & dyspnea. PE was significant for hypotension (97/61), tachycardia (131), tachypnea (40), and a large, firm, non-tender abdominal mass with warm periphery. She had an anion gap metabolic acidosis 27, bicarbonate 11, Cr 2.83 (baseline 0.7 in 2003), leukocytosis with maximum white count 24.9 (87.4% neutrophils), anemia with Hb 5.7 and lactate 12.0. CT of abdomen/pelvis revealed a 33 cm bilobed heterogeneous enhancing vascular mass arising from the uterine fundus. Initially the patient was presumed to be in septic shock, and aggressively fluid resuscitated, transfused several units of packed red blood and empirically treated with cefepime, vancomycin, and flagyl. All the cultures drawn were negative. She did not respond to the aforementioned management and continued to remain hemodynamically unstable; therefore she was started on norepinephrine and vasopressin. On the following day she experienced what was presumed to be Acute Respiratory Distress Syndrome (ARDS) and shock from her severe inflammatory process and was intubated. She subsequently became oliguric and required dialysis. She eventually became anuric with elevated urea nitrogen levels and FeNa of 0.7%. She continued to remain anemic despite the several attempted transfusions, the hypothesis being she was bleeding from the mass and it needed to be removed. On day five, pressors were weaned, and her renal function improved so that she no longer required dialysis. On day nine, she was taken to the operating room by OBGYN and underwent colposcopy, exploratory laparotomy, total abdominal hysterectomy, and bilateral salpingooophorectomy. The operative findings revealed entire abdominal and pelvic cavity to be filled with a large, fleshy, soft tissue tumor with a necrotic and hemorrhagic center which appeared to be rising from the fundus of the uterus weighing five kilograms. There were multiple large ruptured fibroids. There was some nectrotic tissue on the omentum. Pathology report confirmed necrotic leiomyoma. Post-op she remained stable, and on post-op day 17 she was ambulating, voiding, tolerating regular diet, and was discharged. Discussion: Leiomyoma is the most common tumor in women; its behaviors and complications are wellrecognized. Clinical manifestations include uterine bleeding, pelvic pressure and pain, and reproductive dysfunction. Increased size can cause the mass to become necrotic, hemorrhage, or cause obstruction of surrounding structures, leading to severe complications. The presence of large areas of damaged or necrotic tissue can mimic an infectious focus. Systemic inflammatory response syndrome (SIRS) is characterized by 2 or more of: temp >38.5C or <35C, HR >90, RR>20, or PaCO2 <32mmHg & can initiate shock and multi organ failure. The tissue injury from necrotic mass can initiate an acute and severe inflammatory response. Multiple organ failure may be due to an over vigorous manifestation of this normally protective defense mechanism. This case is unique due to the severe potentially life-threatening inflammatory syndrome leading to shock from a relatively benign mass. The ruptured necrotic 5kg leiomyoma led to intraabdominal hemorrhage, severe inflammatory response, and multi-organ failure. After excising the 33cm mass, she stabilized and recovered. Prompt surgical management was essential to make the diagnosis and save the patientвЂ™s life. Strongyloides Hyperinfection in a Patient with Dermatomyositis Linda Sharp, MD; Darrell Harrington, MD Harbor-UCLA Medical Center A 39-year old Guatemalan man had 1 day of shortness of breath, nausea, vomiting, and abdominal pain. He had a tracheostomy 2 months prior, and he noted 1 day of increased tracheal secretions. Normally he used a ventilator at night, but for several days he had required constant respiratory support. On the morning of admission the patient noted diffuse, constant abdominal pain as well as constipation for several days. He had dermatomyositis for 66 5 years and a recent hospitalization complicated by A. baumanii pneumonia. Medications included prednisone, methotrexate, and leucovorin, plus rituximab 4 months prior. T 99.8, HR 123, BP 90/60. He was cachectic and had coarse breath sounds bilaterally. Abdomen was distended, diffusely tender; bowel sounds were absent, and there were positive peritoneal signs. He had diffuse muscle wasting. WBC 30.5, P83, bands 12, E none. ABG: pH 7.47, PCO2 56 mmHg, PO2 56 mmHg. CT scan showed bibasilar infiltrates, dilated fluid filled loops of small bowel with 4-5 mm wall thickness. He was given antibiotics to cover for pulmonary and enteric sources, 2 units of PRBCs after his Hgb dropped from 10.7 g/dL to 6.1 g/dL, pressors, and stress-dose corticosteroids. After 24 hours, his abdominal exam improved and after 48 hours he no longer required pressors. On hospital day 3 he had a macular rash that spread to his legs, abdomen, and back. Initial blood cultures grew K. pneumoniae and E. Coli. Sputum grew P. mirabilis, A. calcoaceticus-baumanii complex, K. pneumoneae, and E. Coli. Fever persisted, and blood and respiratory cultures remained positive throughout the hospital course despite adequate antibiotic therapy. After one week, the patientвЂ™s level of consciousness declined, and he required 70% FiO2. Bronchioalveolar lavage returned bloody, frothy fluid in several segments consistent with alveolar hemorrhage, and showed Strongyloides stercoralis. Biopsy of the rash also revealed strongyloides. He was started on albendazole on day 10 and switched to ivermectin on day 11. However, on day 11, the patient required escalating amounts of oxygen, with poor arterial oxygenation on 100% oxygen. The patient expired on day 12. Strongyloides stercoralis is endemic to tropical and subtropical regions, with global prevalence of 30 to 100 million cases. Prevalence in endemic areas ranges from 1% to 30%, including some areas in the U.S. Individuals may be asymptomatic carriers with or without peripheral blood eosinophilia. This patient likely acquired Strongyloides in the remote past, then developed hyperinfection as a result of immunosuppressive therapy. Understanding the geographic distribution of Strongyloides endemicity may help clinicians identify at-risk patients prior to immunosuppressive therapy, thus preventing the often-fatal hyperinfection. outcome. Sometimes Only Time Will Tell Bryant Sheh, MD; Siamak Basiratmand, MD Olive View-UCLA Medical Center Case: A patient presented to UCLA-Olive View Medical Center several times over a 2 week period, each time with new symptoms and signs, before a satisfactory diagnosis was reached. A 25 year old female presented to the ED complaining of several years of intermittent abdominal pain, diarrhea, and pelvic pain, acutely worsened the past 3 days. She denied fevers and weight loss. Review of systems was otherwise negative. Past medical history was significant for treatment of Salmonella gastroenteritis, Helicobacter pylori gastritis, and Gardnerella vaginosis in Mexico over the last year. Her medications included famotidine, butylscopalamine, and recently begun oral contraceptives. Family history was significant for an aunt with lupus. Social history was noncontributory. Vital signs, physical examination and labs were within normal limits. She was diagnosed with irritable bowel syndrome, urinary tract infection and discharged with ciprofloxacin, psyllium, donnatal, and omeprazole. The patient returned ten days later reporting dysuria, a new oral lesion, and lower extremity pain. A 1cm aphthous ulcer was noted on the lower lip. Diffuse tenderness was present in both lower extremities. Urinalysis was abnormal and the patient was discharged with nitrofurantoin and a diagnosis of resistant urinary tract infection. Two days later, she returned with severe bilateral ankle pain, vaginal pain, and a new rash. Her temperature was 38.1В°C. Two new 5mm ulcers were seen on the right labia majora. Raised, erythematous, tender nodules on her shins were consistent with erythema nodosum. Laboratory testing revealed WBC 10.1, ESR 33, CRP 51, and a negative ANA. The patient was admitted for observation. An infectious and serologic workup for secondary causes of erythema nodosum was ultimately negative. A PPD was considered positive when a 2cm red, tender bump formed at the site. Biopsy of the oral ulcer revealed nonspecific inflammatory cells and a negative viral culture. When pain and ambulation improved with ibuprofen, the patient was discharged home. The patient was readmitted three days later with severe odynophagia. New oral ulcers were seen on the soft palate. Her right eye was red and the right knee swollen. Knee arthrocentesis revealed lymphocytosis and negative cultures. Ophthalmologic exam confirmed uveitis. Given the clinical picture of uveitis, oral and genital ulcers, arthritis, erythema nodosum, and what now appeared to be a positive pathergy test, the patient was diagnosed with BehcetвЂ™s disease. Colchicine and prednisone were begun. Within days, her symptoms resolved and she was discharged home. Discussion: BehcetвЂ™s disease is a clinically-diagnosed systemic inflammatory disorder. Patients such as this who initially present with partial symptoms and signs can make recognition of the disease challenging for any physician. A delay in diagnosis is typical. Ultimately, clinicians should maintain a broad differential when confronted with unusual symptoms or those that are refractory to usual treatment. Refractory Hyponatremia and Abnormal Thyroid Function Test in an End Stage HIV Patient 67 Rong (Cindy) Shi, MD; Terence Gan, MD, MPH; Bhupinder Khehar MD Scripps Mercy Hospital Background: Symptomatic HIV infection is a common cause of hyponatremia & many patients have SIADH. Common cause of HIV related SIADH include pneumonia, CNS infection, and malignancy. In euvolemic hyponatremia, adrenal insufficiency or hypothyroidism needs to be ruled out before diagnosis of SIADH is made. Case Presentation: 44 year old homosexual male, end stage HIV presented with generalized weakness. Recent admission for weakness, found hypovolemic hyponatremia, improved after hydration; discharge Na 123 with diagnosis SIADH secondary to HIV. Re-admitted 1 week later with progressive weakness, admission Na 124. PE: T 100.7, RR 22, HR 75, BP 97/50 without orthostatic changes. Cachectic, lethargic but A&O x3 male, otherwise no pertinent positive findings. LABs: WBC 1.8, CD4=3, Na 124, K 3.5, renal and liver panel normal, TSH 1.4520, fT4 0.53, fT3 1.83, thyroglobulin 10.4, antithyroglobulin 1.0, urine Na 130, urine Osm 951, FeNa 1.17, basal cortisol 4.9, after ACTH 20.3, extensive ID work up negative, rest of pituitary hormone workup normal. Imaging: CT head on prior admission negative. MRI brain (HD #3): 2.3 cm mildly enhancing lesion in the region of left caudate head. Repeat CT (HD #9): Enlargement of mass-like density in left caudate nucleus. Pathology: Brain biopsy at left basal ganglion lesion (HD #14): Diffuse large B-cell lymphoma, (+) CD20, (-) CD5, (-) CD10. Treatment: Refractory euvolemic hyponatremia: Minimally response to fluid restriction, salt tablets, demeclocycline, normal saline, or levothyroxine, requires continuous hypertonic saline to maintain serum Na. Only after lymphoma treatment initiated, serum Na quickly improved to normal range, no additional treatment necessary. Hypothyroidism: levothyroxine started on HD #2, titrated up to 75 mcg on discharge with no improvement in fT4, eventually fT4 corrected at dose 125 mcg. CNS lymphoma: dexamethasone started after pathology report, then MTX with leucovorin rescue. Patients overall condition much improved including energy level and normalized Na level, despite low fT4. Patient was discharged on HD #27 with oncology follow-up. Discussion: Refractory euvolemic hyponatremia: hyponatremia not responsive to conventional SIADH treatment, neither to thyroid hormone replacement, but dramatically improved after dexamethasone started for treatment of CNS lymphoma. Thus, etiology might be multifactorial, but mostly CNS lymphoma causing SIADH. CNS lymphoma: Quick-growth mass lesion with doubling time в‰¤ 3 weeks, if ruled out infection, highly suspicious for lymphoma, which has quick response to steroids, dexamethasone would reduces edema, also is treatment for lymphoma, thus quickly improving hyponatremia. Hypothyroidism: May represent an extreme of euthyroid sick syndrome, central hypothyroidism debatable, as rest of pituitary hormones was all normal. Possible hypotheses: HIV related vs. mass effect vs. lymphocytic infiltration. Conclusion: SIADH in HIV patient is not uncommon, however, with symptomatic refractory hyponatremia, further work up should be warranted, including MRI to rule out primary CNS lymphoma. Anti-NMDA Receptor Encephalitis: A Rare Diagnosis with a Treatment Neeharika Srivastva, MD University of California, Los Angeles Learning Objectives: 1. Develop a differential diagnosis of reversible etiologies of encephalitis 2. Management of anti-NMDA receptor encephalitis A 27-year-old female presented after her first witnessed generalized tonic-clonic seizure. One week prior to presentation, she had had flu-like symptoms, difficulty with speech & typing, & personality changes. Her symptoms were attributed to her identical twin sisterвЂ™s recent death from meningo-encephalitis. In the ER, she had fluctuating levels of consciousness with episodes of nonsensical laughing and hysterical crying. She was unable to follow commands, and had decreased muscle tone, rhythmic movements alternating with semi-purposeful movements (automatisms) of the upper extremities. A lumbar puncture demonstrated 65 WBC with 100% lymphocytes. She was given phenytoin, acyclovir, vancomycin and ceftriaxone. An MRI study of the brain demonstrated a T2 FLAIR hyperintensity within the cerebral sulci. MRV, MRA and CT scans of the brain were unremarkable. Thorough investigations to evaluate for encephalitis were pursued that demonstrated N-methyl-D-aspartic acid (NMDA) receptor antibodies in the CSF. Incidentally, the patientвЂ™s twin sisterвЂ™s final autopsy results were completed at this time and also showed these antibodies in the CSF. Anevaluation was undertaken for an underlying malignancy, including a bilateral salpingo-oophorectomy, all of which was unrevealing. The patient received immunotherapy with plasmapheresis, cyclophosphamide, and methylprednisolone with no improvement. She remained non-communicative, unable to follow commands, combative, and displayed oral dyskinesias. Following surgery and further cycles of immunotherapy including rituximab, she began to open her eyes and within two weeks of surgery she could follow commands appropriately. After three months of neuro-rehabilitation, the patient is now independent in her self-care activities. She has fre- 68 quent facial twitches, but is able to read and socialize appropriately with friends. She plans to attend community college. Anti-NMDA receptor encephalitis presents with a neuropsychiatric syndrome due to antibodies against NR1-NR2 heteromers in the hippocampus. It occurs mostly in young women who have tumors, commonly ovarian teratomas. Early tumor removal followed by immunotherapy leads to a better prognosis with minimal risk of recurrences. This case illustrates the importance of developing a comprehensive differential diagnosis for encephalitis presenting with neuro-psychiatric symptoms and then pursuing definitive diagnostic evaluation for those illnesses that can be treated. The differential diagnosis of reversible causes of encephalitis includes drug ingestion, porphyria, Systemic Lupus Erythematosus cerebritis, Sjogren's syndrome, Hashimoto's encephalopathy, Cytomegalovirus, Herpes Simplex Virus and paraneoplastic encephalitis. Early diagnosis of anti- NMDA receptor encephalitis allowed for aggressive management of the patient based on experiences from various case reports. This led to a meaningful recovery for the patient. Bizarre Behavior And Bezoar - A Thought To Chew On Joanna Tan, MD; Jasminka Criley, MD, FACP, FHM St. Mary Medical Center Background: Chewing gum is a common behavior. Swallowing gum is often thought to be harmless. Gastric bezoars are a rare finding involving <1% of upper gastrointestinal endoscopy and are often related to decreased gastrointestinal motility. Chewing gum has been described as a foreign object bezoar, typically in textbooks. Reported cases of chewing gum bezoar in the literature are few and often involve the pediatric population. In contrast to nonmedication bezoars, pharmacobezoars may produce additional symptoms related to the release of active ingredients or produce subtherapeutic medication levels. Case: 54 year old with history of seizure, pseudoseizure, depression and diabetic gastroparesis presented with yet another episode of an acute periumbilical abdominal pain, nausea, vomiting, вЂњseizureвЂќ and bizarre behavior. Patient was recently discharged from a psychiatric ward and had a subtherapeutic phenytoin level. History was difficult to obtain but it appeared that gastrointestinal symptoms were most prominent. Patient had poor dentition and was mostly edentulous. Abdominal physical exam findings were inconsistent ranging from benign to periumbilical tenderness. Laboratory data was consistent with dehydration. Abdominal x-ray, ultrasound and computer tomography scans with oral contrast revealed no abnormalities. An esophagogastroduodenoscopy (EGD) performed 2 months prior revealed esophagitis and gastritis. Due to her failed medical management, a repeat EGD was performed revealing a