Basic Concepts п‚§ In USMLE step 2 CK, we get few different kind of questions like which of the following is the best initial test? Which of the following is the most accurate test? Which of the following is the best initial treatment? Which of the following is the most accurate treatment? Which of the following is the best next step in the management of this patient? Which of the following is the first step in the management of this patient? п‚§ To give answer to this kind of questions, understand following concepts. Test with good sensitivity is the best initial test. For example, EKG has good sensitivity to diagnose and differentiate chest pain so it will be the best initial test for patient with chest pain. Test with good specificity is the best accurate test. For example, barium esophagus is the best initial test to diagnose and differentiate dysphagia due to obstruction or motility disorder but esophageal manometry is the most accurate test to diagnose dysphagia due to motility disorder. Other important points in choosing test are cost effectiveness and safety of test. Same way in the treatment, look at the cost effectiveness and safety of the treatment. For example, atropine and pace maker, both are good to start heart in first degree heart block but we use pace maker as a last resort because atropine is more safe and as effective as pace maker and very easy to use (just give IV). The most difficult question is the next best step in the management of this patient. It depends on the condition of the patient when patient arrives at the hospital / clinic or patient leaving the hospital / clinic [Stable, Unstable or discharging time]. For example, patient with chest pain admitted to the hospital and treated with Aspirin, Clopidogrel, Morphine and Angioplasty, now its time to discharge, which of the following is the best next step in the management of this patient? (a) Stress test, (b) Metoprolol, (c) Captopril, (e) Furosemide. Answer is Metoprolol. Studies have shown that beta blockers have improved mortality rate in patient with recent MI so adding Metoprolol to patientвЂ™s regimen will be the best next step in the management of this patient. Another example, patient with upper GI bleed comes to ER, BP вЂ“ 90/60, which of the following is the best next step in the management of this patient? (a) Upper GI endoscopy, (b) IV normal saline, (c) fresh blood (e) Barium Esophagus. Answer is IV normal saline. First we need to stabilize the patient then we can order imaging studies. Fresh blood comes with its disadvantages like infectious diseases so not a good idea! I hope youвЂ™ve got a better idea by now about how to give an answer to the step 2 CK questions. I have written best initial test, most accurate test, best initial treatment, most accurate treatment, next best step and first step in the parenthesis in my notes so it will be easy for you to recognize and remember everything. These notes are for exam purpose only so I have written them in a way that they ask question on the real exam. In the real life, patient can present with many different ways and we deal them accordingly but always remember standard recommendation and current modification / new in the management. Good Luck S.S.Patel. All rights reserved. Registered with US copy right office 1 CARDIOLOGY п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Myocardial Ischemia / Myocardial Infarction: Substernal squeezing chest pain [pressure / heavy feeling on chest] Pericarditis: chest pain relieve by leaning forward Costochondritis: chest pain reproduce by palpation Dissecting aortic aneurism: tearing chest pain radiate to back Pneumonia: pleuritic (increase on inspiration) chest pain Pulmonary embolism: pleuritic chest pain, dyspnea, tachypnea Esophageal spam (вЂњnut cracker diseaseвЂќ): h/o GERD, gastritis, pain occur after eating, pain relieved by nitroglycerin or CCB, normal EKG п‚§ п‚§ п‚§ п‚§ Stable angina: chest pain after exertion Unstable angina: chest pain at rest [ST Depression] [D в†’ E] Myocardial Infarction: chest pain at rest [ST Elevation] Prinzmetal angina: chest pain at rest [ST elevation вЂ“ Transmural Ischemia] [due to coronary artery spasm. Pain may relieve by little exercise like patient gets up and walk & pain relieved (Physiology: because exercise causes increase in Adenosine which is a potent coronary vasodilator)] [Best diagnostic test вЂ“ Angiography вЂ“ shows No atherosclerosis] [Treatment: Ca++ channel blockers (CCB), Nitrates] [Not Aspirin and ОІ-blockers] п‚§ How will you differentiate between unstable / Prinzmetal angina and MI? Angina means chest pain comes and goes so even though chest pain occur at rest in unstable / Prinzmetal angina, you can easily diagnose from the presentation in the question. п‚§ п‚§ Best Initial test: EKG; Stress test for Stable angina Most accurate diagnostic test: Angiography (Ischemia) / Cardiac Troponin & CK-MB (Infarction) [Both begin to elevate in 4-6 hrs] [Cardiac Troponin remains elevated for 1-2 wks] [CK-MB remains elevated for 2-3 days] [best test to check re-infarction within a week вЂ“ CK-MB because it disappears in 2-3 days] Most accurate treatment for MI / Unstable angina: Angioplasty, Bypass п‚§ * п‚§ п‚§ п‚§ п‚§ S.S.Patel. Approach to pt present with Chest pain: If hx, initial EKG & cardiac biomarkers negative вЂ“ admit the pt and repeat EKG and biomarkers in 6-12 h If initial EKG & biomarkers are nl, but high suspicion from hx вЂ“ admit the pt and r/o CAD with stress test / angiography вЂ“ if high risk pt [> 65 yr, prior CAD / ACS] вЂ“ admit and evaluate for ischemia by stress test or angiography [useful in further Mx вЂ“ Angioplasty / Bypass] вЂ“ if low risk [<65 yrs, no prior CAD / ACS] вЂ“ can do out patient stress test within 72 h If second EKG & biomarkers are nl and low suspicion from hx вЂ“ ruled out MI so start looking for other causes of chest pain If initial EKG & biomarkers are positive вЂ“ admit & start treatment as per below All rights reserved. Registered with US copy right office 2 п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ * Patient with Stable/Unstable angina and MI should receive Aspirin, Nitrates and ОІ-blockers (if no contraindications like Asthma, low BP, Heart block etc.) Patient should also receive oxygen (if oxygen saturation is low) and morphine (if patient is still having chest pain; presence of pulmonary edema) Stable Angina: once you make diagnosis of stable angina from stress test, do an angiography followed by angioplasty; if 3 vessels disease or left main coronary artery disease on angiography, go for bypass [CABG] [CABG has shown reduce in mortality in pt with 3 vessel disease & left main coronary artery stenosis] Unstable Angina (due to vessel blockage by plaque so clot is forming): above 2 steps [Aspirin, Morphine...] + Clopidogrel + Heparin (to prevent further clotting) (not thrombolytics) + Statins [As discussed above, angiography should be done to guide further management like angioplasty / CABG] MI (due to vessel blockage from disrupted plaques so clot is already formed): above 2 steps [Aspirin, Morphine...] + Clopidogrel + Thrombolytics (to lyse clot and releive obstruction) (If certain criteria meets) / Angioplasty / CABG [As discussed above, angiography should be done to guide further management like angioplasty / CABG] + Statins + Low molecular weight Heparin (If not contraindicated) + ACE inhibitors (only If CHF due to acute MI) + Lidocaine (only If ventricular arrhythmias) Angioplasty is superior than thrombolytics for Unstable angina & MI but it is not readily available all the time [Door-balloon time should be <90 min for maximum benefit. If delays in angioplasty, give thrombolytics] Criteria to use thrombolytics (If it is not contraindicated, if Angioplasty is not readily available) [C/I: prior ICH, active internal bleeding, etc] п‚§ Within 12 hrs of the onset of MI. п‚§ > 1 mm ST segment elevation in two contiguous EKG. п‚§ New LBBB (Left Bundle Branch Block). Stress Test в†’ Indication: to dx CAD (stable angina), to evaluate & risk stratification in pt with known CAD, ACS, low ejection fraction in asymptomatic patient в†’ Contra indications (C/I): acute coronary syndrome (ACS) [Unstable angina & non-ST elevation MI], acute MI within 48 hours, symptomatic severe aortic stenosis, uncontrolled arrhythmia, aortic dissection, pulmonary embolism, and pericarditis в†’ Types: Exercise & Pharmacological в†’ Test Results: HR [must be achieving >85% of max predicted (220 вЂ“ age)], BP response, exercise tolerance, EKG changes [ST depression / elevation] & Imaging [with thallium201 or 99m Tc or Echocardiography] [abnormal results of imaging вЂ“ radionuclide defect with gamma camera or wall motion abnormalities on echocardiography] * Complication of MI : Ventricular Arrhythmia (MCC of Death) Л— Rupture (Ant Wall, Papillary muscles, Interventricular Septum) вЂ“ 3-7 days Л— Autoimmune Pericarditis (DresslerвЂ™s Syndrome) вЂ“ 6-8 weeks PostвЂ“MI вЂ“ Tx: NSAIDs - Sexual activity can be resumed after 2-4 weeks post-MI - Management of bradycardia following acute MI вЂ“ IV Atropine. If bradycardia persist for more than 24-hrs вЂ“ Transcutaneous pacemaker S.S.Patel. All rights reserved. Registered with US copy right office 3 - Sick sinus syndrome вЂ“ Alternate tachycardia and bradycardia in a patient who had MI вЂ“ Tx: Pacemaker Acute right ventricular infarction вЂ“ IV normal saline bolus (first step); then if it fails to improve hypotension, give ionotropic agents like dobutamine at 70% of target rate (after 5-7 days) вЂў Stress Test PostвЂ“MI п‚§ EKG changes in Acute MI: Peak tall T-wave в†’ ST elevation в†’ T-wave inversion в†’ Q-wave Inferior wall MI [RC]: II, III, aVF Anterior wall MI [LAD]: V2 вЂ“ V4 Anteroseptal [LAD]: V1 вЂ“ V3 Lateral wall MI [LAD / Circumflex]: I, aVL, V4 вЂ“ V6 Posterior wall MI [Posterior Descending]: V1 вЂ“ V2 п‚§ п‚§ п‚§ п‚§ п‚§ at 80% of target rate (after 2-3 weeks) LIPID MANAGEMENT в†“ (<100 mg /dl) < goal в†“ Coronary artery disease or Diabetes, Hypertension, Tobacco в†‘ One пЈ¦ в†“ LDL > 160 mg/dl вЂў Diet п‚§ п‚§ вЂў вЂў вЂў вЂў вЂў вЂў вЂў S.S.Patel. > (< 70 mg / dl) в†“ Coronary artery disease + Diabetes, HTN, Tobacco Risk Factors пЈ¦ в†“ в†“ LDL > 190mg/dl вЂў Statins в†“ LDL > 130 mg/dl вЂў Diet в†‘ More than One пЈ¦ в†“ LDL > 160 mg/dl вЂў Statins Statins are best initial drugs for management of high LDL. Generalized aches & pain with extremely high CPK in patient taking Statins, next step? пѓ stop Statins Two main factors that reduce risk of CAD вЂ“ reduce LDL & HTN (in DM patient) Discharge Check List: Risk stratification: stress test / echocardiogram (to assess EF) lower cholesterol [as above] lower HTN [<140/90 mmHg and <130/80 mmHg if DM] Smoking cessation, Exercise & Wt loss ASA (life long), Clopidogrel (9-12 months), Nitrates (prn), ОІ-blockers, ACEI (if CHF, HTN, DM) All rights reserved. Registered with US copy right office 4 п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ S.S.Patel. Different Clinical Scenario Most important is to know when you will do a diagnostic test and when you will start the treatment for вЂњnext best step in MxвЂќ question. For other questions answer what they've asked [ like best initial