large yellow crystalline mass, removed per os using basket with remnants broken by forceps and irrigated. Subsequently, her bizarre behavior and abdominal pain resolved. The patient then admitted to frequently swallowing gum вЂ“ up to a pack a day. It is suspected the bezoar consisted of chewing gum and pharmacobezoar. Conclusion: In a patient with multiple psychiatric disorders, a history of non-compliance, poor presentation history, inconsistent physical exam findings and benign laboratory and imaging results, it is difficult to not suspect malingering or a psychological component as a likely etiology of abdominal pain. However, a bezoar should be in the differential diagnosis if patients have a history of decreased GI motility, particularly with the presence of psychiatric disorders and subtherapeutic medication levels. It is still unknown if the bezoar caused her bizarre behavior by its mere physical presence or by the pharmacobezoar component. Stop Being a вЂњPain in the ButtocksвЂќ Laren Tan, MD; Raymond Yau; Gary Thompson Loma Linda University Pyomyositis is rare in the United States and is often overlooked when muscular pain is the initial presenting symptom. The concurrence of systemic illness including fever and leukocytosis obscures the clinical picture and without appropriate imaging the diagnosis will remain elusive. An 18-year-old female with no significant PMH was admitted because of sharp left gluteal pain. One week prior to admission, she reported of nocturnal sharp pain in the same region, which progressively worsened until she was no longer able to ambulate. The patient was febrile on admission and had leukocytosis. Other laboratory tests were including, urine analysis, C-reactive protein, ESR were normal. Initial CT scan of the abdomen/pelvis did not reveal any abnormalities. She was started on empiric antibiotics while blood cultures were pending. Despite aggressive treatment, she continued to have intermittent fevers and complained of increasing pain over her affected area. The patient had magnetic resonance imaging (MRI) of the abdomen and pelvis demonstrating significant diffuse muscle inflammation of the left iliacus muscle. Bacteremia was reported which later cultured coagulase-negative S. aureus. Significant inflammation of the iliacus muscle, along with fever and bacteremia were consistent with the diagnosis of pyomyositis. After initial MRI, repeat CT scan showed a small abscess that was percutaneously drained, resulting in considerable clinical improvement. A survey with transesophageal echocardiogram was done which documented no evidence of vegetations. Her symptoms continued to improve and she was discharged home. Historically pyomyositis has been differentiated into tropical pyomyositis or temperate pyomyositis. The latter is considered atypical in the United States and it gener- 69 ally occurs in immuno-compromised host and in intravenous drug users. The case presented here is amongst a handful of reports where spontaneous occurrence had taken place in a normal host with no risk factors. Even though temperate pyomyositis was once thought to be uncommon, early clinical suspicion along with proper imaging can help in the early diagnosis and aggressive treatment that usually results in a successful outcome. Treatment Failure of Ganciclovir for Ebstein-Barr Virus Induced Transverse Myelitis in a Systemic Lupus Erythematosus Patient T. Tan DO; R. Stewart BS; A. Zadeh DO; K. Raoufi MD Western University of Health Sciences We report a case of a 28 year old female, on prednisone and cellcept for systemic lupus erythematosus (SLE), who was diagnosed with EBV induced transverse myelitis. Ebstein-Barr virus (EBV) is a herpes virus that is spread by salivary secretions. Clinical manifestations can range from infectious mononucleosis to BurkittвЂ™s lymphoma. Cellular immunity is important in controlling the proliferation of EBV infected cells. On March 5, 2009, the patient presented to the hospital with lower back pain and bilateral lower extremity paresis with no loss of sensation. During her hospital stay, she progressed to complete paraplegia, complete anesthesia below T10, urinary retention and fecal incontinence. The diagnosis was made by magnetic resonance imaging (MRI) of the thoracic and lumbar spine, viral serologies and cerebrospinal fluid (CSF) analysis. The total protein in the CSF was 76 mg/dl, and the glucose was 48 mg/dl. PCR studies of the spinal fluid were negative for enterovirus, HHV6, HSV I and II, CMV and Varicella Zoster virus DNA, but were positive for EBV DNA at 44,984 copies per mL. The patient was treated with ganciclovir 10mg/kg/day intravenously for 4 weeks and then oral ganciclovir 60mg/kg/day for 4 weeks. The patient was seen in the hospital two and a half months later with no change in her neurological examination. The degree of damage to the nervous system prior to initiation of therapy may be an important factor in the treatment of EBV induced transverse myelitis. Patients with severe, irreversible axonal damage are less likely to benefit from ganciclovir alone. It is also possible that CMV hyperimmune globulin plays a role in the recovery of patients presented by Gruhn et al and Oettle et al. Loss of Health Insurance Leads to Rapid Progression of Kaposi Sarcoma J. Taylor, MA, MSIII1; R. Gharibjanyan, MD2; H. Van Gelder, MD2; J. Criley, MD1, 2 1 David Geffen School of Medicine at UCLA 2 St. Mary Medical Center Introduction: KaposiВґs Sarcoma (KS) is associated with HHV 8 in AIDS patients. Highly active anti-retroviral therapy (HAART) decreases KS risk. In this health insurance market, it is difficult for some patients to afford HAART. Case Report: A 49-year-old Caucasian male with 20year history of HIV/AIDS was admitted with GI bleed and necrotic KS lesions of the feet. He lost health insurance 2 years prior. He could not afford HAART. He followed a healthy diet and lifestyle to control his illness. He was doing well until about 6 months prior when he noticed violaceous lesions on his feet. Later , the lesions appeared on his face and subsequently the palate. Foot biopsy confirmed KS. Hewas treated with radiation therapy for local control. Three weeks later, he presented to the ED with weakness and foot pain. He also had diarrhea and dark creamy stools. On exam he had purpuric, nummular lesions over his extremities. His feet were necrotic and malodorous. His WBC was 26.7, Hb 4, Hct 12, platelets 25,000, T bili 1.3, D bili 0.8, AST 68, ALT 15, CD4+ count 14. He was transfused multiple times but continued to have GI bleed. Endoscopy showed hyperpigmented lesions in the duodenum, confirmed to be KS. He was found to have KS of the trachea, bronchial tree and likely the lungs. His condition rapidly deteriorated. He expired 1 month after presentation. Discussion: KS has become a rare AIDS-defining lesion. In 2004, the incidence of KS was 1/10 of what it was 10 years prior. HAART reduces KS incidence, prolongs time to treatment failure, and prolongs survival. The United States government passed the Medicare Modernization Act of 2003 requiring that Medicare Part D insurers provide medication therapy management (MTM) services. This legislation was to educate patients, improve outcomes and adherence. Previous studies have demonstrated the power of MTM programs in the setting of HIV/AIDS therapy. Patients were more likely to adhere to medical regimens, achieve significant reduction in viral load and improvement in CD4+ T-lymphocyte counts. Informing HIV/AIDS patients of such programs would have made a difference in the life of this patient. Had the patient been able to receive continuous management on HAART, he would have been less likely to develop end stage KaposiВґs Sarcoma. HIV/AIDS patients in California should be informed of possible Medical Part D eligibility. Uncomplicated Amebic Hepatic Abscess J. Taylor, MA, MSIII 1; A. C. Ortiz, MD2; T. Yang, MD2; C. Craig, MD1,2 1 University of California, Los Angeles 2 UCLA-Olive View Medical Center Introduction: The presenting symptoms of a hepatic abscess can be vague, with fever being the most commonly reported symptom by physicians. A high level of clinical suspicion is key to an appropriate diagnosis. 70 Case Report: A 42-year-old Latino male presented to his primary care physician with a one-week history of fevers, chills, myalgias and abdominal pain. His fever had been present for one week, and he symptoms of nausea, vomiting or diarrhea. His past medical history did not include any recent viral infections or gallbladder disease. He denied any recent surgeries. He was sent home from his initial outpatient visit with reassurance alone. As the pain persisted, the patient returned to his primary care physician and began a three-day course of antibiotics. A CT with contrast of the abdomen and pelvis revealed a 10x6 cm right-lobe hepatic abscess, and he was referred to our service for inpatient evaluation. Further history taking revealed a 2 pack-year smoking history, and occasional use of intranasal cocaine. The patient had traveled to Mexico three years prior to presentation. He was sexually active with several female partners, with intermittent use of condoms. He had intentionally lost weight in the previous three weeks by taking an herbal medication (HerbaLife). On Physical examination, the patient was anicteric, had a soft, non-tender abdomen. There was no guarding or rebound tenderness. The liver edge was not palpable. At the time of admission, the patient had a leukocyte count of 10.8/mm3 and a platelet count of 712,000/mm3. The patient had an AST of 20 u/l, ALT of 17 u/l. His alkaline phosphatase was slightly elevated at 171 u/l, bilirubin was normal at 0.5 g/dl and serum albumin was slightly decreased at 3.0 g/dl. He was started on intravenous metronidazole and ceftriaxone. The patientвЂ™s symptoms improved and repeat imaging three days after admission revealed a slight decrease in size of the abscess. Percutaneous culture of the abscess fluid was positive for Entamoeba histolytica. The patient was continued on intravenous metronidazole alone. A percutaneous drain was placed, and 75cc of bloody purulent fluid was removed. The drain was subsequently removed and the patient was discharged on oral metronidazole two days later. Discussion: This case illustrates that hepatic abscessвЂ™ can present with few symptoms, and recognition necessitates a high level of clinical suspicion along with mindfulness of risk factors. Risk factors associated with increased mortality include a bilirubin greater than 3.5 g/dl, serum albumin less than 2 g/dl, multiple abscesses and/or a large abscess cavity. Mortality associated with an uncomplicated hepatic abscess, such as the case we present, is less than 1% if treated with antibiotics and appropriate drainage. The Great Escape: An Unexpected Diagnosis from Lung Entrapment Ana Cecilia Uribe-Wiechers, MD; Visal Nga, MD; Hemal Kadakia, MD; Joyti Datta; Alexander Stein, Jasminka Criley, MD St. Mary Medical Center and David Geffen School of Medicine at UCLA Introduction: Finding the etiology of lung entrapment can be challenging. Lung entrapment can result from malignant or nonmalignant diseases, including sequela of remote inflammatory condition. We present a case of lung entrapment that led to a diagnosis of pleural tuberculosis only after surgical pathology. Case Description: A 48 year old previously healthy Caucasian man was admitted with one month history of worsening shortness of breath, dry cough, easy fatigability, night sweats and fevers. He denied hemoptysis, weight loss, weakness, or recent travel. Physical examination was significant for fever to 102 F, decreased breath sounds and dullness to percussion on the right hemithorax. PatientвЂ™s cell counts and chemistries were normal and HIV was negative. Chest x-ray showed a large right pleural effusion, subsequently determined to be exudative with lymphocytic predominance. Pathological review of the pleural fluid was suspicious for lymphocytic lymphoma, however flow cytometry and cytology were negative. Broncho-alveolar lavage, and sputum cultures were unrevealing. Bone marrow biopsy was negative. Other laboratories including blood cultures, RF, ANA, and ADA were also non-diagnostic. Thus, cardiothoracic surgeon was consulted for mediastinoscopy and lymph node biopsies. On mediastinoscopy, paratracheal lymph nodes were firm and rubbery; a frozen section showed small caseating granulomata. A full thoracotomy was performed, revealing trapped right lung. A formal decortication was performed to liberate the lung. Analysis of the lymph nodes and pleural biopsy revealed caseating granulomata with AFB stain positive for tuberculosis. Pt was immediately started on Rifampin, INH, Pyrazinamide and Ethambutol (RIPE). Six weeks later the pleural fluid culture grew mycobacterium tuberculosis. Discussion: Pleural effusions and trapped lung are challenging diagnosis requiring multi pronged approach to obtain a definitive diagnosis. Lung entrapment is characterized by inability of the lung to expand and fill thoracic cavity due to a restricting fibrous visceral pleural strip. It is usually asymptomatic but in some patients, it may be the cause of dyspnea. In symptomatic patients, the only available treatment is surgical decortication. Tuberculous pleural effusion occurs in approximately 5% of patients with Mycobacterium tuberculosis infection. The HIV pandemic has been associated with increasing incidence of extrapulmonary tuberculosis, which has resulted in increased recognition of TB pleural effusions even in developing nations. Interestingly our patient did not have any risk factors for developing extrapulmonary tuberculosis. Here we presented a case with an extensive unrevealing workup including negative ADA assay, but the fact that the patient had symptoms of lung entrapment led to more invasive procedures. Such procedures uncovered the etiology and treatment of his dypsnea. 71 Cardiac Metastasis of Malignant Melanoma: A Rare Cause of Right Bundle Branch Block Nicky Wadiwala, MD University of Southern California Background: Ocular melanoma is diagnosed in approximately 6 million people annually. Ocular melanoma metastasizes less frequently than cutaneous melanoma 75% vs. 96%, respectively. The liver is the most common location for metastases from ocular melanoma and the heart is thought to be another common site that is clinically underdiagnosed. Cardiac metastases are uncommon, with the exception of malignant melanoma. A large autopsy study showed cardiac metastases in 64% of patients dying from metastatic melanoma. However, cardiac metastases are rarely detected antemortem (2% of cases), despite sometimes massive involvement due to the lack of specific clinical manifestation. Case: A 45-year-old patient, with a history of right ocular choroidal melanoma treated with radiation 10 years ago, was admitted due to fatigue, mild dyspnea on exertion and dry cough. The electrocardiography showed a new right bundle branch block. Discussion: The clinician should be alert to the development of new cardiac conduction defects in a patient with prior melanoma history and should perform a detailed cardiac examination including echocardiography to exclude cardiac metastasis of the tumor. Early detection of cardiac melanoma is important for therapeutic and prognostic reasons. Churg-Strauss Syndrome: An Uncommon Cause of Adult Respiratory Distress Syndrome (ARDS) Tzu-Fei Wang, MD; Naveen El-Farra, MD University of California, Los Angeles Case: An 80-year-old man with history of asthma, coronary artery disease, remote history of polyarteritis nodosa (PAN) presented with progressive dyspnea, lethargy, cough, and fever for three weeks. He was intubated for respiratory failure, and presumptively treated for pneumonia and congestive heart failure. His condition did not improve. During routine laboratory studies he was found to have an eosinophilia with 23% eosinophils. Additional studies were sent and notable for a markedly elevated ESR, CRP, and IgE. His serum anti-neutrophil cytoplasmic antibody (ANCA) panel was negative. Given his history of asthma and pauci-immune glomerulonephritis, eosinophilia, and highly elevated IgE, he was diagnosed with Churg-Strauss syndrome. He was started on high dose steroids and daily oral cyclophosphamide and within two weeks had marked clinical improvement was extubated. Discussion: Churg-Strauss Syndrome is an uncommon disease with annual incidence of one to three per million. Common manifestations include asthma, peripheral eosinophilia, and vasculitis in multiple organ systems. Lungs are most commonly affected; however, sinuses, skin, heart, kidney, gastrointestinal tract, and peripheral nervous system can all be involved. The exact pathogenesis is unknown, but it is thought to be autoimmune in nature. The pathology of involved organs often reveals eosinophilic vasculitis and perivascular necrotizing granulomas. IgE is frequently elevated. About 30-50% of patients are ANCA positive, of which 70-80% is p-ANCA (antimyeloperoxidase antibody). Treatment of choice is high dose glucocorticoids. In patients with multi-system failure due to vasculitis, daily cyclophosphamide or azathioprine can be added. This case demonstrates the importance of broadening differential diagnoses when initial treatments yield suboptimal responses. A Case of Thrombotic Microangiopathy in Systemic Lupus Erythematosus Brandon B. Woodbury, MD; James Wilson, MD Olive View-ULA Medical Center A 25 year-old woman with systemic lupus erythematosus and a