choice / most accurate choice] If patient comes to the ER with chest pain for last 1-hr and still having pain, best initial test? вЂ“ EKG If patient comes to the ER with chest pain for last 1-hr and still having pain, EKG shows ST depression / ST elevation, what will you do now? вЂ“ Start initial treatment [Aspirin, Nitrates, ОІ-blockers, Oxygen, Morphine] + pending confirmatory tests If patient comes to the office with c/o chest pain off & on but no pain at present, what will you do first? вЂ“ Stress test (EKG may not show anything cause no pain now so r/o CAD by stress testing) If patient comes to the office with c/o chest pain off & on but no pain at present, on examination you find 4/6 grade systolic murmur / known case of AS, what will you do first? - Echocardiogram [Stress test is C/I in symptomatic AS] Patient with past medical h/o CAD & other comorbidities (which restrict from exercise) present with symptoms suggestive of ischemia (i.e. c/o chest discomfort or feeling heavy in chest for few mins and goes away, etc), next step? вЂ“ Pharmacological Stress Testing [eg. Adenosine myocardial perfusing scan] (to see the progression of ischemia and risk stratification of CAD) [Adenosine and Dipyridamole scan are C/I in patient with COPD / Asthma. Use Dobutamine in those patient][Dobutamine may precipitate tachyarrhythmias] If above scenario but patient is able to exercise, next step? вЂ“ exercise stress test Different scenario for Stress Testing: Patient present with typical / atypical intermittent chest pain (exercise stress test); Patient present with typical / atypical intermittent chest pain with comorbidities which restrict patient from doing exercise (Adenosine scan); Patient present with typical / atypical intermittent chest pain with comorbidities which restrict patient from doing exercise and has Asthma / COPD (Dobutamine scan) Patient with previous Unstable angina / MI / bypass present with typical intermittent chest pain, EKG show changes (depression / elevation) or Stress test show ST depression / elevation, pt is on initial treatment for chest pain, next step? Angiography (useful in further Mx вЂ“ Angioplasty / Bypass) Any male patient present with chest pain (usually 2-3 hrs after dinner to confuse us on the exam), next step? вЂ“ EKG. If EKG is done & is normal and patientвЂ™s pain relieved by sub-lingual nitroglycerine, next step? вЂ“ admit the patient and serial monitoring of cardiac enzymes (always rule out cardiac problem first) Any male present with chest pain which didnвЂ™t relieve by nitroglycerine & EKG is non-specific, next step? вЂ“ admit the patient and serial monitoring of cardiac enzymes (always try to rule out cardiac cause first) Cocaine induced transmural ischemia (ST elevation on EKG), next step? вЂ“ initial management of ischemia (Oxygen, Aspirin, Nitroglycerin, Morphine) and Benzodiazepines; If patient continues to have pain after administration of benzodiazepines, next step? вЂ“ Give Phentolamine [alpha1-blocker] [cocaine causes increase in Norepinephrine which causes HTN by prominent alpha1 action] вЂ“ If still c/o pain, next step? вЂ“ Angiography All rights reserved. Registered with US copy right office 5 Congestive Heart Failure пЈ¦ вЂў вЂў вЂў вЂў в†“ Right Sided JVD Hepatomegaly Ascites Pedal edema в†“ Left Sided вЂў Pulmonary Edema вЂў Paroxysmal nocturnal dyspnea (PND) вЂў Orthopnea вЂў S3 gallop & murmurs вЂў Positive Hepato jugular reflex is seen in right side heart failure is helpful in differentiate cause of lower extremity edema from Heart problem / Liver problem вЂў - Diagnosis : History & Physical Examination CXR , Echocardiography MUGA scan / radionuclide ventriculography for measurement of ejection fraction (most accurate test) Treatment : Acute CHF (c/o short of breath, etc) вЂ“ 1st step вЂ“ give Oxygen вЂ“ Next step вЂ“ Diuretics (reduce pre-load) [Nitrates, Morphine are helpful too] вЂ“ If still no improvement вЂ“ give Dobutamine (ionotropic agent) [Digoxin takes time; Dopamine increase after-load by vasoconstriction] After stabilization вЂ“ ACE inhibitor (reduce after-load) / Angiotensin II receptor antagonist (those who donвЂ™t tolerate ACE inhibitors), ОІ -blockers (Studies have shown decrease in mortality by beta blockers in patient with CHF) Chronic CHF patients should be on Diuretics, ACE inhibitors, ОІ -blockers, Digoxin Spironolactone have been shown to reduce mortality when added with all of the above chronic CHF drugs вЂў - - Asymptomatic patient with low ejection fraction (means heart is failing), next step? вЂ“ find out etiology (IHD most common cause so order a stress test) - Management of acute pulmonary edema due to any cause вЂ“ Oxygen, Morphine, Loop Diuretics. If it is due to hypertensive crisis вЂ“ Give Nitroglycerin or Nitroprusside. If it is due to acute heart failure вЂ“ Dobutamine. Next step after stabilization of patient with acute pulmonary edema of unknown etiology вЂ“ echocardiography (to rule out MS or AI) вЂў Pt on multiple drugs for multiple problems including digoxin, furosemide, next step? пѓ serum electrolyte measurement (furosemide cause hypokalemia which may cause digoxin toxicity) в– Mitral valve Prolapse: Mid-late Systolic Click - Presentation в†’ Asymptomatic, Palpitation (most common presentation), Chest pain, Syncope, Sudden death S.S.Patel. All rights reserved. Registered with US copy right office 6 * - Marfan Syndrome / Asymptomatic Most common cause of mitral regurgitation in U.S. Treatment в†’ No specific treatment in most cases Endocarditis Prophylaxis в†’ Only If murmur present ОІ - blockers в†’ if chest pain occurs, palpitation