history of cerebritis and World Health Organization (WHO) Stage V nephritis presented with pleuritic chest pain, shortness of breath, dry cough, and fever. She had experienced intermittent swelling of her legs and face for several months prior to presentation. She was febrile and tachycardic, with an elevated blood pressure of 189/111. Pulmonary auscultation was clear except for bibasilar decreased sounds. A preexisting 3/6 systolic murmur throughout her precordium was noted. She had trace bilateral leg edema, but no jugular venous distension. Creatinine was elevated from her baseline of 1.2 to 1.9 mg/dL, and she had a leukocytosis of 14.6 cells/uL. Urinalysis showed specific gravity of 1.021, pH of 7, with 10 red blood cells/field and proteinuria>300 mg/dL. Serum tests also revealed C3/C4 of 52/10 mg/dL, anti-dsDNA >1:1280, positive lupus anticoagulant, ANA 1:320 (homogenous pattern), and an elevated ESR of 63 mm/h. Direct coombs, anti-cardiolipin, anti-Smith, anti-DNAse B, beta-2 glycoprotein, and RPR were negative. An HIV test was negative 3 months previously. Heparin drip was begun after a ventilation-perfusion scan showed intermediate probability of pulmonary embolus. Intravenous steroids were also started for suspected lupus exacerbation. Renal ultrasound showed bilateral increased cortical echogenicity and pelviectasis. Her renal function continued to decline, eventually requiring hemodialysis. A renal biopsy showed WHO stage IV (diffuse proliferative) and WHO stage V (membranous) lupus nephritis, as well as evidence of WHO thrombotic microangiopathy (TMA). Cyclophosphamide and plasmapheresis were started to treat her stage IV nephritis and thrombotic microangiopathy, respectively. Her platelet count decreased, and argatroban was substituted for heparin due to concern for heparin-induced thrombocytopenia (HIT assay was eventually negative). After several days of plasmapheresis, the patientвЂ™s renal function improved and her creatinine returned to baseline. She returned home and was doing well 72 at a follow-up visit several weeks later. TMA manifests itself in thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, malignant hypertension, systemic sclerosis, preeclampsia, and drug reactions. As the laboratory findings are similar in the disorders resulting in TMA, specific diagnosis is best determined by history. TMA is characterized by endothelial dysfunction and platelet aggregation with resulting thrombocytopenia and injury to erythrocytes. Examination of the platelet thrombi shows von Willebrand factor (VWF) without fibrin or fibrinogen. VWF normally exists as a spectrum of multimeresвЂ” the smaller ones are secreted while the larger (& more pro-thrombotic) multimeres are stored in the synthesizing cells. Normally these larger multimeres are broken down by the metalloproteinase ADAMTS-13. Dysfunction of ADAMTS-13, VWF, or the platelets themselves predispose to pathologic platelet aggregation and thus TMA. Therapy mainly involves replacing the larger-multimered, prothrombotic VWF with normal VWF through plasmapheresis. This case represents a rare occurrence of TMA in a patient with systemic lupus erythematosus. Acute Myocardial Infarct in Patient with Idiopathic Anasarca and Chronic Eosinophilic Leukemia Following Treatment with Imatinib Kevin Wu, MD; Casey OвЂ™Connell, MD University of Southern California Chronic eosinophilic leukemia is a rare myeloproliferative disorder that by WHO 2009 criteria has a clonal cytogenetic abnormality, most commonly FIP-PDGFR-О± mutation. It is treated with Imatinib, a tyrosine kinase inhibitor targeting this fusion protein. In this report I describe a previously unreported phenomenon. 62 y/o Hispanic male with a h/o of idiopathic anasarca presents with progressive dyspnea on exertion, orthopnea, chronic and left chest pressure worsens with inspiration, escalating over a 3 month period. Exam is significant for decreased breath sounds and anasarca. CXR shows complete opacification of his right hemithorax and left pleural effusion. Patient was ruled out for ACS. Labs: WBC 88k, (90% eosinophilia). Peripheral smear shows significant eosinophilia but otherwise normal. Secondary causes such as infectious, rheumatologic, and other secondary hematologic processes were ruled out. Marrow biopsy shows normocellular marrow, severe hypereosinophilia. FIP-PDGFR mutation analysis is (-) in the context of a (+) control thatвЂ™s (-). We diagnose the patient with chronic eosinophilic leukemia (CEL) and treat with Imatinib and hydroxyurea. Patient was followed in clinic 4 days post-discharge and his WBC decreased from 108K to 93k (99% eos). He complained of substernal chest pressure unchanged from initial admission. Four days later, he was admitted for worsened substernal chest pressure. He had new findings of ST depression in V3 вЂ“ V6, increased troponins (0.64,0.69,0.72) and mildly decreased EF with regurgitation which were all new findings. His WBC had further decreased to 42k on admission and ultimately 16k. Cardiology did not perform catheterization, attributing his disease process to CEL. He was managed with heparin & beta-blockers. He had asystole. CPR revived him. Troponin continued to increase, and he developed multiorgan failure. Care was withdrawn. Autopsy revealed pale yellow discoloration and hemorrhage in left ventricular posterior/ lateral wall, with moderate atherosclerosis, but no intraluminal thrombus or plaque. Microscopic evaluation showed neutrophilic infiltration but no eosinophils. The pathological diagnosis was severe acute MI as cause of death. It is unclear what roles idiopathic anasarca, the eosinophilia, or Imatinib treatment played. In the literature there are few case reports of acute development of CHF in CEL patients treated with Imatinib, as well as AMI in patients with untreated hypereosinophilia. There are no reports of Imatinib treatment being associated with AMI especially in the setting of a normalized WBC. This case may improve future decision-making. Complete A [V8] block: A Case of V8-Associated Hyperkalemia and Subsequent Complete Heart Block Kevin Wu, MD; Brandon Itagaki, MD; John Sim, MD Kaiser Permanente Southern California Case: A 71-year-old female with a history of aortic stenosis, hypertension, diabetes mellitus and hyperlipidemia presented to the emergency room with a three day history of generalized weakness, fatigue and dyspnea. On examination, she was noted to have a heart rate of 30 beats per minute with a blood pressure of 101/71. Her examination was significant for a bradycardic heart rate, with a 2/6 early systolic murmur. Lungs were clear, abdomen soft and extremities warm. Initial laboratories revealed potassium of 6.7 mmol/L, BUN of 18, and creatinine of 0.9. Outpatient medications include Metformin, Glipizide, Aldactone, Lisinopril, Atenolol, and Zocor. EKG revealed complete AV dissociation with peaked T waves. The patient was treated for hyperkalemia and started on isoproterenol for bradycardia. Additional history revealed that over the past 2 weeks, the patient was drinking more than four, 12 ounce cans of V8 per day because she saw advertisements promoting this supplement for seniors. The patientвЂ™s potassium level returned to normal and isoproterenol was weaned off. Her 3rd degree heart block resolved and she was discharged two days later. Discussion: This case illustrates the potential harm that can result from overuse of a nutritional supplement. We describe a case of hyperkalemia and subsequent third degree heart block that resulted from consumption of a high potassium concentration drink while on 2 medications that inhibited kaliuresis. The patient had also been on an additional medication that predisposes her to hyperkalemia from intracellular shifting. V8, which is a common readily available juice, has 670mg of potassium per 12 ounce serving. This amount of potassium is considered safe in moderation, but there is a clinical need to be aware of hyperkalemia in certain populations who are 73 at risk of developing it and its clinical consequences such as heart block in our case illustration. Two Distinct Clinical Entities Causing Severe Hypereosinophilia Stroke at 21: Catastrophic Antiphospholipid Syndrome Kevin Wu, MD; Casey OвЂ™Connell, MD University of Southern California Wei-I Vickie Wu, MD, MHS; Kuo-Chiang Lian, MD University of California, Los Angeles Hypereosinophilia is a nondescript clinical term that can be associated with many different diseases including infectious, allergic, rheumatologic and hematologic processes. Here I illustrate two patients with very different causes of eosinophilia. In the first case, a 58 y/o Hispanic female with no significant history presents with 6 month history of right upper quadrant abdominal pain, nausea and bloating. She denies other significant complaints on ROS. She does report frequent trips to Mexico and has a pet dog. Exam is significant for liver edge palpable below the R costal margin. Chest radiograph shows elevation of right hemidiaphragm. Initial labs are significant for WBC of 75k with 70% eosinophilia and mild elevations in AST, ALT and alkaline phosphatase. CT scan shows large 6 cm lesion with central necrosis in the right lobe of the liver. Stool O&P, strongyloides, visceral larval migrans, echinococcus and entamoeba histolytica serologies are all negative. Biopsy of the lesion shows necrotic material. Patient is empirically treated with ivermectin for two weeks, with improvement of eosinophilia. WBC increases after treatment completion. Patient is treated with a 6 week course of ivermectin, with complete normalization of leukocytosis and eosinophilia. There is complete liver regeneration at one year follow-up. In the second case, a 62 y/o Hispanic male with a history of idiopathic anasarca presents with progressive dyspnea on exertion, orthopnea, chronic and left chest pressure worsens with inspiration, escalating over a three month period. Exam is significant for decreased breath sounds on right and left base and anasarca of extremities. Chest radiograph shows complete opacification of his right hemithorax and left pleural effusion. Serial troponins are negative and echo shows normal systolic function. Basic labs are significant for WBC of 88k, with 90% eosinophilia. Effusion is exudative on analysis. Peripheral smear is significant for significant eosinophilia but otherwise normal. A workup to exclude secondary causes of hypereosinophilia is initiated. Bacterial, mycobacterial and fungal cultures of sputum and pleural fluid are negative. Cocciodiodes CF and histoplasmosis antigens are negative. Stool O&P, strongyloides and visceral larval migrans antibody are negative. Churg-Strauss and other rheumatologic causes are unlikely given the clinical history. Pleural fluid cytology shows no malignant cells. Flow cytometry of pleural fluid shows no evidence of atypical lymphocytes, CT shows no lymphadenopathy, and IgE/serum tryptase levels are normal indicating that lymphoma is unlikely to be the cause. Marrow biopsy shows normocellular marrow, severe hypereosinophilia with normal maturation. FIPPDGFR mutation analysis is negative in the context of a positive control thatвЂ™s negative. We diagnose the patient with chronic eosinophilic leukemia and treat with Imatinib and hydroxyurea leading to normalization of leukocytosis and eosinophilia within two weeks. With the Catastrophic antiphospholipid syndrome is a rare event associated with a high mortality rate. Suspicion should be high in young patients who present with unexplained sudden thrombotic events. With prompt recognition and intensive treatment, outcomes can be dramatically improved. Case: A previously healthy 21-year-old woman with a history of asthma and eczema was found to be acutely unresponsive with seizure-like activity. On further questioning, she had been experiencing headaches for the past six months but denied asociated fevers, chills, weight loss, sick contacts, or unusual travel. Upon arrival at the hospital she was hemiplegic on exam and initial head CT found a total right internal carotid artery occlusion and a right anterior cerebral artery and middle cerebral artery stroke with significant associated edema. She underwent a decompressive craniotomy due to the concern for potential herniation. Echocardiography revealed a large mitral valve vegetation consistent with Libman-Sacks endocarditis and she subsequently underwent a mitral valvuloplasty. Because her laboratories showed an elevated INR and thrombocytopenia, she underwent an aggressive hematology workup revealing a positive ANA, DRVVT, beta-2 glycoprotein, and anticardiolipin IgG to 790. Initial urinalysis was also significant for proteinuria and hematuria. The patient was diagnosed with antiphospholipid syndrome and initiated on anticoagulation given her procoagulable state. She subsequently developed severe thrombocytopenia and anemia without evidence of heparin-induced thrombocytopenia. She was found to have Coombs' positive autoimmune hemolytic anemia and was treated with steroids and intravenous immunoglobulin with resultant improvement in her thrombocytopenia and anemia. She was diagnosed with systemic lupus erythematous given a positive ANA, positive antiphospholipid antibodies, proteinuria, and thrombocytopenia. Discussion: Catastrophic antiphospholipid syndrome is a rare presentation of a small subset of patients with antiphospholipid syndrome. Currently proposed criteria include involvement of at least three organs, rapid development of sequalae (less than one week), histopathologic confirmation of small vessel occlusion, and positive antiphospholipid antibodies. Comprehensive therapy including anticoagulation, steroids, plasmapheresis, and intravenous immunoglobulin has been associated with improved outcomes. In patients who survive the initial catastrophic event, long-term anticoagulation is necessary to prevent further thromboembolic events. 74 two cases, IвЂ™m illustrating the complexities in the workup of eosinophilia. A Case of Elephantiasic Nodular Pretibial Myxedema E.G. Yan, MD; Ronald S. Swerdloff, MD Harbor-UCLA Medical Center Case: A 46-year-old woman with hypothyroidism after radioactive iodine ablation for Graves' disease twenty years ago presented with bilateral lower extremity edema. Since her therapy, the patient had been non-adherent to levothyroxine and subsequently developed dryness to her hands and lower extremities. On review of systems, she endorsed chronic fatigue, cold intolerance, constipation, slow speech but was remarkably thin. Of note, her mother also was hypothyroid after a similar radioablation therapy for Graves' disease in the 1950s. The examination was notable for hypothermia with a temperature of 95.8, pulse 78, and blood pressure 111/56. The patient was disheveled and malodorus. She had mild proptosis, and the thyroid was non-palpable. The lower extremities were grossly edematous with several large, tumorous growths around the circumferential distal limbs with overlying erythema, brawny induration, and areas of necrotic tissue. The skin on her hands were erythematous and dry with deep fissures and thinning of the hair. The laboratory results were significant for a white blood count 20.7, thyroid simulating hormone168, free T4 <0.4, and free T3 1.9. The patient was started on levothyroxine 100 mcg daily, and was diagnosed with elephantiasic nodular pretibial myxedema. Given the extensive swelling to her lower extremities, she underwent surgical debridement of the necrotic tissue and was instructed to wear compressive bandages to promote lymphatic flow. Pathological examination with colloidal iron did not demonstrate glycosaminoglycan, which would confirm the diagnosis of pretibial myxedema. Over the course of the next several months, the skin to her lower extremities eventually softened and the swelling decreased markedly. Discussion: Pretibial myxedema is an uncommon presentation of GravesвЂ™ disease, occurring approximately 5% of the time with the elephantiasic nodular type being rare. The cause of this dermopathy is unclear but is known to preferentially involve the pretibial aspects of the legs. Fibroblast-simulating factors, antibodies against fibroblasts, and production of glycosaminoglycans have been suggested. Additionally, proptosis is invariably seen with pretibial myxedema since the deposition of glycosaminoglycans behind the eyes leads to this condition. The diagnosis can be made based on clinical presentation with the associated findings of Graves' disease and confimed by the presence of glycosaminoglycans, but it is unclear why the patientвЂ™s tissue was stain-negative. Patients may have clinical hyperthyroidism while others are rendered hypothyroid before the onset of pretibial myxedema. In addition to the therapies mentioned previously, other modalities include routine topical corticosteroids under occlusion with plastic wrap or systemic immunotherapy. Upon review of the literature, to the best of our knowledge, there have been no prospective trials evaluating the efficacies of various therapies for this advanced stage of thyroid dermopathy. Management options have largely been based on anecdotal evidence and case series. A Case of Microscopic Polyangitis Refractory to Steroids and Immunosuppressants Wan-ting Yang, MD; Nader Kamangar, MD, FACP, FCCP Olive View-UCLA Medical Center Case: A 57 year old Filipino female with no past medical history presented with complaints of fever, night sweats, 20 pound weight loss, abdominal pain, and left lower leg numbness for 2 months. Her initial labs were notable for renal failure of unclear chronicity, normocytic anemia, and leukocytosis with bandemia. Her initial urine analysis showed trace protein, 1 RBC, and 1 RBC cast. A chest CT was significant for bilateral upper lobe centrilobular nodules and tree-in-bud opacities suspicious for an atypical infection. Her hospital course was complicated by acute respiratory distress requiring intubation. Fiberoptic bronchoscopy with bronchoalveolar lavage revealed sequentially bloody aliquots consistent with diffuse alveolar hemorrhage (DAH). Her renal failure had also progressed requiring hemodialysis. Her laboratory work up was significant for an elevated CRP, low total complement, negative ANA, negative GBM Ab, negative Proteinase3Ab, and a positive Myeloperoxidase (MPO-ANCA) value of >100. The infectious work up was negative with the exception of strongyloides stercoralis larvae found in her stools but not in the BAL, and the BAL findings were only significant for inflammatory cells on cytology. In light of these abnormal findings, she was started on the treatment for microscopic polyangitis (MPA) that included three days of pulse steroids with cyclophosphamide followed by maintenance steroids. She responded immediately to the steroids and was clinically stable until day 9 when she suddenly developed respiratory distress with recurrent hemoptysis leading to emergent intubation. She was again given the same therapy of pulse steroids and cyclophosphamide. Once again her clinical response was immediate, but two days later she redeveloped hemoptysis and respiratory distress requiring reintubation. A repeat bronchoscopy was done and reconfirmed the DAH. A decision was then made to initiate plasmapharesis in addition to the corticosteroids and cyclophosphamide. Discussion: ANCA-associated vasculitis is an important differential for patients with hemoptysis. WegenerвЂ™s granulomatosis (WG) and microscopic polyangitis (MPA) are medium- to small-size vasculitides, and patients classically present with diffuse alveolar hemorrhage and glomerulonephritis. The diagnosis is suggested by the presence of antineutrophil cytoplasmic antibodies (ANCA), which can be detected by immunofluorescence and ELISA. The indirect immunofluorescence assay reveals 2 types of staining patterns: cytoplasmic (c-ANCA) 75 and perinuclear (p-ANCA). The ELISA detects the antibodies against Proteinase 3 (PR3) and Myeloperoxidase (MPO), which are responsible for the cytoplasmic and perinuclear staining patterns respectively. This is an interesting case of MPA with a classic presentation and diagnostic findings but is unresponsive to conventional treatment. Plasmapharesis is not traditionally the first line therapy for MPA, but there has been reported cases showing its benefit when patients are not in remission on steroids and immunosuppressive therapy. Urgent dialysis was critical in the improved mental status by removing lactate and repleting bicarbonate. This case also illustrates several potentially life-saving techniques that can be applied when encountering severe metabolic acidosis. Oral Contrast Induced Acute Respiratory Distress Syndrome Joseph Yeh, MD; Seth Rivera, MD University of California, Los Angeles вЂњBasicвЂќ Treatment of Metabolic Acidosis Neda Yazdi, MD; Stanley Amundson, MD Scripps Mercy Hospital Case: A 67 year old female presented to the Emergency Department complaining of 1-day history of lethargy and visual changes specifically that surrounding appeared dark. Her medical history is pertinent for diabetes mellitus type 2, hypertension, and coronary artery disease status post CABG. Two days prior to admission she had a few episodes of nausea, vomitting, and diarrhea but continued taking her medications. Her medications included Metformin, Glipizide, Lisinopril, Metoprolol, Aspirin, HCTZ, and Lipitor, none of which were recently prescribed or adjusted. On examination, the patient had SBP 100/60 and tachypnea on nonrebreather mask. She was alert and oriented, speaking full sentences but speaking slowly. Exam was essentially unremarkable. Arterial blood gas was significant for pH 6.64, CO2 15, HCO3 <5, O2 saturation 97% on 32% O2. Basic metabolic panel was significant for Cr 7.3 with baseline Cr 1.6. The patient had a severe anion gap acidosis likely MALA. Beta hydroxybutyrate was 6.5, lactic acid 20 and positive serum osmolar gap greater than 30. Urinalysis showed specific gravity greater than 1.030. Nephrology was consulted for urgent dialysis and bicarbonate drip was started. Within 24 hours of admission, the patient was feeling nearly at her baseline state and her creatinine quickly improved to baseline function within 48 hours. The cause of the acute renal failure was likely secondary to prerenal etiology from viral gastroenteritis and continuation of blood pressure medications. Discussion: Metformin associated lactic acidosis (MALA) is a rare condition with an estimated prevalence of one to five cases per 100,000. Accumulation of metformin alone is rarely reported as cause as a cause of lactic acidosis, and tissue hypoxia is the usual cause. Acute lactic acidosis is a known complication of metformin. This case is unique both in severity of acidosis and that the patient was concious at presentation. The risks and benefits of metformin need to be carefully assessed prior to initiating the drug particularly when used in combination of Lisinopril. Secondly, the differential diagnosis of anion gap acidosis should be carefully reviewed and evaluate for less common etiologies when applicable. A large osmolar gap can be secondary to severe metabolic acidosis but ethylene glycol, methanol, and electrolytes need to be considered. Case: A 28 year-old woman with end-stage renal disease secondary to FSGS, status post three kidney transplants, and PUD with 2-weeks of nausea, vomiting and abdominal pain. As part of her work-up, she drank oral contrast for a CT abdomen and pelvis. She aspirated in her haste and, shortly thereafter, developed pruritic hives on her back which resolved with Benadryl 40mg IV and hydrocortisone 100mg IV. Her initial chest x-ray showed no interval change. Eleven hours later, she became obtunded and required intubation for hypoxemic and hypercapneic respiratory failure with PaO2/FiO2 53. Her lungs were coarse bilaterally and repeat chest x-ray showed diffuse bilateral opacities. She was transferred to the ICU, placed on broad spectrum antibiotics, and started on ARDSnet. All cultures returned negative. She was given Solumedrol 125mg IV bid for suspected delayed anaphylactoid reaction. Four days after admission the ICU, she was extubated. She was discharged home eight days later with steroid taper. Discussion: Anaphylactoid reactions are rapid and severe allergic reactions induced by the release of mast cell mediators such as histamine, leukotrienes and prostaglandins. Unlike traditional anaphylaxis, in anaphylactoid reactions, the offending substance directly causes the release of contents in basophils and mast cells. This process is not mediated by immunoglobulin E (IgE) and, consequently, does not require prior sensitization to an allergen. The most common manifestations are cutaneous (90%) and respiratory (70%). Our patient clearly described pruritis, utricaria, cough and dyspnea during the course of her reaction. Gastrointestinal (40%) and cardiovascular manifestations (35%) are less commonly recognized. Although the initial anaphylactoid reaction usually occur within minutes to hours of exposure, a recurrence of symptoms, defined as a biphasic reaction, can occur up to 72 hours after resolution of initial symptoms. Even though anaphylactoid reactions are a clinical diagnosis, there are blood tests including plasma histamine and total tryptase. Unfortunately, these tests have poor overall sensitivity (36%). Treatment requires immediate removal of the offending agent. In an acute decompensation, ABCвЂ™s should be addressed. Epinephrine 0.5mg IM has been shown to be effective in anaphylactic shock. Furthermore, rapid normal saline boluses, H1 and H2 antihistamines, oxygen and steroids can be used as appropriate. Home is Where the Heart is: Chest Pain and Syncope in a Patient with Agoraphobia 76 Andrew Young, MD; Arta Bakshandeh, MD; Radha Sarma, MD University of Southern California Introduction: Panic attacks are a discrete period of intense fear or discomfort, with a variety of symptoms including chest pain, dyspnea, palpitations, dizziness, sweating and chills. Recurrent attacks are associated with drastic behavioral changes & agoraphobia. We present a patient with a diagnosis of panic disorder with agoraphobia found to have an underlying cardiac condition. Case Presentation: A 46 year old Caucasian female with a history of intermittent syncope since childhood presented with three day history of dyspnea, chest pain and syncope. The patient had a history of chest pain and syncope beginning at the age of 13. These episodes, which occurred when she went outdoors or in public, were diagnosed as panic attacks. The panic attacks stopped in her twenties, when she developed agoraphobia and began spending most of her time at home. Since that time she has led a generally sedentary lifestyle. A year before admission the patient began having similar episodes again. On physical exam, she had a systolic murmur, loudest along the left sternal border and increasing with valsalva maneuvers. Work-up for syncope began with an electrocardiogram showing sinus bradycardia, negative cardiac enzymes and a nuclear stress test which was negative. Carotid ultrasound showed atherosclerotic disease without significant stenosis. An echocardiogram showed hypertrophy, an ejection fraction of 70% and a left ventricular outflow gradient of 19 mmHg that increased to 81 mmHg with valsalva. A diagnosis of obstructive Hypertrophic Cardiomyopathy (HCM) was made. An exercise treadmill test showed moderate functional capacity. The patient was treated with diltiazem, advised to restrict her physical activity, to stay well hydrated and to have her family screened for HCM. Discussion: Hypertrophic Cardiomyopathy (HCM) is a disease that is a well-described autosomal dominant genetic disorder in the cardiac sarcomere. It is associated with a spectrum of manifestations ranging from no symptoms to chest pain, syncope and sudden cardiac death (SCD). Although the overall annual incidence of SCD in patient with HCM is approximately 1 percent, there are features that increase a patientвЂ™s risk of SCD. These include prior cardiac arrest or arrhythmias, a family history of SCD, syncope (especially if exercise-related), outflow obstruction and an abnormal blood pressure response to exercise. Physical activity restriction, specifically avoiding high-intensity sports and activities, is recommended for patients with HCM. Patients at higher risk for SCD may be considered for an implantable defibrillator. Our patientвЂ™s panic attacks were likely manifestations of undiagnosed HCM, and her agoraphobia effectively restricted her activities, perhaps saving her from SCD. This case demonstrates not only how the diagnosing of HCM can be missed, but also how if left undiagnosed HCM can be at least debilitating and potentially life-threatening. Research Abstracts Patent Foramen Ovale Size as a Predictor of Stroke Size in Patients with Paradoxical Embolization Andre Akhondi, MD; Rubine Gevorgyan, MD; Leo Slavin, MD; Catherine Dao, MD; David S. Liebeskind, MD; Jonathan M. Tobis, MD David Geffen School of Medicine at UCLA Background: Patent foramen ovale (PFO) has been implicated in the pathogenesis of cryptogenic stroke via paradoxical embolization to the cerebral circulation. The criteria for PFO closure in stroke prevention have not been established. The purpose of this study was to evaluate the relationship between the morphological and functional size of the PFO and cerebral infarct volume identified on magnetic resonance imaging (MRI). Methods: Patients who were referred to the UCLA Interventional Cardiology Program with the diagnosis of cryptogenic stroke and who had both a Transesophageal echocardiogram (TEE), intracardiac echo (ICE) and a brain MRI at the time of stroke were selected. TEE or ICE were used to obtain PFO measurements, specifically PFO opening height, length, and the thickness of the septum secundum. The degree of right-to-left shunt was quantified by agitated saline injection. MRI of the brain with three sequences (T2, DWI, and FLAIR) was used to diagnose acute stroke and measure the infarct volume. Results: In the 62 patients studied, the average measured stroke volume was 8.2 cm3 on DWI, 7.9 cm3 on T2, and 8.2 cm3 on FLAIR. There was no significant correlation between the PFO height, length, septum secundum thickness, septal excursion distance, the presence of atrial septal aneurysm, or echo bubble grade compared with the infarct volume measured from the three MRI sequences (p = NS). Conclusions: This analysis revealed no significant association between PFO size or function by echo and brain infarct volume. The size of PFO by echo or right-to-left shunt on bubble study cannot be used to predict the potential size of subsequent strokes. Therefore PFO size should not be used to decide if a PFO should be closed. We conclude that the size of the thrombus is the major determinant of stroke size due to paradoxical embolism. The PFO size, presence of atrial septal aneurysm or function by echo does not appear to be the limiting factor for a thrombus to pass from the right atrium (RA) to left atrium (LA). Prevalence and Screening Methods for Depression in Chronic Heart Failure Patients Andre B. Akhondi, MD; Haneen Khamag, MD; Aurelia Macabasco-O'Connell, PhD, RN; Shahriar Pirouz, MD; Tracy Finegan, NP, RN; Mark McDon- 77 nell, MD, MBA; Robert Bryg, MD; Sheba Meymandi, MD, FACC Olive View-UCLA Medical Center Introduction: The association between chronic heart failure (CHF) and depression are well documented and associated with poor prognosis and poor quality of life. However, little is known about this relationship within the Hispanic population and non-Hispanic population. Therefore, the purpose of this study is to determine the prevalence of depression in patients with CHF. Methods: A total sample of 274 patients (46% Hispanic 54% non-Hispanic, 75% male, mean ageВ± SD was 52.6В±13, 60% had less than a high school education, 61% unemployed, 75% with non-ischemic cardiomyopathy, and 34% in New York Heart Association (NYHA) Class III-IV) were enrolled in a CHF Disease Management Program (DMP) at a Los Angeles County Facility. Each patient underwent a thorough initial screening evaluation, including sociodemographic information and clinical history, baseline ejection fraction, and NYHA functional class. All patients were asked two depression screening questions from the Patient Health Questionnaire-2 (PHQ2). If the patient responded yes to either, they were asked to complete the Patient Health Questionnaire-9 (PHQ9), which is a validated depression screening tool. Results: A total of 150 subjects (75%) reported having depressive symptoms: 25% with minimal, 26% mild, 16.5% moderate and 8.5% severe. Mean PHQ9 scores for the total sample was 9.85В±6.6. Hispanics revealed more severe depressive symptoms than non-Hispanics with mean PHQ9 scores of 11.08В±6 vs 8.74В±6, (p=0.007) with moderate and severe symptoms (61% vs. 39%, moderate; 69% vs. 31%, severe, p=0.04). More Hispanics had less than a high school education (<12 years) than nonHispanics (63% vs. 37%, respectively, p<0.001) while more non-Hispanics were unemployed (55% vs. 45%, respectively, p=0.008). There is a significant relationship between depression severity and initial NYHA Classification with those in NYHA class III and IV revealing higher levels of depressive symptoms [пЂ¬пЂ p<.001]. Conclusion: A high prevalence of depressive symptoms was found in both Hispanic and non-Hispanic patients with CHF. It appears that Hispanics tend to have more severe depressive symptoms when compared to nonHispanics. Better outcomes in health care, particularly in Hispanics, may result from incorporating depression screening and management protocols into the standard treatment of CHF. How Does Structured Data Entry Affect Measured Quality of Care: A Case Study in Patients with Falls & Mobility Disorders Shone Almeida, BS; Carol P. Roth, NP, MPH; David B. Reuben, MD; Neil S. Wenger, MD, MPH; David A. Ganz, MD, PhD University of California, Los Angeles Background: Structured data entry and electronic medical records are increasingly prevalent in the healthcare industry. However, an understanding of how structured data affects quality measurement and patient care is unknown. This study examines how structured visit notes (SVNs) are utilized and how they affect measured quality of care across 3 clinics in the Assessing Care of Vulnerable Elders: Practice Redesign for Improved Medical Care for Elders (ACOVEprime) study. Focusing specifically on patients age; 75 who screened positive for falls or fear of falling, we had 3 primary objectives: 1) Compare measured quality of care between patients with and without an SVN as determined from previously calculated ACOVEprime quality scores 2) Compare how thorough physicians were in completing different sections of the SVN, and 3) Evaluate the SVNвЂ™s potential for acting as a surrogate for full-chart reviews in analyzing quality of care. Methods: Across the 3 clinic sites (Salem, Canandaigua, and Scranton), we reviewed 226 primary care records of patients who had