and autonomic symptoms AntiвЂ“arrhythmic в†’ if arrhythmia occurs в– Infective Endocarditis: Splinter hemorrhages , RothвЂ™s spot in eye , Janeway lesions, Valve regurgitation - Pathophysiology of RothвЂ™s spot & OslerвЂ™s node вЂ“ immune vasculitis; JanwayвЂ™s lesion вЂ“ septic emboli - Strep. Viridians в†’ most common overall cause (previously damage valve ) - Staph. Aureus в†’ IV drug abuse ( Normal / Previously damage valve ) Tricuspid Valve - Staph. Epidermidis в†’ Prosthetic devices - Strep. bovis в†’ ulcerative colitis / colorectal cancer patient - Conditions that do not require infective Endocarditis Prophylaxis вЂў pt. with cardiac pacemaker and defibrillation вЂў Mitral valve prolapsed without regurgitation (without murmur) вЂў Surgically repaired ASD, VSD, PDA вЂў h/o Rheumatic fever without valvular dysfunction вЂў h/o Kawasaki disease without valvular dysfunction вЂў h/o isolated bypass surgery вЂў h/o isolated ostium secondum ASD Л— Prophylaxis of Infective Endocarditis in patient allergic to Penicillin: Dental, oral, respiratory, esophageal procedure пѓ Clindamycin, Azithromycin GI & genitourinary пѓ High risk пѓ Vancomycin + Gentamycin Low risk пѓ Vancomycin Л— First step вЂ“ start empiric antibiotics after drawing blood for culture Next step вЂ“ Transesophageal echocardiography to see vegetation Л— Empiric antibiotic for IE in IV drug abuser вЂ“ Vancomycin + Gentamycin Л— In general вЂ“ Nafcillin + Gentamycin - S.S.Patel. Loeffler Endocarditis вЂ“ restricive cardiomyopathy вЂ“ prominent Eosinophil infiltrates leading to the fibrotic thickening of portions of the heart All rights reserved. Registered with US copy right office 7 в– Valvular Heart Disease : вЂў вЂў Stenosis в†’ Problem in opening of valve therefore murmur occurs during opening of the valve Regurgitation в†’ Problem in closing of valve therefore murmur occurs during closing of the valve Mitral Stenosis вЂў Diastolic murmur вЂў Opening snap Aortic Regurgitation вЂў Diastolic decrescendo вЂў Austin Flint murmur Mitral Regurgitation вЂў Holosystolic radiate to the Axilla Aortic Stenosis вЂў Systolic ejection radiate to carotid вЂў CXR в†’ double density Right heart Border. вЂўEchocardiography вЂўEchocardiography вЂўEchocardiography вЂўEchocardiography Treatment вЂў Diuretics (best initial) вЂў Balloon valvuloplasty (if diuretics donвЂ™t help) вЂў Valve replacement (If both of above fail to relieve symptoms) Treatment вЂў Endocarditis Prophylaxis вЂў Diuretic, ACE inhibitors Treatment вЂў Digitalis вЂў Valve replacement ( Presence of Austin Flint murmur indicates the need for replacement of the valve ) вЂў Anticoagulant вЂў Digitalis(if AF) вЂў Anticoagulant (if AF) вЂў Diuretics , ACE inhibitors вЂў Valve replacement Treatment вЂў Endocarditis Prophylaxis вЂў Surgery if valve area < 0.8 cm2 ( Normal 2.5-3 cm2 ) вЂў Balloon valvuloplasty вЂў Indication for surgery вЂ“ chest pain / Syncope during exercise in elders; Stable angina due to stenosis next step? Echocardiography [not stress test] вЂў Austin Flint murmur: regurgitant stream from incompetent Aortic valve hits anterior mitral valve leaflet producing a diastolic murmur. вЂў Right sided murmur increase in intensity with Inspiration S.S.Patel. All rights reserved. Registered with US copy right office 8 в€— Cardiomyopathies в€— Dilated вЂў Echocardiography вЂў CHF following URTI; Anemia, B1 deficiency, etc вЂў Treatment - Diuretics - Vasodilators - Digoxin вЂў If Arrhythmia в†“ Procainamide / Quinidine вЂў Anticoagulants вЂў вЂў вЂў S.S.Patel. Hypertrophic Restrictive вЂў Echocardiography вЂў Autosomal Dominant chromosome 14 вЂў thickening of Interventricular septum вЂў Mechanism of mitral regurgitation вЂ“ systolic anterior motion of mitral leaflet вЂў ejection fraction 80-90 % ( Normal 60% В± 5% ) вЂў Sudden Death in athletes Treatment вЂў ОІ- blockers вЂў Ca+ 2 channel blockers вЂў Disopyramide вЂў Echocardiography вЂў Catheterization: square root sign on tracing ventricular pressure вЂў Mimic Constrictive Pericarditis (see below) вЂў Amyloidosis, вЂў Hemochromatosis вЂў Sarcoidosis вЂў Loeffler's syndrome вЂў Scleroderma вЂў EKG вЂ“ low voltage вЂў No good treatment вЂў Consider Heart transplant вЂў вЂњspeckled patternвЂќ on echocardiography пѓ cardiac amyloidosis Valsalva / Standing: в†“ Preloadв†’в†‘ Obstruction Squatting / Hand grip: в†‘ Preload в†’ в†“ Obstruction Increase in Pre-load вЂ“ decrease murmur in Mitral valve prolapse and Hypertrophic cardiomyopathy. All rights reserved. Registered with US copy right office 9 в€— Pericardial Diseases в€— Acute Pericarditis Cardiac Temponade Constrictive Pericarditis вЂў Chest pain в†“ Relieved by leaning forward вЂў Pericardial friction rub (Diagnostic) вЂў EKG вЂ“ diffuse ST segment elevation (In MI вЂ“ ST elevation is in different leads according to involvement of heart and it is convex) вЂў Pulsus paradoxus в†“ в†“ SBP more than 10 mmHg on normal inspiration вЂў Neck vein distension with clear lung вЂў Shock [BeckвЂ™s triad вЂ“ Hypotension, JVP, muffled heart sound], next step? в†’ Pericardiocentesis. If no blood on pericardiocentesis, next? в†’ Thoracotomy (blood is clotted in pericardial sac) вЂў Echocardiography в†“ small Heart вЂў KussmaulвЂ™s Sign в†“ в†‘ jugular venous distension with inspiration вЂў Pericardial knock вЂў Treatment в†“ - Pericardiocentesis вЂў D/D: Restrictive cardiomyopathy вЂў CT scan - best choice to demonstrate thickened Pericardium вЂў Treatment в†“ - Diuretics - Pericardiectomy вЂў Treatment involves treating its etiology - Subxiphoid Surgical drainage. вЂў EKG вЂ“ low voltage вЂў Catheterization: square root sign в– Pericardial Effusion: вЂў вЂў вЂў вЂў S.S.Patel. Serosanguineous в†’ TB / neoplasm CXR вЂ“ вЂњwater вЂ“ bottleвЂќ configuration of cardiac silhouette Echocardiography (Best test) Treatment: Fluid Aspiration, Treat etiology All rights reserved. Registered with US copy right office 10 в€— Arrhythmias в€— Sinus Bradycardia вЂў Rate < 60 / min вЂў QRS complex вЂ“ Normal Treatment вЂў None if asymptomatic Sinus Tachycardia вЂў Rate > 100 / min вЂў QRS complex - Normal Treatment вЂў Right carotid sinus massage PSVT вЂў Rate 130-220 / min вЂў Regular rhythm Treatment вЂў IV Adenosine. вЂў Atropine (1st choice) вЂў Pacemaker в€— Heart Block в€— 1st Degree вЂў PR interval > 0.2 sec. вЂў usually asymptomatic вЂў Tx: usually none. If Symptomatic в†’ Atropine. 