screened positive for falls or fear of falling. We checked for the presence of the SVN and, if present, we abstracted patient data from it. We calculated descriptive statistics regarding how often various sections of the SVN were utilized and developed algorithms for measuring quality of care for falls solely from the SVN. We compared our algorithms with previously calculated quality scores based on abstraction of the whole chart. Results: Of 226 patient charts, 41% contained the structured visit note. Among those charts containing the SVN, Salem, Canandaigua, and Scranton had quality scores of 87%, 89%, and 74%, respectively. Without an SVN, the quality scores were 30% and 45% for Salem and Canandaigua, respectively; similar results for Scranton cannot be computed since it was a fully-electronic site. The вЂњHistoryвЂќ and вЂњExamвЂќ sections of the SVN had much higher rates of utilization (80.9% and 70.9% respectively) than the вЂњAssessmentвЂќ and вЂњPlanвЂќ components (16.8% and 20.8% respectively). An average of 55% of the charts had identical quality scores using SVN-based and fullchart based quality measurement algorithms, while the SVN was shown to underestimate chart-based quality in 34% of patients and overestimate in 10%. Conclusion: Structured data entry may be a means of both prompting appropriate care as well as capturing quality data without relying on a full-chart review. Although this study is limited by lack of direct observation of how structured data entry affected physiciansвЂ™ workflow, we hope to conduct additional research along these lines in the future. Is There an Association Between Premature Ventricular Contractions and LV Dysfunction? Kandan Baban, MD; T. Haywood, MD Scripps Green Hospital 78 Introduction: Isolated premature ventricular contractions (PVCs) are commonly encountered in clinical practice. They are typically considered benign entities brought on by a multitude of factors. More recent studies have demonstrated that there may be a causal relationship between frequent PVCs and left ventricular dysfunction, even dilated cardiomyopathy. The hypothesis of PVC-induced cardiomyopathy is based on the observation that there is improvement in LV function after suppression of the ectopic focus with medication or radiofrequency ablations. We decided to evaluate our own population of patients at Cardiology division at Scripps Green Hospital for frequent PVCs and determine if there is any significant decrease in ejection fraction (EF). CrohnвЂ™s disease (CD) is not as well characterized. Colon cancer was first reported as a complication of вЂњcicatrizing enteritisвЂќ (regional enteritis) in 1948 and has been described in a number of reports. In fact, there is data to support that the risk of colorectal cancer is similar in CrohnвЂ™s colitis (CC) and UC of similar duration and severity, and significantly higher in CC than in the general population. While outcomes of screening and surveillance colonoscopy with biopsy have been widely reported in patients with UC, only one study exists, to the best of our knowledge, which evaluates these outcomes in CD. In this study, we report a high detection rate of dysplasia and cancer in chronic extensive CC. Herein, we report our colonoscopy and biopsy findings in CC. Method: We conducted a retrospective, Case-Controlled chart review and statistical analysis of the effect of frequent PVC (as defined by PVC >10,000) on left ventricular ejection fraction. 1024 holter monitors were evaluated for the presence or absence of more than 10,000 premature ventricular contractions in a 24 hour period from December 2007 through August 2008. Study population consisted of 50 patients (avg. age 70 +/- 0.6) with PVC > 10,000. 42% of patients had coronary artery disease. Controlled population consisted of 100 patients (average age of 68 +/- 0.6) with PVC < 5,000. Two controls were matched to each case, the matching factors being sex, presence or absence of CAD, and age (+/- two years). Transthoracic echocardiograms within 2 years of 24 hours holter monitoring were compared for EF in both study populations and controls. Once data was assembled we compared cases with 2 matched controls to evaluate for a significant difference in EF. Methods: This is a retrospective study that involves prospectively collected data. A CD patient database at a large inflammatory bowel disease (IBD) center was reviewed for colonoscopies and biopsies performed between January 1995 to July 2009 from patients with a long duration of disease. Those who had colonoscopies presumably had screening and surveillance for colonic CD, not colorectal cancer. During colonoscopy, random biopsies were typically taken at 10 cm intervals, as well as from strictures, masses, or polyploid lesions. Disease distal to the splenic flexure was defined as left-sided. Biopsy pathology was specified as normal, polyp (i.e. neoplasia without dysplasia), indefinite for dysplasia (ID), low-grade dysplasia (LGD), high-grade dysplasia (HGD), or carcinoma (CA). ID is included in our definition of dysplasia. Results: Patients with PVCвЂ™s > 10,000 had lower mean ejection fraction (57 +/- 14%) than patients with PVC < 5,000 (EF 64 +/-14%) with P value of 0.002. Conclusion: There is clear evidence from recent literature and our data that frequent PVCs place an individual at increased risk for left ventricular dysfunction and the development of significant cardiomyopathy. Patients with frequent PVC >10,000 were more likely to have abnormal EF. Our data support that frequent PVCs are associated with a reduced ejection fraction. Although the study is suggestive of causality there is no way to prove a clear relationship. Future goal of our study is a prospective follow up over time of the LV function in patient with >10000 PVCs, who have no current evidence of LV dysfunction. Colorectal Cancer in Patients with CrohnвЂ™s Colitis Robert J. Basseri, MD; Benjamin Basseri, MD; Konstantinos Papadakis, MD Department of Gastroenterology, Cedars-Sinai Medical Center Introduction: Chronic ulcerative colitis (UC) is an established risk factor for colonic neoplasms and carcinoma. This observation has resulted in the common practice of surveillance colonoscopies with biopsies for patients with UC. Contrastingly, the risk of dysplasia and carcinoma in Results: 411 patients were included in the study, with a total of 869 colonoscopies. The mean number of examinations per patient was 2.2 (range, 1-8), 19.5 months apart and 74 patients (18%) were found to have colorectal neoplasia. This shines light on a group previously not associated with higher incidence CRC. The study population was composed of 50% females (Table 1). Mean age at diagnosis was 26.7 years, with a mean disease duration of 19.4 years. In those with neoplasia, eleven percent had a first degree relative with inflammatory bowel disease and only twenty five percent presented with increased symptoms on colonoscopy. Age greater than 45 years correlated with a two fold incidence of colorectal cancer, while disease duration and earlier onset of CC were also risk factors for developing CRC. Conclusion: There is a significant incidence of colorectal cancer in patients with CrohnвЂ™s colitis, demonstrating that screening and surveillance in CrohnвЂ™s patient population could benefit risk reduction of colorectal cancer. MRSA Colonization Rates among Housestaff in Different Departments at a University County Hospital Lex Chen, MD; Arthur Jeng, MD Olive View- UCLA Medical Center 79 Background: Once primarily contracted in the hospital setting, methicillin-resistent Staphylococcus aureus (MRSA) has now become prevalent in the community setting, causing infections in otherwise healthy individuals. Because of this prevalence in the community and the greater exposure to patients with MRSA, concern exists that healthcare workers may themselves be colonized. In 41 studies that provided data on the nasal colonization with MRSA, 4.6% (1545 of 33318) of healthcare workers were found to be colonized with MRSA; however, no studies have targeted resident physicians. Current infection control guidelines mandate the screening of all inpatients and the cohorting and isolation of MRSAcolonized patients. Many physicians believe this to be a costly and unnecessary measure, because they feel health care providers are also likely to be colonized, but are not isolated per se. Also unknown is whether a difference in MRSA colonization exists among the various departments, as exposure and interaction with patients in the different medical specialties vary tremendously. Methods: This study was conducted at Olive ViewUCLA Medical Center, a county hospital of Los Angeles. Swabs from the anterior nares were collected from all housestaff rotating through the hospital during the study period, which included those from the departments of medicine, surgery, OB-gyn, emergency medicine, and pediatrics. All participants were asked to fill out a standardized survey regarding their perceptions about MRSA. Results: Eighty-two resident physicians (post graduate year 1-6, 34 males, 48 females), including 57 medicine, 10 surgery/OG-gyn, 10 emergency medicine, and 5 pediatric residents; three housestaff refused participation. Of 82 residents, 1 from the department of medicine was found to be positive for MRSA, giving a 1.2% housestaff colonization rate during the study period. No emergency room residents were found to be colonized, despite the lack of patient isolation/formal infection control procedures (gowning) in that department. Of the 82 housestaff physicians, 64 responded to the pretest survey. The majority of participants (46/64, 73%) believed that they were colonized with MRSA, and 34% believed that that 41-60% of their colleagues were also colonized with MRSA. Nine participants (9/64, 14%) answered correctly that 0-20% of their fellow residents were colonized with MRSA. Conclusion: This study revealed that despite a community MRSA prevalence of 50% in Staphylococcus aureus isolates, housestaff caring for such patients have very low rates of MRSA carriage in the nares, even among those in hospital areas where infection control is difficult (emergency department). No difference in MRSA colonization rates was seen among the various departments. These findings lead credence to the basis for the current patient isolation recommendations, despite the prevailing beliefs among housestaff. Is There a Role for Coronary Calcium Screening in Diabetes Patients Younger than 40 Years Old to Identify a Statin In-Need Group? Nikhil Daga, MD; Hussain Ismaeel, MD; Yasmin Hamirani, MD; Jigar Kadakia, MD; Philip Bach, MD; Matthew Budoff, MD Harbor-UCLA Medical Center Background: The ADA recommends statin use in diabetes patients > 40 years old with multiple cardiovascular risk factors. Coronary artery calcium (CAC) is a very sensitive surrogate of CAD and is a powerful and independent predictor of mortality and cardiac events. In this retrospective study we aimed to report the prevalence of CAC among those < 40 y.o. and to describe the determinants of CAC in diabetics. Methods: The charts of 2142 diabetic patients who had undergone CAC scoring at our center were reviewed. Their clinical and demographic data were collected. CAC score (CACS) was categorized into CAC 0= normal, 1-99 low, 100-399 intermediate and >=400 severe; and statistical analysis was performed. Results: The overall prevalence of CACS is 83%; in those < 40 y.o, prevalence of CACS 45%. Approximately 35% of diabetics had a severe CACS measurement (>400) - none < 40 years old. CACS median severity increased generally with age, with median CACS >100 seen in patients > 61-65 y.o, our mean and median age. Gender, male>female and hypertensive patients had statistically significant (SS) greater (p<0.001) CACS medians. Initially, ethnicity median CACS were SD (p<0.024) between Asians vs. Caucasians, Hispanics, and Others, but age was a confounding risk factor. SS differences in median CACS were not associated with family hx (p<0.177), and smoking (p<0.185). Odds ratios (OR) calculated for low, intermediate, and high CACS category values compared to age were 1.046 (p<0.001), 1.099 (p<0.001), and 1.119 (p<0.001), respectively; and compared to female gender were 0.525 (p<0.003), 0.328 (p<0.001), and 0.180 (p<0.001), respectively. Finally, OR for intermediate and high CACS compared to not having hypertension were 0.496 (p<0.006) and 0.477 (p<0.002), respectively. Conclusion: CACS were markedly higher among male diabetics and hypertensive diabetic patients independently. Age in diabetic patients only had a minor, but significant roll with regards to CAC. Oxidized High-Density Lipoprotein (HDL) from Patients with Coronary Artery Disease Can Regain its Anti-Inflammatory Properties Using HDL Mimetic Peptides Omeed Elboudwarej; Susan Hama, B.Sc; Greg Hough, MS; Mckenna Garife; Mohamad Navab, PhD University of California, Los Angeles 80 Introduction: Plasma levels of high-density lipoprotein (HDL) are known to inversely correlate with the incidence of coronary artery disease (CAD). The protective function of HDL is attributed to its role in reverse cholesterol transport from peripheral tissues for elimination, preventing lipid oxidation and inhibiting the formation of inflammatory molecules. Under inflammatory conditions, however, HDL can lose its beneficial properties and become pro-inflammatory. Methods: In the current study, we used plasma from 13 CAD patients and 10 healthy controls to determine if treatment with HDL mimetic peptides L-4F, apoJ[113122] or the tetrapeptide FREL would lower the HDL inflammatory index (HII). We used a bioassay in which normal low-density lipoprotein (LDL) from healthy controls was added to human aortic endothelial cultures to determine the extent of lipid oxidation in LDL and the resulting induction of monocyte chemotactic protein 1 (MCP-1). Results: In the absence of HDL, the cells oxidized the lipids in LDL, which in turn stimulated the cells to produce MCP-1. In the presence of normal HDL, the extent of LDL oxidation and thus MCP-1 production was reduced. HDL from CAD patients, however, had a high HII and actually amplified LDL oxidation. Treatment of both patient plasma and control plasma with mimetic peptides resulted in patient HDL preventing LDL oxidation and MCP-1 induction (i.e. resulted in HDL with low HII). Conclusion: HDL mimetic peptides can restore the antiinflammatory properties of HDL from patients with CAD, suggesting a potential therapeutic benefit using these mimetic peptides. Barrett's Esophagus Eradication by Radiofrequency and Cryoablation Avegail Gascon-Flores, MD; Sofiya Reicher; David S. Chung; Binh V. Pham; Viktor E. Eysselein Harbor-UCLA Medical Center Background and Aims: Radiofrequency ablation (RFA) with Halo devices (Barrx Inc) and cryoablation (CSA Medical) with liquid nitrogen/low pressure system are emerging as effective & safe techniques for Barrett's esophagus (BE) eradication. However, in our experience, RFA can be technically challenging in the distal esophagus, especially in the presence of moderate to large hiatal hernia. Here, we report interim analysis of a study aimed to evaluate the efficacy and safety of RFA with addition of cryoablation for nondysplastic and dysplastic BE. Methods: From 2005-2008, 41 patients with histologically confirmed BE (dysplastic and nondysplastic; short and long segment) were included in the study. All initially received RFA with follow-up endoscopy at 3 months. Patients with endoscopically visible BE on follow up underwent repeated RFA. Plate-based Halo-90 ablation device was used for small (<1 cm) BE islands; otherwise, balloon-based Halo-360 was used. If there was no endoscopically visible BE, 4-quadrant biopsies every 1 cm were performed. Cryoablation was performed if BE persisted after at least 2 RFA sessions. Cryoablation was done at 10-sec freeze/thaw cycles (4 cycles per each BE lesion). Results: 41 patients received at least one RFA session. 32 patients had no dysplasia, 3 patients were indeterminate, 5 low grade and 1 high grade. 6 patients were lost to follow up. Mean BE length was 4.4cm (1-14 cm). To date, 12 patients (1 high grade, 3 low grade, and 8 nondysplastic) achieved complete BE eradication endoscopically and histologically with all negative biopsies. The average number of RFA sessions in this group was 1.8. One patient with low grade dysplasia had small residual islands of BE in distal esophagus after 4 RFA sessions, which were successfully eradicated after one cryoablation. 7 more patients (1 low grade, 1 indeterminate and 5 nondysplastic) had small residual islands of BE after the average of 2.3 RFA sessions and thus received cryoablation. Follow-up endoscopy is pending at this time. 4 patients with residual BE or no change in BE after the average of 2.6 RFA sessions are planned for cryoablation. 12 patients await the second RFA. To date we have found no major complications when using dual ablative therapies. Conclusion: RFA and cryoablation are both promising new techniques for BE eradication. Our rates of BE eradication after RFA are similar to previous reports. Our study highlights the use of cryoablation as an adjunct tool to RFA, in particular in distal esophagus where contact with RFA devices can be hampered. Studying the Ferroportin Expression in the Eyes and the Brain Matthew Hakimi, BS; Tomas Ganz, MD, PhD University of California, Los Angeles Hepcidin is a small peptide hormone that is the principal regulator of iron absorption and distribution among tissues. Hepcidin binds to ferroportin (FPN), its receptor, on membranes of duodenal enterocytes, macrophages and hepatocytes. These cells normally export iron into plasma through ferroportin. Upon binding hepcidin, ferroportin is endocytosed and degraded, and iron export to plasma is diminished. Continued consumption of iron then decreases plasma iron levels. Our understanding of iron regulation is limited to the periphery and little is known about iron regulation inside the blood-brain-barrier. Several pathologies such as ParkinsonвЂ™s disease, FriedrichвЂ™s ataxia, age-related macular degeneration and possibly Restless Legs Syndrome are linked to iron homeostasis imbalance in this region. To determine how the hepcidinFPN system functions in the CNS, I am analyzing the expression of ferroportin on the different cells within the retina and brain in knock-in mice whose endogenous FPN has been tagged with green fluorescent protein (GFP). FPN-GFP may be directly observable in highly express- 81 ing tissues under fluorescence microscopy, or in lowerexpressing cells by immunostaining for GFP. Detecting protein expression in tissues requires fixing them in a solution of formaldehyde in order to immobilize proteins. Because this step can interfere with GFP detection by changing the proteinвЂ™s conformation, I assessed a variety of fixation techniques to detect their effects on GFP fluorescence. I grew HEK 293(Human Embryonic Kidney) cells that were expressing FPN-GFP on Poly-D-Lysine chamber slides and multi-well plates and induced them with ponasterone A. Fixatives at different concentrations (4% and 10% formalin, 4% paraformaldehyde) were used and fixations were stopped at different times (10 minutes up to 24 hours) and analyzed by epifluorescent microscopy and flow cytometry for GFP signals. These experiments suggest an optimal GFP signal at 10 minutes of fixation in 4% formalin, with a decline in GFP fluorescence intensity as fixation time is increased. Based on my experiments on wild-type mice, ferroportin-rich organs including the spleen and the duodenum can be harvested fresh and observed by immunofluorescence after 10 min fixation with 4% formalin and disaggregation. More delicate organs such as the eye will be cryosectioned. After the mice are killed, I remove the eyes, take out the cornea and lens under a dissecting microscope, immerse them in a fixative solution for 10 minutes, then cryoprotect them in sucrose before freezing them in the OCT freezing media at -80В°c overnight. Then, using a cryostat machine, I section the eyes at 7 micron thickness and stain with specific antibodies for markers of different cell types. I am using immunohistochemistry with anti-ferroportin antibodies as well as markers for the cell types which are suspected to express FPN at moderate levels, namely photoreceptor cells, MГјller and retinal pigment epithelium (RPE) cells. Yield of Colonoscopy in Patients with Metastatic Cancer of Unknown Primary Andrew Ho, MD; Brennan Spiegel, MD, MSHS Greater Los Angeles VA Healthcare System Introduction: Metastatic cancer of unknown primary(MCUP) is a diagnosis given to patients with evidence of cancer that has already spread in the body, but where the original or primary site of cancer has not been determined based on history , physical exam, laboratory testing and imaging studies. MCUP accounts for 2-10% of cancer diagnoses and since colon cancer is one of the most common cancers in the U.S. , gastroenterologists are often asked to perform a colonoscopy to evaluate if colon cancer is the primary site of metastasis. Despite this common practice, in searching through databases(Pubmed, Ovid, Cochrane), there have been no studies on the utility of colonoscopy for MCUP. Methods: Patients having a colonoscopy with indication of MCUP were eligible. Exclusion criteria included prior colon cancer, familial colon cancer syndromes, positive fecal occult blood test, iron deficiency anemia, or imaging study suggesting colonic abnormality. Colonoscopy reports from 2000-2009 for inpatients and outpatients at the West Los Angeles VA were reviewed retrospectively to identify eligible patients. This study was in conjunction with a concurrent study at LAC-USC Medical Center. Results: 15 patients were identified. Mean age was 59 years (range 40-74). 1 was female. 10 of the 15 were Caucasian. 8 of the patients died with the mean survival of 236 days(~8 months) after colonoscopy performed. None of the 15 patients were identified as having primary colon cancer. 5 of the 15 had the final diagnosis of MCUP despite a thorough workup including biopsies. 3of the 15 were found to have a lung primary. 2of the 15 were found to have a liver primary. 2 of the 15 were found to have a prostate primary. The remaining patients had as their primary cancer, peritoneal, bladder and pancreatic cancer. Conclusions: Patients with MCUP but no standard indications for diagnostic colonoscopy rarely have primary colon cancer. Patients with colonic abnormality on imaging were more likely to have colon cancer as their primary site and further studies are needed. We would not recommend routine colonoscopy for MCUP at this time. Comparison of Coronary Artery Bypass Graft Surgery with Drug-Eluting Stenting in Diabetic Patients with Multivessel Disease: Meta-Analysis of Clinical Studies Zahid Iqbal, BS; Michael Lee, MD David Geffen School of Medicine at UCLA Objectives: This meta-analysis was undertaken to assess the efficacy and safety of coronary artery bypass graft (CABG) surgery compared with drug-eluting stenting (DES) in diabetic patients with multivessel disease. Background: Previous studies that compared the merits of CABG with PCI suggested that CABG may be the preferred revascularization strategy in diabetic patients. Drug-eluting stents reduce the rate of target vessel revascularization compared with bare metal stents. The ideal revascularization strategy for diabetic patients with multivessel disease in the DES era is unknown. Methods: A literature search was performed to identify clinical studies comparing CABG with DES in diabetic patients with multivessel disease with a minimum followup of one year. Results: Five studies which included 1543 diabetic patients (CABG: n=757 and DES: n=786) met the selection criteria. The mean follow-up was 18 months (range from 12 to 36 months). Compared with DES, CABG is associated with a lower risk of major adverse cardiac events (odds ratio [OR] 0.48, 95% confidence interval [CI] 0.36 to 0.65), driven mainly by a lower risk of repeat revascularization (OR 0.18, 95% CI 0.11 to 0.30), despite having 82 a higher percentage of triple-vessel disease. There was no difference in death (OR 0.85, 95% CI 0.52 to 1.39) and myocardial infarction (OR 0.82, 95% CI 0.41 to 1.61). Patients in the CABG group had a higher risk of cerebrovascular events (OR 2.15, 95% CI 0.99 to 4.68). There was no significant heterogeneity of the treatment effect in clinical subgroups. Conclusions: In diabetic patients with multivessel disease, CABG is associated with a lower risk of major adverse cardiac events mainly driven by a lower risk of repeat revascularization despite having more extensive coronary disease. Mortality and myocardial infarction are similar after CABG and DES, but CABG is associated with a higher rate of cerebrovascular events. Cholecystectomy for Acute Cholecystitis at Night Does Not Result in Adverse Outcomes Brandon Ishaque, BA; Arezou Yaghoubian, MD; Christian DeVirgilio David Geffen School of Medicine at UCLA Background: In December 2008, the Institute of Medicine report on resident duty hours recommended further cuts in duty hours to avoid fatigue related errors. The purpose of our study was to determine whether the outcomes of laparoscopic cholecystectomy, the most frequently performed procedure by surgical residents, were affected by the time of operation. Methods: A retrospective review of all laparoscopic cholecystectomies at Harbor-UCLA Medical Center for acute cholecystitis by the Acute Care Surgery residents was performed between 1/1/2002-3/31/2009. Data collected include patient age, ethnicity, gender, length of hospital stay as well as need for intensive care, time of operation, need for intraoperative cholangiogram (IOC) and endoscopic retrograde cholangiopancreatography (ERCP). A comparison was performed of cholecystectomies performed in the first 16 hours of the typical resident on call day (6AM-10 PM) and the last 8 hours of a resident on call day (10 PM-6AM). Outcome measures were complications, bile duct injury, need to convert to an open operation, and mortality. Results: A total of 1337 patients with acute cholecystitis received laparoscopic cholecystectomy. 1019 patients received the operation between 6AM-10PM (Period 1), and 265 patients received the operation between 10PM6AM (Period 2). When comparing the two time periods, the median age was similar between the two groups (37 vs. 36) and there were a similar proportion of females (74.1% vs. 79.2%). The majority of patients were Hispanic (86.2% vs. 85.7%). The median length of hospital stay was also similar (5 vs. 4 days). With respect to the outcomes, there was a similar rate of complications for the two periods (2.6% vs. 3.4%). One percent of patients in the Period 1 had a bile duct injury vs. 0.4% in Period 2 (p=0.5). There was only one mortality in the total patient population. Conclusion: The findings of the study suggest that in the current duty hour era, laparoscopic cholecystectomy performed at night, by residents working beyond 16 hours does not affect outcomes. The new recommendations by the IOM to further decrease duty hours is unlikely to result in improvement in outcomes of this frequently performed operation. Progression Rates of Coronary Artery Calcification in Diabetic Subjects Compared to Matched Controls Sarkis Kiramijyan, MD; Naser Ahmadi, MD; Ferdinand Flores, MS; Khurram Nasir, MD; Matthew Budoff, MD Harbor-UCLA Medical Center Background: Coronary artery calcium (CAC) is a marker that is correlated with the presence of coronary artery disease (CAD). This study evaluates the rates of CAC progression between diabetics (DM) and matched controls. Methods: 296 asymptomatic DM and 300 matched controls (age 59В±6 years, 29% female) underwent baseline and follow-up electron beam computed tomography within a 2 year interval. The controls were matched to the DM with age, gender, CAD risk factors and baseline CAC. Measurements of baseline CAC, annual changes in CAC and О”CAC(%) ([annual changes in CAC/ baseline CAC] x 100) were assessed. Follow-up was completed 56В±11 months after the 2nd scan. Multivariable Cox proportional hazards models were developed to predict allcause mortality. Results: Annual change in CAC was 34.3В±4.8 in matched controls vs. 80.6В±10 in DM (p=0.0001). The О”CAC(%) was 10.2В±6.7 in matched controls vs. 29.4В±8.7 in DM (p=0.0001). Hazard ratios of death in matched controls vs. DM was 1.88 (95% CI 1.51-2.36, p=0.0001) for 10в‰¤О”CACв‰¤20%, 2.29 (95% CI 1.56-3.38, p=0.0001) for 20<О”CACв‰¤30%, and 6.95 (95% CI 2.23-11.53, p=0.0001) for О”CAC>30% compared to О”CAC<10% independent of age, gender and CAD risk factors. The event free survival decreased more in DM than in matched controls in proportion to the rate of CAC progression (Figure). Conclusion: CAC progression rate is higher in DM compared to matched controls and event free survival decreased more in DM than in matched controls in proportion to the rate of CAC progression. Severe Cases of Pandemic H1N1 2009 (Also Known as вЂњNovel Influenza A H1N1вЂќ or вЂњSwine FluвЂќ) Requiring Intensive Care: a Case Series from Olive View-UCLA Medical Center Julie Lam, MD; Tarik Ngab, MD; Richard Tennant, MD; Nidhi Nikhanj, MD; Anita Sircar, MD; Kamyar 83 Shahedi, MD; Glenn Mathisen, MD; Suzanne Donovan, MD; Nader Kamangar, MD Olive View-UCLA Medical Center Background: The World Health Organization raised the level of the influenza pandemic alert to phase 6 on June 11, 2009. The range of illness for this new strain of virus is still uncertain, varying in severity between different patients, countries, and other factors. The recommendations are constantly evolving as new information is obtained. Objective: To analyze the Intensive Care Unit cases of the pandemic H1N1 2009 virus at Olive View-UCLA Medical Center and report the similarities observed between these cases. Design: Case series. Setting: Olive View-UCLA Medical Center, a Los Angeles County Hospital in Sylmar, California. Patients: 8 patients with confirmed or high suspicion for H1N1 who required ICU admission and intubation. Results: All patients with severe illness at our hospital were men between ages 23 and 56. Obesity was the most common and often the only co-morbid condition. All patients presented with fevers, cough, and infiltrates on chest x-ray, usually bilateral. Most patients were also found to be hypoxic, tachycardic, and tachypneic in the emergency room during triage. Laboratory data was significant for leukopenia, elevated creatine kinase levels, and transaminitis in most of the patients, even if the patient did not complain of myalgias. Patients received oseltamivir at different times and experienced different outcome. Conclusions: Our patients were not in the extremes of age. They were young, obese men with no significant PMH, who presented with respiratory distress and rapid deterioration in the summer. They had bilateral pneumonias and increasing oxygen requirements. Some patients could not oxygenate well even with maximal mechanical ventilation. Doubling the dose of oseltamivir and administering it on admission may have improved the outcome of our later patients. We were made aware that a (-) rapid influenza diagnostic test does not exclude pandemic H1N1.These patients should still be treated with high dose oseltamivir. Although the recommendations for management of these critically ill patients are being modified, early recognition is crucial in preventing mortality and morbidity. We hope our report would help raise clinical suspicion, especially in patients who do not appear to have known risk factors for severe influenza. Combination Strategies Using Kinase Inhibitors in Colorectal Cancer In Vitro Jeremy Lorber, BA; Adrian Anghel, MD; Zev A. Wainberg, MD University of California, Los Angeles The RAS and PI3 kinase pathways play a critical role in cancer cell survival, motility, and angiogenesis. It was recently shown in in vitro and in vivo breast cancer cell lines that the growth inhibition caused by MEK (part of the RAS pathway) inhibitors is limited due to upregulation of the PI3k pathway. The aim of this research is to identify subsets of colorectal cell lines sensitive to a PI3k inhibitor alone and in combination with a MEK inhibitor. It is hypothesized that the combination of the inhibitors would provide synergistic inhibition. We exposed colorectal cancer cell lines to 12 different concentrations of MEK inhibitors, PI3k inhibitors, and a combination of the two inhibitors. The ability of inhibitors to inhibit proliferation and induce cell death was measured and IC50 values were calculated. Sensitivities of the 27 cell lines exposed to the PI3k inhibitor varied from IC50 of .010 to .669 ВµM. Additionally, there was no clustering of sensitivities based on mutational statuses of the PI3k or KRAS genes. When applied in combination, PI3k and MEK inhibitors caused a synergistic growth inhibition effect in all cell lines exposed to the combination treatment. These findings confirm the hypothesis of interaction between the PI3k and MEK pathways. Furthermore, it suggests study of additional colorectal cancer cell lines using combination treatment and exploring the mechanism of such synergy. Increased Duration of Red Blood Cell Storage Is Associated with Elevated Potassium Concentrations during Liver Transplantation in Adults Masood Memarzadeh, BA; Victor W. Xia, MD University of California, Los Angeles Background: Increased potassium (K+) concentration poses serious perils to patients undergoing orthotopic liver transplantation (OLT). Although there are several interventions that can be used to lower serum K+, prevention remains most effective in the management of intraoperative hyperkalemia. Previous studies have shown that increased duration of red blood cell (RBC) storage is associated with higher mortality and risk of complications in patients receiving RBC transfusions. Moreover, it is well known that stored RBCs undergo progressive structural and functional changes after 2 to 3 weeks that reduces their viability and functionality. We tested the hypothesis that increased duration of red blood cell (RBC) storage in transfused blood is associated with significantly higher K+ concentrations during OLT. Methods: After receiving IRB approval, we retrospectively studied 602 adult patients who underwent OLT at the UCLA medical center between January 2004 and April 2007. RBCs were categorized into two groups based on the mean storage duration (newer RBC в‰¤14 and older RBC >14 days). The relationship between serum K+ and duration of RBC storage was analyzed at multiple 84 time points of OLT using Student t test or chi-square analysis. The incidence of hyperkalemia in these groups was also recorded. Hyperkalemia was defined as a serum K+ в‰Ґ5.5 mmol/L. Recipient, donor and intraoperative variables of patients receiving newer and older blood were compared as well. Results: 258 patients received fresh RBCs (mean RBC age of 9.3 days) and 397 patients received older RBCs (mean RBC age of 21.7 