2nd Degree в†“ Type вЂ“ 1 в†“ ( Wenckebach ) в†“ Type -2 в†“ dropped beat occur suddenly No PR lengthening в†“ в†“ Progressive Tx: Prolongation of Pace PR until a P wave maker is completely (no respBlocked & onse to Dropped Atropine) Beat в†“ вЂў Tx: usually none. If Symptomatic в†’ give Atropine. 3rd Degree вЂў All atrial beasts are blocked. вЂў Ventricles beat by a focus distal to the site of block. (Automaticity of heart) вЂў Tx : Pacemaker Л— Premature atrial beats and PVC (premature ventricular contraction) пѓ observe (never required any treatment) S.S.Patel. All rights reserved. Registered with US copy right office 11 в€— Atrial Arrhythmias в€— Multifocal Atrial Tachycardia вЂў Irregular rhythm Atrial Flutter вЂў morphology of P wave varies from beat to beat вЂў Cardioversion if hemodynamically unstable вЂў Regular rhythm вЂў Irregular rhythm вЂў 2 : 1 block вЂў 300-500 impulse / min вЂў Tx : cardioversion if hemodynamically unstable Tx : cardioversion if hemodynamically unstable or AF is due to angina / MI вЂў If hemodynamically stable then give вЂў Digitalis / ОІ-blockers (IV Metoprolol) / CCB (IV Diltiazam) to lower the HR [below 100] and then elective cardioversion вЂў If AF forвЂє 48 hrs then give Warfarin 3 weeks before an elective cardioversion and continue 4 wks after normal sinus rhythm or do echocardiography to see if there is any clot or not and then do cardioversion вЂў Digoxin вЂ“ preferred if poor LV function вЂў CCB & ОІ-blockers are C/I in patient with poor LV function вЂў If hemodynamically stable then give Digitalis / Calcium Channel Blockers (CCB) вЂў Patient with following sign / symptoms should be considered as hemodynamically unstable: - Hypotension - Chest pain - Confusion - Atrial Fibrillation ( AF ) DOC for Paroxysmal Atrial fibrillation вЂ“ Amiodarone First step in management (Mx) of stable patient with Multifocal Atrial Tachycardia вЂ“ rule out etiology [hypoxia, hypokalamia, hypomagnesemia] в€— Wolff вЂ“ Parkinson вЂ“ White Syndrome ( WPW ): - Short PR interval followed by a wide QRS complex with a Slurred initial deflection, or delta wave, that represent early ventricular activation (PreвЂ“excitation Syndrome) - Tx: Cardioversion if hemodynamically unstable - Procainamide is DOC for WPW +2 вЂ“ Digoxin & Ca channel blockers are contraindicated S.S.Patel. All rights reserved. Registered with US copy right office 12 в€— Torsade de Pointes: - Prolong QT interval, wide QRS complex - Drugs : Quinidine в†“ Procainamide which Disopyramide block K+ channel Anti- Psychotics [Phenothiazine & Thioridazine] - Tx: Magnesium - First step in management of unstable patient with Torsade de pointes вЂ“ immediate defibrillation. DOC for Tx & prevention in stable pt вЂ“ IV Mg (give Mg regardless of patientвЂ™s Mg level) Ventricular Arrhythmias Ventricular Tachycardia вЂў Rate > 120 beats / min Ventricular Fibrillation вЂў Significant electrical activity on EKG with no signs of an organized pattern вЂў IHD вЂ“ (most commonly seen) вЂў QRS complexes are wide and often bizarre VT (Pulse) пЈ¦ в†“ в†“ Stable unstable в†“ в†“ IV lidocaine 1 mg/kg cardioversion or Amiodarone / Procainamide (Amiodarone is preferred when poor LV function) Pulseless VT / VF в†“ Cardioversion в€— Asystole : 1mg Epinephrine every 3-5 mins в†“ 1 mg Atropine every 3-5 mins в†“ 2-5 mg Epinephrine every 3-5 mins п‚§ п‚§ п‚§ п‚§ S.S.Patel. Patent ostium primum (patent foramen ovale) вЂ“ failure of septum primum to fuse with endocardial cushions Atrial Septal Defect (ASD) вЂ“ incomplete adhesion b/w septum primum & septum secondum вЂ“ Wide fixed split S2 VSD вЂ“ defect in membranous interventricular septum PDA вЂ“ machinery murmur вЂ“ associated with congenital rubella вЂ“ PGE2 keep it open вЂ“ it shunts pulmonary artery blood to aorta in fetus All rights reserved. Registered with US copy right office 13 вЂў Л— Л— Л— Л— Coarctation of Aorta: TurnerвЂ™s syndrome Rib notching on CXR, MRI of chest (best test) Tx: surgery Endocarditis prophylaxis required вЂў Л— Л— Л— Л— Л— Л— Tetralogy of Fallot (TOF): Pulmonary stenosis, VSD, RVH and Over riding of aorta Boot shaped hert on CXR Murmur disappears & cyanosis improves when child squats Single or soft S2 Tx: Surgery Endocarditis prophylaxis required вЂў - Transposition of great vessels: Cyanosis in first 24-hrs of life, Infants of diabetic mothers Give PGE1 to keep open PDA until surgical correction done в– Acute Rheumatic fever: - Aschoff bodies (pathognomic) [central area of necrosis surrounded by reactive Histiocytes (Anitschkow cells)] - Pericarditis, Polyarthritis, Chorea, Erythema marginatum and subcutaneous Nodules (Jones criteria) - Usually occur 1-3 weeks after a preceding Strep. Pyogens pharyngitis - в†‘в†‘в†‘ ASO titers - Treatment of acute infection and monthly Penicillin prophylaxis then after. - Tx of SydenhamвЂ™s Chorea (seen several months after acute attack of Rheumatic fever; carditis and arthritis manifest within 21 days) вЂ“ Oral penicillin for 10 days immediately вЂў Yong child with Mitral stenosis due to Acute Rheumatic fever without any symptoms, next step? пѓ Penicillin prophylaxis; If AF develop then consider anticoagulation Location of Murmur Upper Rt sternal border Upper Lt sternal border Lower Lt sternal border Apex Conditions AS, IHSS PS, PDA VSD MVP п‚§ Cardiac Myxoma вЂ“ left atria вЂ“ mesenchymal tumor вЂ“ embolic episode in young person (next step? Send thrombus for pathologic examination, order echo), syncopal episode вЂ“ young adult п‚§ Rhabdomyoma вЂ“ children вЂ“ associated with tuberous sclerosis вЂ“ hemartoma S.S.Patel. All rights reserved. Registered with US copy right office 14 пЃ® Aortic Dissection в†’ cystic medial degeneration (elastic tissue fragmentation) вЂ“ Marfan syndrome & EDS (Ehler-Donlas Syndrome) вЂ“ usually occurs with in 10cm of the aortic valve вЂ“ cardiac temponade most common cause of death вЂ“ Aortic regurgitation, widening of aortic valve root on Echo вЂ“ loss of upper extremity pulse due to compression of subclavian artery п‚§ Best step in management of patient with Acute Aortic Dissection вЂ“ emergent surgery. First step вЂ“ give IV beta blockers [Type A вЂ“ Ascending aorta (emergent surgery) Type B вЂ“ Descending aorta (IV beta blockers)] п‚§ Diagnostic test for Aortic dissection пѓ Transesophageal echocardiography or CT scan пЃ® Abdominal Aortic Aneurysm в†’ pulsatile abdominal mass, next step? в†’ USG (first step) / CT scan (before repair) в†’ < 4 cm (observe), > 6 cm (elective repair) в—Џ Tender AAA / Excruciating back pain, fainting episodes, unequal femoral pulses in patient with known case of AAA в†’ Emergency surgery [Retroperitoneal bleed causes fainting attacks] в—Џ Complication of AAA repair в†’ Ischemic colitis [30% of cases IMA is the only artery which supply sigmoid colon and by repairing AAA obstruct IMA which present as severe abdominal pain, foul smelling diarrhea 2-3 days after repair, next step? в†’ Sigmoidoscopy followed by exploration] пЃ® Peripheral Arterial Disease (PVD): Intermittent claudication / Rest pain, first step? в†’ Ankle-Brachial pressure index measurement в†’ Arteriogram (most accurate test) в†’ stop smoking, stop beta-blockers в†’ start Aspirin, Cilostazol (Antiplatelet vasodilator) в†’ stent / Bypass (most effective treatment) пЃ® Lerich Syndrome: Aortoillio atherosclerosis вЂ“ atherosclerosis of hypogastric artery вЂ“ c/o impotence and claudication пЃ® Deep vein thrombosis: Duplex USG (Best initial test) в†’ Venography (most accurate test) в†’ If Duplex USG positive, next step? в†’ LMW Heparin (sc) for 5-7 days followed by Warfarin for 3-6 months (INR 2-3) [only in patient with metallic heart valve keep INR at 3-4] вЂў Sign & Symptoms of DVT, next step? пѓ Compression UGS to confirm diagnosis before anticoagulation treatment (to avoid potentially serious side effects of anticoagulation therapy in unnecessary pt.) п‚§ Fludrocortisone is the first line medicine for orthostatic hypotension but should be tried after non-pharmacologic trial fail like discontinue dugs causing orthostatic hypotension [Nitrates, CCB, TCA, Opiates analgesics] п‚§ Best initial test / next step in management of patient with acute syncope вЂ“ EKG S.S.Patel. All rights reserved. Registered with US copy right office 15 DERMATOLOGY в– Urticaria: Type вЂ“ 1 HS вЂ“ IgE & mast cell mediated вЂ“ wheals & hives вЂ“ Tx: antihistamines, Desensitization (when trigger cannot be avoided) в– Morbilliform rashes: rash resembles measles вЂ“ вЂњtypicalвЂќ type of drug reaction вЂ“ lymphocyte mediated вЂ“ maculopapular eruption that blanches with pressure вЂ“ Tx: Antihistamines в– Erythema multiforme: Mycoplasma / Herpes Simplex вЂ“ target- like lesions that occur on the palms & soles вЂ“ Tx: antihistamines and treat underlying problem (infection) в– Stevens вЂ“ Johnson Syndrome: (Erythema multiforme major) вЂ“ usually involve < 10-15 % of the total body surface area вЂ“ target-like lesions вЂ“ mucous membrane involvement oral cavity , conjunctiva , respiratory tract вЂ“ Hypersensitivity reaction to drugs в– Toxic epidermal necrolysis: Cutaneous hypersensitivity reaction to drugs вЂ“ (+) Nikolsky sign вЂ“ skin easily sloughs off вЂ“ 40-50 % mortality rate вЂ“ Skin biopsy (most accurate diagnostic test) в– Staphylococcal scalded skin syndrome: loss of the superficial layers of the epidermis вЂ“ Toxin mediated вЂ“ (+) Nikolsky sign вЂ“ Tx: AntiвЂ“staphylococcal antibiotics в– Fixed drug reaction: localized allergic drug reaction that recurs at precisely the same anatomic site on the skin with repeated drug exposure вЂ“ round , sharply demarcated lesions that leave a hyperpigmented spot at the site after they resolved в– Erythema nodosum: multiple painful, red, raised nodules on the anterior surface of the lower extremities вЂ“ recent streptococcal infection, Celiac Sprue Initial work up (ASO titer, CXR, PPD) вЂ“ Tx: treat underlying disease в– Toxic Shock Syndrome: toxin produced from staphylococcal attached to a foreign body (tampon use in female during menstruation) вЂ“ fever , Hypotension , desquamating rash , vomiting , involvement of mucous membrane вЂ“ Hypocalcemia due to capillary leak leads to в†“ albumin level вЂ“ Tx: fluid resuscitation, dopamine (for shock), anti-staphylococcal antibiotics в– Anthrax: Bacillus Anthracis вЂ“ infected livestock (occupation hazard of wool sorters) вЂ“ black eschar вЂ“ Diagnosis: gram stain & culture вЂ“ Tx: ciprofloxacin / Doxycycline S.S.Patel. All rights reserved. Registered