days). Baseline demographics including age, gender, MELD scores, K+, and creatinine were similar in both groups. Patients in both groups received similar amounts of RBCs, insulin and furosemide. However, patients in the older blood group had significantly higher serum K+ at 1 hour pre-reperfusion and at 15 minutes, 1 hour, 2 hours and 3 hours post-reperfusion (Table 1, all p<0.02). Patients in this group also had a significantly higher incidence of hyperkalemia at 1 hour pre-reperfusion and at15 minutes, 2 hours and 3 hours post-reperfusion (all p<0.05). Discussion: Our results indicate that increased duration of RBC storage is associated with higher intraoperative serum K+ during OLT. This information can be used to better prepare anticipatory interventions in patients at increased risk for developing hyperkalemia during OLT. These results may also be applicable to other operations requiring massive blood transfusions, such as trauma surgery or ruptured aortic aneurysm repair. They may also warrant efforts to assign relatively newer blood to patients at particularly high risk of intraoperative hyperkalemia during OLT, such as those with renal failure or high preoperative serum K+. The Effects of Race on Do-Not-Resuscitate (DNR) Status in a Tertiary Military Medical Center LT Russell J. Miller, MD; LCDR Christopher Stafford, MD Depts. of Internal Medicine and Pulmonary/ Critical Care Naval Medical Center San Diego Introduction: Do Not Resuscitate (DNR) orders were developed to document patients' wishes regarding medical care in the event of cardiopulmonary arrest. Multiple factors influence end-of-life care discussions including understanding of disease process, medical staff and family influence, and cultural and religious beliefs. Studies have found large variations in rates of DNR orders across hospitals. When evaluating the effects of race in previous studies, African Americans were substantially less likely to choose DNR status than Caucasians. Speculation concerning this disparity is often based on socio-economic status and access to care. We performed a retrospective review of code status, demographic information, and outcomes in patients admitted to a military medical center. Since all patents are TRICARE beneficiariesвЂ™, we postulated our data would be more revealing than previous studies in assessing whether race plays a role in end-of- life wishes independent of factors affecting access to care. Methods: We performed a retrospective observational study of patients > 18 years of age admitted to Naval Medical Center San Diego between January 2005 and January 2009 through an electronic search. Information obtained included code status, ethnicity, length and cost of hospital stay. Results: During the study period 56,988 patients were admitted, of which 438 selected DNR status (0.76%). 1.05% (348/32968) of Caucasians chose to be DNR compared to only 0.37% (25/6757) African Americans and 0.5% (27/5408) of Asian/Pacific Islanders (p value < 0.01). Although Caucasians choose DNR status more often, African Americans and Asian/Pacific Islanders who were DNR had hospital stays significantly longer than their full-code counterparts. In Caucasians hospital stay was only extended by 1.4 days in DNR patients compared to 3.5 days in African Americans and 5.1 days in Asian/Pacific Islanders. Cost of hospitalization was also similar between races in the full code group, but in DNR patients the cost of hospitalization rose significantly more in African Americans and Asian/ Pacific Islanders compared to Caucasians. Conclusion: The higher cost and longer stays associated with DNR status in ethnic minority patients compared to Caucasians may indicate that non-white patients choose DNR in more advanced stages of disease. The reason for this disparity is unclear since issues of access to care are largely nullified in a military hospital setting. Potential explanations for the discrepancy include provider bias, socio-economic status, and cultural beliefs. We believe further research is warranted to understand the complex issues involved in patient choices regarding end-of-life care. A Highly Purified Omega-3 Fatty Acid Derivative Does Not Alter QTc Interval but Does Reduce QT Dispersion in Patients with Stable Coronary Artery Disease Tarik Ngab, MD; Sheba Meymandi, MD; Mahmoud Traina, MD; Mark McDonnell, MD, MD; Atef ElGassier, MBBch; Mohamed Labedi, MD; Robin Wachsner, MD Olive View-UCLA Medical Center Background: Fish oil has been shown to reduce sudden death in patients with coronary artery disease (CAD) potentially by decreasing fatal arrhythmias. Increased QTc and QT dispersion (QTd) have been found to be associated with increased risk of ventricular arrhythmias. Several epidemiologic trials have suggested increased fish intake may reduce the QTc interval, potentially reducing the risk of sudden death. However, no prior randomized trial has evaluated the effects of fish oil on QTc interval and QTd in this population. 85 Methods: In a randomized, double-blinded, placebocontrolled trial, 71 patients with established CAD and normal or near-normal ejection fraction received either 1 gram twice a day of a highly purified omega-3 fatty acid derivative, Lovaza, (n=37) or placebo (n=34) for 2 months. One patient in each group developed atrial fibrillation and were excluded from the analysis. At day 0 and day 60, electrocardiograms were obtained. Measurements of QTd were performed manually. Results: Fish oil supplementation did not result in any significant change in QTc (2.1ms in fish oil, -2.0ms in placebo, P=0.34). However, a significant reduction in QT dispersion was noted (6.4В±13.2ms in fish oil, 0.07В±9.8ms in placebo, P=0.047). Conclusions: Fish oil supplementation in patients with CAD did not result in any significant effect on QTc interval. However, there was a significant reduction in QTd. This effect may partly explain the mechanism of reduction of sudden death with fish oil supplementation. The Prevalence of Chagas Disease in a US Population of Latin American Immigrants with Conduction Abnormalities on EKG Tarik Ngab, MD; Sheba Meymandi, MD; Mahmoud Traina, MD; Salvador Hernandez, MBBch; Aiman Smer, MBBch; Haneen Khamag, MBBch; Eva Garcia, MBBch; Yacin Zwam, MBBch; Mahmoud Shebel, MBBch; Robin Wachsner, MD Olive View-UCLA Medical Center Chagas disease is the leading cause of cardiomyopathy in Latin America and is believed to infect 16-18 million people. Given recent immigration trends in the United States, there is a large population at risk. Early stage cardiac involvement usually presents with conduction abnormalities on electrocardiogram (EKG), including right bundle branch block (RBBB), left anterior and posterior fascicular blocks (LAFB/LPFB), and left bundle branch block (LBBB). Identification of disease at this stage may lead to early treatment and potentially delay the progression to endstage cardiomyopathy. This study aims to determine the prevalence of Chagas disease in a US population of Latin American immigrants with conduction abnormalities on EKG. All EKGs performed in a Los Angeles County hospital and clinic system were screened for presence of RBBB, LAFB, LPFB, or LBBB. Patients were contacted and enrolled in the trial if they resided in Latin America for at least 12 months and had no history of cardiac disease. 238 consecutive patients were screened for Chagas disease was performed with both an immunoflourescence assay (IFA) and enzyme-linked immunosorbent assay (ELISA). Testing was performed by the Center for Disease Control and Prevention (CDC). The mean age of patients was 47.6 years and had lived in the US for an average of 20.15 years. 32.7% of patients had isolated RBBB, 43.7% LAFB, 7.2% LBBB, 7.2% RBBB/LAFB, and 8.8% RBBB/LPFB. Countries of origin were as follows: Mexico 146, El Salvador 56, Guatemala 19, Nicaragua 4, Honduras 5, Argentina 4, Peru 2, Costa Rica 2, and Bolivia 1. Eleven patients were positive by both IFA and ELISA (4.6%). 6 patients were from El Salvador (10.7%), 3 from Mexico (2.1%), 1 from Honduras (20%), and 1 from Argentina (25%). 4 patients had RBBB (5.1%), 4 LAFB (3.8%), 2 RBBB/LAFB (11.8%), and 1 RBBB/LPFB (4.8%). No positive patients had LBBB. One patient was positive by IFA and negative by ELISA with LAFB. Our study demonstrates a significant prevalence of Chagas disease in Latin American immigrants with conduction abnormalities on EKG. Evidence of conduction abnormalities in Latin American immigrants without explanation should prompt consideration of Chagas disease as the etiology. Respiratory Muscle Weakness in Neuromuscular Disease: Association with Lung Function, Respiratory Impairment and Initiation of Non-Invasive Ventilation Nathan Nguyen, MD; Ahmet Baydur, MD, FACP University of Southern California Introduction: Patients with neuromuscular disorders exhibit a decrease in lung function and respiratory muscle strength. Symptoms such as orthopnea, daytime drowsiness, and the forced vital capacity (FVC) (50 % predicted) are used as guides to determine the need for assisted ventilation. The change in FVC from seated to supine position has been suggested as an indication for initiation of non-invasive mechanical ventilation (NIV). In individuals with normal respiratory function this difference has been shown to be 4%-6%, versus 16%-18% in patients with neuromuscular disease. Furthermore, a forced expiratory flow (FEF) less than 2.7 L/s predicts successful extubation or conversion from tracheostomy to NIV (Bach JR, Saporito LR. Chest 1996; 110:1566вЂ“ 1571). Objectives: In this retrospective study of patients with neuromuscular disease who developed respiratory impairment, we assessed (1) respiratory function in seated and supine postures, and (2) the relation between lung volumes and respiratory muscle strength as close to when NIV initiated. Methods: Spirometry in seated and supine posture and seated peak inspiratory and expiratory mouth pressures (Pimax and Pemax, respectively) measured in closest proximity to the initiation of NIV were assessed in 12 patients (6 males). The relation between FVC and FEF, between FVC and Pmax, and between postural change in FVC (FVCseated вЂ“ FVCsupine)/FVCseated [= %∆FVC], Pmax and respiratory muscle strength [RMS = (Pimax + Pemax)/2] were assessed. Results: Diagnoses included motor neuron disease (n= 5), autoimmune disease (n=3), post-polio syndrome (n= 3), and paralyzed left hemi-diaphragm (n=1). All patients 86 experienced orthopnea or dyspnea. Respiratory function was assessed within [median (range)] 3 (1-132) months of initiation of NIV. Anthropometric and physiologic values (mean В± SD) were as follows: Age 62.8 В± 9.91, BMI 30.3 В± 10.7, FVCseated 2.0 В± 1.0 L (61 В± 27 % predicted), FVCsupine 1.74 В± 0.99 L (56.3 В± 29.7 % pred), %∆FVC 17 В± 17%, FEFseated 5.1 В± 2.1 L/s (81 В± 35 % pred), FEFsup 4.5 В± 2.4 L/s (69 В± 39 %pred); Pimax 49 В± 24 cm H2O (55 В± 29 %pred), Pemax 67 В± 24 cm H2O (47 В± 29 %pred); RMS 58.6 В± 21.7 cmH2O (50.6 В± 23 %pred). Correlations (Spearman) between lung function and respiratory muscle strength were as follows: FVCseated (l) vs FEF (l/sec): r=0.59, p=0.04; FEF (l/sec) vs RMS (cmH20): r=0.69, p=0.02; %∆FVC vs Pi max (% pred) r=-0.52, p=0.08; %∆FVC vs FVCseated (% pred): r=0.52, p=0.08. Conclusion: These findings support FVC of 17 % is a useful marker of respiratory muscle weakness in patients with neuromuscular disease, and more useful than FEF and FVC as a guide to determine need for NIV, sometimes even long before respiratory failure ensues. Population Based Analysis of Prognostic Factors for Survival in Rectal Cancer Chuan-Ju Pan, MD; Kavitha Raj; Argyrios Ziogas, PhD; Steven D. Mills, MD; Andrew Barleben, MD; Michael J. Stamos, MD; Jason A. Zell, DO University of California, Irvine Background: Colorectal cancer is the second most common cause of cancer-related death among men and women in the United States. Several key prognostic factors have been extensively studied for colorectal cancer including TNM staging, numbers of lymph nodes removed, tumor grade, socioeconomic status (SES), race and treatment; however, prognostic factors specifically related to rectal cancer are not well-defined. This study focused on analysis of prognostic factors for survival in rectal cancer patients. We set out to determine these factors by using data from a large population-based California Cancer Registry (CCR). Methods: A retrospective analysis of mid/distal rectal cancers from 1994-2006 with a follow-up until March 2007 in CCR was conducted. Rectosigmoid cancers were excluded for the analysis. Multivariate colorectal cancerspecific survival analyses were performed using Cox proportional-hazard ratios (HR). Multivariate survival regression models were stratified by TNM stage and adjusted for the following relevant clinical factors: age, sex, race, SES, histology, tumor grade, number of lymph nodes removed, surgical procedure, radiation therapy and chemotherapy. Results: A total of 23,240 incident cases of stage I, II and III rectal cancer were identified. CRC-specific mortality benefit was observed for Stage II rectal cancer patients with 7 to 11 lymph nodes removed (HR, 0.68; 95% CI, 0.56-0.82) and with more than 12 lymph nodes removed (HR, 0.59; 95% CI, 0.48-0.72) compared with no lymph nodes removed; no mortality differences were noted for 1 to 3 or 4 to 6 lymph nodes removed vs no lymph node removed. In addition, African-Americans, and cases from lower SES had increased risk of CRC-specific mortality. Compared with well differentiated tumor after adjustment for relevant clinical factors and treatments, cases with poor (Grade III) and undifferentiated (Grade IV) tumor grade also had increased risk of CRC-specific mortality among stage I (Grade III HR= 2.01; 95% CI, 1.59-2.52; Grade IV HR= 4.15; 95% CI, 2.04-8.44), stage II (Grade III HR=1.83; 95% CI, 1.49-2.23; Grade IV HR= 2.66; 95% CI, 1.50-4.72) and Stage III (Grade III HR= 1.75; 95% CI, 1.43-2.14; Grade IV HR= 3.76; 95% CI, 2.385.93). Conclusions: Number of lymph nodes removed, AfricanAmerican race/ethnicity, SES, and tumor grade were observed to be independent prognostic factors for survival among rectal cancer patients. Examination of at least 7 lymph nodes was associated with decreased CRC-specific mortality. Interestingly, within each cancer stage tumor grade was noted to be the strongest prognostic factor of CRC-specific mortality. These data may be useful for evaluation of prognosis and for guiding rectal cancer treatment. Conditional Knockout of Nkx2.5 in Mesp1 Expressing Cells Results in Cardiac Developmental Abnormalities and Early Embryonic Lethality Reza Rafie, MD; Sina Tavakoli, MD; Ali Darahzereshki, MD; Sharmineh Shams, MD; Amir Hajrasouliha, MD; Roodabeh Mohamad zadeh, MD; Mohamad Pashmforoush, MD, PhD University of Southern California Background: Recent advances in cardiovascular genetics have improved our understanding of congenital heart disease. Developmental biology and molecular genetics studies have clarified the role of various gene products in cardiac development. Nkx2.5 and Mesp1 are two transcription factors that have been extensively studied in cardiovascular development. Mesp1, a member of basic helix-loop-helix transcription factor family located on chromosome 7, is being considered as the earliest transcription factor which can be detected in cardiac precursor cells. Nkx2.5 mutations have been associated in humans with disorders such as atrial septal defect, ventricular septal defect, Tetralogy of Fallot, and mitral valve abnormalities. In order to learn more about the effect of Nkx2.5 and Mesp1 in cardiac development, we conditionally knocked out Nkx2.5 in cardiac Mesp1 expressing cells by using a Cre-Lox p system. Methods: Using a Nkx2.5 floxed allele and Mesp1-Cre transgenic mice lines, we conditionally knocked out Nkx2.5 in the majority of right and left atrial and ventricular cardiomyocytes, with the exception of a small group of cells that are derived from non-Mesp1 expressing progenitors. We also crossed Mesp1-Cre and Nkx2.5- 87 Cre with ROSA26R mice lines to better identify the contribution of Mesp1 and Nkx2.5 expressing cells to different components of embryonic heart at different developmental stages. Results: Our results showed no live neonate with Msep1Cre+, Nkx2.5 genotype. As we evaluated embryos at different developmental stages, we noticed early lethality around embryonic day 10.5. Conditional Nkx2.5 knockout embryos had smaller body size and heart/body size ratio compared to wild type embryos. Conditional Nkx2.5 knockout embryos had pericardial effusion and did not start looping. According to previous studies, lineage analysis using ROSA26R line showed extensive expression of Mesp1 in both atria and ventricles and conditional knockout of Nkx2.5 in these cells was associated with severe cardiac malformations. Conclusion: Previous studies showed early embryonic lethality with global knockout of Nkx2.5 in cardiac cells. In this study, conditional knockout of Nkx2.5 in cardiac cells deriving from Mesp1 expressing progenitors caused severe cardiac malformation and early embryonic lethalithy. Cardiac malformation is one of the main causes of early pregnancy loss in human. Ectopic Expression of Pacemaker Channel Marker in Atrial Cardiomyocytes Adjacent to Vessels by Conditional Knockout of Nkx2.5 Reza Rafie, MD; Sina Tavakoli, MD; Sharmineh Shams, MD; Amir Hajrasouliha, MD; Roodabeh Mohamad zadeh, MD; Ali Darahzereshki, MD; Mohamad Pashmforoush, MD, PhD University of Southern California Introduction: Previous studies depicted the role of Nkx2.5 in cardiac conduction system development. In this study conditional knockout of Nkx2.5 in cardiomyocytes originating from Islet1 expressing progenitors resulted in ectopic expression of pacemaker channel (defined by HCN4) in limited areas of atria and ventricles. Islet1 and Nkx2.5 have extensive expression in atria and right ventricle but we found few ectopic sites in knockout mice. We attempted to figure out the underlying cause for this discrepancy. Methods: We evaluated 100 mice at neonatal and late embryonic stages with conditionally ablated Nkx2.5, using Islet1-IRES-Cre transgenic mouse line and the floxed allele of Nkx2.5. We studied the cardiac tissues by immunostaining using antibodies against the pacemaker channel marker (HCN4), actinin and connexin 40. We also performed hematoxyllin and eosin staining on these tissues. We crossed Islet1-IRES-Cre with ROSA 26R line to define the expression pattern of Islet1. Results: Conditional knockout of Nkx2.5 caused ectopic sites of HCN4 expression in atrial and small part of right ventricular myocytes. Lineage analysis using ROSA26R line showed most of the cells in atrial and right ventricular myocytes express Islet1 but ectopic expression of HCN4 was limited to few areas in atrium and right ventricle. These areas with pacemaker channel marker expression outside of sinoatrial node (SA node) has histologic characteristics similar to SA node with compact, round cells that is different from the rest of atrium. We performed immunostaining with HCN4 and connexin 40 on atrium that showed ectopic HCN4 sites in atrium do not express connexin 40 unlike the rest of atrium but similar to SA node. More careful evaluation of large ectopic HCN4 sites depicted the vessel section in middle or adjacent to that area. Ectopic HCN4 sites in ventricle were also located near to vascular sections. Discussion: Previous studies showed upregulation of HCN4 after Nkx2.5 knocking out at embryonic stage 8.59 post coital day. In this study we showed ectopic expression of HCN4 after conditionally knockout of Nkx2.5 in Islet1 expressing cells. Islet1 has diffuse expression in atrium and right ventricle. With conditional knockout of Nkx2.5, we expected to detect ectopic sites of HCN4 expression in most parts of atrium and right ventricle but there was only few ectopic sites in each knock mouse. With more evaluation, we noticed the vessel section in large HCN4 ectopic sites similar to artery that can be visualized in SA node. This needs more evaluation if different cytokine that are secreted from endothelium may have role in pacemaker cells formation. This finding can help to produce cells with intrinsic pacemaker activity in the future. Late-Onset Dilated Cardiomyopathy in Cap Knockout Mice Reza Rafie, MD; Sina Tavakoli, MD; Sharmineh Shams, MD; Amir Hajrasouliha, MD; Roodabeh Mohamad zadeh, MD; Ali Darahzereshki, MD; Mohamad Pashmforoush, MD, PhD University of Southern California Introduction: Insulin affects cardiac tissues by translocation of the glucose transporter 4 (GLUT4). Cbl-associated protein (Cap) is involved in the recruitment of GLUT4 to cell membrane through a phosphatidylinositol 3-kinaseindependent pathway. Deletion of the Cap gene protects against high-fat diet-induced insulin resistance. However, the functions of Cap gene have never been studied in cardiac tissue. Methods: Cardiac metabolism was evaluated by dynamic micro-positron emission tomography (microPET) imaging studies after intravenous injection of 200 ВµCi 18Ffluorodeoxyglucose in Cap knockout and wild type mice. Transthoracic echocardiography was also performed in young (2-3 months) and aged (1 year) mice. Pressure overload induced cardiac failure was induced by thoracic aortic constriction. Results: MicroPET scan studies demonstrated an early increased and a late decrease in 18F-FDG uptake in knockout mice. Basal echocardiographic findings as well 88 as response to thoracic aortic constriction at young age were comparable between wild type and knockout mice and no statistically significant changes were found. However, senescent knockout mice demonstrated echocardiographic evidence of dilated cardiomyopathy characterized by a decreased ejection fraction (wild type: 67.6% versus knockout: 56.8%; P<0.05) and an increased end systolic volume (wild type:`20.15 Вµ versus knockout: 37.39 Вµ; P=0.055). Conclusion: Previous studies showed the changes in cardiac metabolism during human and mouse development. There is tendency toward using glucose instead of fatty acids by cardiomyocytes at older ages. This study demonstrates the critical function of Cap gene in regulation of cardiac glucose metabolism. The indispensable role of CAP in maintenance of cardiac function may be one an underlying mechanism for development of idiopathic dilated cardiomyopathy in senescence. QRS Duration Variability as Predictor of Cardiac Resynchronization Therapy (CRT) Response Reza Rafie, MD; Pedram Razavi, MD; Michael Cao, MD University of Southern California Introduction: Cardiac resynchronization therapy (CRT) attempts to correct for ventricular dyssynchrony. Current indications for CRT therapy includes: NYHA class IIIIV, left ventricular ejection fraction < 35%, prolonged QRS duration( QRS duration>120 ms). Recent studies have shown that baseline QRS duration is not a predictor for CRT response. Studies have included a prolonged QRS duration >120-130ms. This is determined by a single 12 lead ECG prior to the CRT implant. Patient with underlying heart failure (HF) can have variable QRS duration. In this study we attempted to measure QRS duration variability before a CRT implant and to evaluate if this helps predict CRT response. Methods: Our study examined 45 patients that were treated with CRT in USC between 2005-2008 . Patients were selected for CRT based on the current indications: 1) NYHA classes II to IV that was unresponsive to maximum pharmacologic therapy. 2) Ejection fraction <35% 3)QRS duration > 120 ms on the last visit before CRT implantation. A total of 31 patients had at least 2 EKG available for interpretation prior to CRT implantation Results: CRT response was defined as either a functional improvement or increase in LVEF. A total of 82% of patients improved clinically and 55% had an increase in LVEF. The mean LVEF increased by 20% and the mean functional class improved by 1.1. Our results showed patients with clinical and/or echocardiographic response to CRT had longer QRS duration not only 1 month before starting CRT but also in period of 1 to 6 months before CRT implantation. (p=0.23). Our study was limited by the small study population. The results of Box plot depicts that a chronically prolonged QRS duration >1 month prior to implantation was associated with CRT response. Conclusion: Approximately 30%-40% of patients treated with CRT are considered non-responders for following reasons: scar tissue, echocardiographic parameter variability in to detect dssynchrony and positions of the LV lead. ECG is an inexpensive and simple method to evaluate cardiac dssynchrony. Previous studies have demonstrated weakness of QRS duration to predict response to CRT in spite of its current use as one of the criteria to select patients for CRT. Our study suggests that QRS duration variability can help to identifying patients who respond to CRT therapy. An Atherogenic Diet Reduces the Anti Inflammatory Capacity of High Density Lipoprotein and Increases the Susceptibility of Low Density Lipoprotein to Oxidation Shila Safarpoor, MD; Susan Hama, B.Sc.; Greg Hough, MS David Geffen School of Medicine at UCLA, Division of Cardiology Background: Our antioxidant and anti inflammatory defense mechanisms contribute to a balance between the stimulators and the inhibitors of inflammation. Beyond a point, however, these systems might be overwhelmed and eventually fail. High density lipoprotein (HDL) is one of the efficient anti oxidant and anti inflammatory systems in our organism. Under high oxidative and /or inflammatory pressure HDL can lose its ant inflammatory capacity and even become proinflammatory. Method: In the present study LDL receptor deficient mice (n=8 per group) were place on a Western type diet or on a rodent chow diet for 6 weeks. Plasma was fractionated by fast performance liquid chromatography and HDL (at 14 ug per ml) was added to cultures of human aortic endothelial cells that also received standard normal low density lipoprotein (LDL) (at 100 ug per ml). Result: In the absence of HDL the cells oxidize LDL and oxidized LDL induces the potent proinflammatory molecule monocyte chemotactic protein (MCP-1). In the presence of HDL from mice on the chow diet HDL prevented LDL oxidation and MCP-1 formation as compared with LDL alone without HDL (p=0.021). HDL obtained from the mice on the Western diet however was not anti inflammatory and did not prevent LDL oxidation and MCP-1 induction (p=0.53). Additionally, LDL from the mice that were on the Western diet was incubated with the cells (alone, and without HDL), LDL was oxidized to a higher degree as compared with LDL from the mice on the chow diet (p=0.017) and induced a significantly higher levels of MCP-1 (p=0.026). 89 Conclusion: We conclude that an atherogenic diet renders HDL proinflammatory and makes LDL more susceptible to oxidation by artery wall cells. Heritability of Bone Mineral Density: Regional and Gender Differences in Monozygotic Twins Kevin Y. Tse, MD; Brandon R. Macias, MS; R. Scott Meyer, MD; Alan R. Hargens, MD Scripps Mercy Hospital Introduction: Bone mineral density (BMD) is a measure of a personвЂ™s skeletal mineral content, and assessing BMD by dual x-ray absorptiometry (DEXA) can diagnose diseases of low bone density. In this study we determine the heritability of BMD in male and female monozygotic twin subjects using DEXA in thirteen specific anatomical regions, as well as various serum and urine markers of bone metabolism. Methods: In an attempt to quantify the genetic contribution of gender and skeletal region to BMD heritability, we scanned fourteen pairs of identical twins using DEXA and calculated the broad-sense heritability coefficient (H2) in each of the thirteen different body regions. We also collected serum and urine markers related to bone turnover to calculate H2. Results: The region of the body that was most heritable for both genders was the head (H2 > 95%). When males were compared to females, H2 values for male hip (H2 = 87%) and lower extremities (H2 = 90%) were higher than those in females (H2 = 49% and 56%, respectively). Conversely, H2 value for the female pelvis (H2 = 68%) was higher than that for males (H2 = 26%). Discussion: These data show that different regions of the skeleton exhibit different degrees of heritability, and that the variation depends on gender. Specifically, we show that among twins, females exhibit more variability in load-bearing regions, while males show more variability in non-load bearing regions. Does Medical Television Influence Physicians InTraining? Nicky Wadiwala, DO; Brian Haas, DO University of Southern California Objective: To determine if residents are influenced by medical television programs and to what extent that influence plays in their clinical decision-making. Design: Single page surveys were distributed to medical residents ranging from PGY1 through PGY7 at different specialties core conferences at University of Southern California / Los Angeles County Medical Center from January 2009 to March 2009. of those polled had seen at least 10 episodes of medical television (TV) with ScrubsВ®, ERВ®, House MDВ®, GreyвЂ™s AnatomyВ®, and Doogie Howser MDВ® being the top 5 shows respectively. Overall, 16% of residents had used a case from medical TV for discussion on rounds, 31% were able to take something they learned on medical TV and apply it into their medical training, 56% saw something on medical TV which led them to further research on the subject, and 13 % were influenced to the point that they ordered more tests and/or increased their differential diagnosis based upon a case that they had seen on television. Conclusions: Medical TV influences a large percentage of residents. Residents in their first year of training were more likely to be influenced by medical TV than those residents in later years of training. Either in the form of using a case for discussion during rounds, applying it to a case they are currently working on, or helping in the diagnosis of a case. There is an overwhelming stigma in the medical community about medical TV shows and their perceived inaccuracies, but our data demonstrate that although medical television programming may not be 100% accurate it encourages many physicians in-training to learn more about the disease they saw on medical TV, thus further contributing to their medical knowledge. What Test Characteristics Are Needed for New Tests to Evaluate Cardiovascular Prevention Strategies? Brandon B. Woodbury, MD; David J. Bryg, PhD; Robert J. Bryg, MD Olive View-UCLA Medical Center Purpose: As new therapies for primary and secondary prevention of cardiac disease become available (and as previously available therapies become better validated), estimation of future cardiac risk has become increasingly important to determine for whom the benefit of using these therapies outweighs their inherent risks and costs. To this end, new tests are continuing to be developed in an effort to improve risk prediction and thus optimize treatment strategies. Although these tests are often evaluated as to whether they independently predict risk, their true value lies in how well they improve stratification compared to or in combination with existing strategies. Method: We developed a Bayesian model utilizing the concept of the expected value of imperfect information to evaluate optimal testing strategies based on the a cost/benefit ratio. We then evaluated the use of a theoretical test in conjunction with cholesterol, highsensitivity C-reactive protein, and coronary calcium scoring over different age groups to determine the minimum diagnostic odds ratio an additional test would need to justify its use. Diagnostic odds ratios were varied from 1.5 to 5.0 and age was varied from 40 to 80. A ten year timeframe for events, similar to the Framingham risk score, was utilized for this analysis. Results: A total of 232 surveys were collected with an overall response rate of 84% (232/275). 91% (209/232) 90 Result: The expected value provided is a function of cost of the test, cost of treatment, and expected loss from non treatment. The diagnostic odds ratio required for the additional test to be included in the optimal stratification scheme varied by age. For a strategy involving lower-cost treatment, the odds ratios required for age groups 40, 50, and 60 were, respectively, 2.5, 2.0, and 2.5. At age 70 and higher, even the maximum evaluated odds ratio of 5 was insufficient for the additional test to be of utility. For a higher-cost treatment strategy, no testing was of use at age 40, and an odds ratio of 1.5 was sufficient for the 50, 60, and above 70 age groups. At high treatment cost, the primary purpose of the fourth test was to find patients unlikely to receive benefit of treatment. Conclusion: There was no significant difference in risks of death or of the composite end point of death, myocardial infarction, or stroke in patients who underwent CABG compared with PCI with DES for ULMCA disease. However, PCI with DES was associated with higher risk of target vessel revascularization. Conclusion: The above analysis demonstrates that not only must tests meet minimum criteria in order to be useful, but that these criteria can vary markedly with age and cost of the treatment. As new tests emerge, they should be evaluated in light of how much truly new information they add to current testing strategies. Can Drug-Eluting Stents Replace CABG in Unprotected Left Main Coronary Disease? A Meta-Analysis Tae Yang, MD; Michael S. Lee MD Olive View-UCLA Medical Center; David Geffen School of Medicine at UCLA Objective: We sought to compare the safety and efficacy of coronary artery bypass grafting (CABG) with percutaneous coronary intervention (PCI) with drug-eluting stents (DES) in patients with unprotected left main coronary artery (ULMCA) disease. Background: The current American College of Cardiology/American Heart Association guidelines recommend CABG for the treatment of patients with ULCMA disease based upon clinical trials that demonstrate a survival benefit with CABG as compared with medical therapy. Drug-eluting stents reduce the rate of target vessel revascularization compared with bare metal stents in ULMCA PCI and may be a safe alternative to CABG. Methods: A meta-analysis of clinical studies comparing CABG and PCI with DES for ULMCA disease with respect to death, the composite of death, myocardial infarction, or stroke, and target vessel revascularization at oneyear follow-up. Results: The analysis included 2905 patients from 8 clinical studies (2 randomized randomized trials and 6 non-randomized studies). At one-year follow-up, there was no significant difference between the CABG and DES groups in the risk of death (odds ratio [OR] 1.12, 95% confidence interval [CI], 0.80 to 1.56) or the composite endpoint of death, myocardial endpoint of death, myocardial infarction, or stroke (OR 1.25, 95% CI 0.86 to 1.82). The risk of target vessel revascularization was significantly lower in the CABG group compared with the PCI group (OR 0.44, 95% CI 0.32 to 0.59). 91