with US copy right office 16 пЃ® Impetigo: superficial bacterial infection вЂ“ up to epidermis вЂ“ honey colored crusted lesions (Strep Pyogens) & Staph Aureus (bullous impetigo) вЂ“ Tx: Mupirocin в– Erysipeals: both dermis & epidermis involve вЂ“ shiny red, edematous, tender lesion, fever, chills, bacteremia вЂ“ Strep Pyogens вЂ“ Tx: Systemic antibiotics (Penicillin G) в– * - Folliculitis , Furuncles , Carbuncles: Staphylococcus Folliculitis в†’ infection of hair follicle Furuncles в†’ collection of infected material around hair follicle Carbuncles в†’ several furuncles become confluent in to a single lesion Hot tub Folliculitis в†’ Pseudomonas Treatment : Folliculitis в†’ mupirocin Furuncle & carbuncles в†’ Systemic anti-staphylococcal antibiotics в– Necrotizing Fasciitis: Very high fever, portal of entry into skin, pain out of proportion to the superficial appearance, presence of bullae, palpable crepitus вЂ“ Group A strep (strep Pyogens) вЂ“ X-ray (best initial test) will show air in the tissue вЂ“ surgical debridement (best initial step, next best step in the management, most accurate test) в– * - Dermatophyte Infection: Microsporum, Trichophyton, Epidermophyton Microsporum вЂ“ Skin , Hair , Nail Trichophyton - Hair , Nail Epidermophyton вЂ“ Skin , Nail Usually annular lesions expand peripherally and clear centrally 10 % KOH preparation ( Best initial test ) Culture ( most accurate test ) Bright green fluorescence on WoodвЂ™s UV lamp вЂ“ Microsporum canis [Easy way to remember вЂ“ all three affect nail, epidermophyton вЂ“ skin (epiderm = skin layer)] Treatment: only skin (curis, pedis, corporis) в†’ Topical ( Miconazole ) . Hair / Nail ( capitis , unguium ) в†’ Oral Terbinafine / Itraconazole . Finger nail в†’ 6 weeks oral Tx . - Toe nail в†’ 12 weeks oral Tx Gresiofulvin is used in pediatric population. (Terbinafine is not approved by FDA in pediatric population) в– Tinea Versicolor: Malassezia furfur (Pityrosporum orbiculare) вЂ“ white, scaling lesions that tend to coalesce вЂ“ Tx : topical Selenium Sulfide в– Pityriasis rosea: вЂњherald patchвЂќ Christmas tree pattern вЂ“self-limited вЂ“ looks like secondary syphilis except it spares palm & sole вЂ“ VDRL / RPR в†’ negative . S.S.Patel. All rights reserved. Registered with US copy right office 17 в– Pediculosis: lice - Dx в†’ direct examination of hear-bearing area вЂ“ Tx: Pyrethrins (OTC вЂ“ over the counter) в– Telogen effluvium: loss of hair in response to excessive physiological stress. в– Scabies: primarily involve web spaces of the hand and feet вЂ“ burrows and excoriation around pruritic lesion вЂ“ Permethrin (Best initial Tx), Lindane вЂў Norwegian Scabies (in immunocompromised person) в†’ Ivermectin oral в– Alopecia areata: autoimmune вЂ“ Tx: localized steroid injection вЂ“ recurrence of hair loss can occur after successful treatment в– Solar lentigo: Freckles вЂ“ sun exposed area in elderly в– Seborrheic keratosis: verrucoid lesion with вЂњstuck on appearanceвЂќ вЂ“ no malignant potential вЂ“ Tx: liquid nitrogen or curettage в– Actinic keratosis: Precancerous lesion вЂ“ sun exposed area вЂ“ can progress to squamous cell CA вЂ“ Tx: sunscreen, Topical 5-fluorouracil (5-FU), Tretinoin (emollient cream) [Tretinoin is also used for wrinkles, hyperpigmentation (mottled) and rough facial skin] в– Acanthosis nigricans: Verrucoid pigmented skin lesion usually located in Axilla вЂ“ Stomach adenocarcinoma, MEN II b, insulin resistance (DM, Obese) в– - Nevocellular nevus ( Mole ): nevus cells are modified melanocytes Junctional nevus в†’ basal cell layer ( childhood ) Compound nevus в†’ extend into superficial dermis ( adolescents ) Intradermal nevus в†’ compound nevus loses its junctional component (Adult) Dysplastic nevus ( Atypical mole ) в†’ в†‘ risk for malignant melanoma вЂ“ yearly dermatologic examination require в– Melanoma: Most common type of malignancy вЂ“ sun exposed area - в†‘ risk in dysplastic nevus syndrome, xeroderma pigmentosa вЂ“ asymmetry, borders irregular, color changes, diameter increased вЂ“ depth of invasion вЂ“ best prognostic factor [< 0.76 mm вЂ“ do not metastasize, > 1.7 mm вЂ“ potential for metastasis] вЂ“ Tx: Surgical excision вЂ“ Prevention of malignant melanoma пѓ Protective clothing в– Basal cell CA : Most common skin cancer вЂ“ sun exposed area вЂ“ upper lip Raised papule, shiny (or) вЂњPearly вЂќ appearance вЂ“ Dx в†’ Punch Biopsy в– Squamous cell CA : Lower lip вЂ“ sun exposed area, tobacco use, scar tissue in 3rd degree burn, Actinic keratosis вЂ“ ulcerated lesion вЂ“ Dx в†’ Biopsy S.S.Patel. All rights reserved. Registered with US copy right office 18 оЂЉ Marjolin ulcer в†’ development of squamous cell CA in a chronic leg ulcer в†’ heaped up tissue growth around the edges в†’ Tx: wide local excision & skin grafting в– Pemphigus vulgaris вЂ“ IgG against desmosomes (intracellular attachment in the epidermis) - Suprabasal, (+) Nikolsky sign, Oral lesions - Acantholysis of karatinocytes in the vesicle fluid в– Bullous Pemphigoid вЂ“ IgG against basement membrane [IgG & C3 deposit at dermal-epidermal junction] - Subepidermal vesicle, (в”Ђ) Nikolsky sign - No Acantholysis of karatinocytes in the vesicle fluid в– Dermatitis Herpetiformis вЂ“ IgA-anti-IgA complex deposit at the tips of the dermal papillae - Subepidermal vesicle with Neutrophils, microscopic blisters - Strong association with Celiac disease - Tx: Dapsone пЃ® Psoriasis вЂ“ coin-shaped lesions cover with silvery-scale вЂ“ on extensor surface вЂ“ nail pitting вЂ“ development of lesion in area of trauma (Koebner phenomenon) вЂ“ bleeding occur when scale is scraped off (Auspitz sign) вЂ“ neutrophil collection in stratum corneum (Munro microabscesses) вЂ“ Tx: Salicylic acid, Vit-A & Vit-D derivatives, coal tar, anthralin derivatives, infliximab (immunomodulatory agents) вЂ“ mild localize [topical high potency steroids (betamethasone 0.05% cream)] вЂ“ Drug causing flare up of psoriasis вЂ“ Lithium, beta-blockers, ACE inhibitors пЃ® Lichen Planus вЂ“ shiny, discrete, intensely pruritic, polygonal shaped violaceous plaques and papules вЂ“ flexor surface вЂ“ mucous membranes (mouth and external genitalia) вЂ“ association with Hepatitis C (30% of cases) пЃ® Porphyria Cutanea Tarda вЂ“ Phototoxicity (painless blistering on sun exposed area), facial hyperpigmentation вЂ“ Dx: urinary uroporphyrins вЂ“ Tx: avoid offending agents (ethanol, estrogen) пЃ® Atopic Dermatitis вЂ“ extreme pruritus вЂ“ flexor surface - в†‘ IgE вЂ“ avoid scratchingвЂ“ Tx: topical steroid, antihistamines, tacrolimus (topical immunosuppressants) вЂ“ Type-1 HS Л— Vesicles over the area of atopic dermatitis вЂ“ Eczema Herpeticum пЃ® Contact Dermatitis вЂ“ linear, streaked vesicles (weeping lesion) вЂ“ Type-4 HS пЃ® Seborrheic Dermatitis (dandruff) вЂ“ scaly, greasy, flaky skin вЂ“ pityrosporum ovale вЂ“ Tx: Zinc, Selenium shampoo S.S.Patel. All rights reserved. Registered with US copy right office 19 пЃ® Candadial diaper dermatitis вЂ“ tomato-red color plaques, satellite papules in perineal area вЂ“ Tx: clotrimazole cream пЃ® Stasis Dermatitis вЂ“ hyperpigmentation built up from hemosiderin in the tissue вЂ“ varicose veins for long time. пЃ® Xerosis (Asteotic Dermatitis) вЂ“ dry skin вЂ“ elderly (due to decrease in lipid) вЂ“ Tx: emollient пЃ® Keratoacanthoma: rapidly growing, benign crateriform tumor with a central keratin plug вЂ“ sun exposed area. Regress spontaneously with scarring пЃ® Nummular Dermatitis: coin-shaped lesions (discoid lesions) пЃ® Pompholyx: deep-seated vesicles on the palms, fingers and soles S.S.Patel. All rights reserved. Registered with US copy right office 20 ENDOCRINOLOGY п‚§ Hyperprolactinemia: Excess production (Pituitary adenoma) or Disinhibition [decrease dopamine вЂ“ dopamine antagonist (Phenothiazine, metoclopramide) вЂ“ dopamine depleting agents (methyldopa, Reserpine) вЂ“ primary hypothyroidism (increase TRH-activated dopamine which overcome the normal dopamine inhibition)] Physiologic [pregnancy, breast feeding, stress] Other [Sarcoidosis] вЂ“ Microadenomas (amenorrhea, galactorrhea, decrease libido) вЂ“ Macroadenomas (visual field defect вЂ“ heteronymous hemianopsia) вЂ“ other important pathology [hypoestrogenism вЂ“ osteoporosis] Л— First step in management of Hyperprolactinemia пѓ rule out Hypothyroidism (measure THS level) Л— Diagnosis вЂ“ Prolactin level вЂє 1000 mIU/L suggest pituitary adenoma. Л— Tx: Bromocriptine (dopamine agonist), surgery if doesnвЂ™t get help with drug. Stop using dopamine antagonist/deplete drugs. п‚§ Acromegaly: в†‘в†‘ GH (gigantism in children) вЂ“ pituitary adenoma вЂ“ b/w 3rd and 5th decade вЂ“ c/o unable to wear wedding ring, increase in shoe size вЂ“ entrapment neuropathy, osteoarthritis, hypertension, impaired glucose tolerance, heart failure (late) вЂ“ symptoms for an average of 9 yrs before diagnosis вЂ“ Diagnosis: GH level remains вЂє 1 Ојg/L after giving 100gm of glucose orally вЂ“ Tx: Bromocriptine, Octreotide, surgery п‚§ Laron Dwarfism: congenital absent of GH receptor - в†‘ GH, в†“ IGF-1 & undetectable GH binding protein in blood п‚§ Hypopituitarism: decrease level of one or more pituitary hormones (decrease in most pituitary hormones is referred as panhypopituitarism) вЂ“ FSH & LH (most common), GH then TSH and lastly ACTH вЂ“ Causes: Pituitary adenoma Craniopharyngioma, meningioma, gliomas, Pituitary apoplexy (acute hemorrhage in preexisting pituitary adenoma - emergency), Sheehan syndrome (postpartum necrosis of pituitary due to loss of blood intrapartum), Infection, Autoimmune, Empty sella syndrome вЂ“ amenorrhea, infertility, decrease libido, fatigue, inability to lactate (1st sign in Sheehan syndrome) - Tx: replacement of hormones. Cortisol and Thyroid hormones replacement is most important step in management during emergency. Treat underlying causes п‚§ CT head вЂ“ No pituitary + normal hormone level вЂ“ Empty sella Syndrome п‚§ Very small pituitary tumor on MRI, but no abnormality in hormones and absence of any symptoms, next step? вЂ“ repeat MRI after 6-12 months п‚§ Diabetes Insipidus: excessive thirst, polyuria (form dilute urine in the presence of Hypernatremia) вЂ“ central (insufficient ADH) & Nephrogenic S.S.Patel. All rights reserved. Registered with US copy right office 21 (unresponsiveness of kidney to ADH) вЂ“ Diagnosis: (1) increase in Urine Osm after giving vasopressin вЂ“ central DI. (2) increase Urine Osm after dehydration вЂ“ psychogenic вЂ“ Tx: central DI вЂ“ vasopressin (SC) / Desmopressin (intranasally, SC, orally), Chlorpropamide, Carbamazapine (increase ADH secretion) вЂ“ Nephrogenic DI вЂ“ Amiloride (K+ sparing diuretics) вЂ“ Mx of Hypotension in pt with DI вЂ“ IV normal saline п‚§ SIADH: cancers (small cell CA of lung), Drugs (Chlorpropamide, carbamazapine) вЂ“ continuously form concentrated urine in the presence of hyponatremia вЂ“ Tx: fluid restriction, Demeclocycline, Lithium. п‚§ * Diabetes Insipidus в†’ elevated Serum Osmolarity * Primary polydipsia (Psychogenic) в†’ both serum & urine diluted * SIADH в†’ elevated urine osmolarity п‚§ ConnвЂ™s Syndrome: Primary Hyperaldosteronism вЂ“ adenoma of zona glomerulosa (adrenal gland) вЂ“ hypertension (sodium retention), hypokalamia (muscle weakness) вЂ“ plasma aldosterone to plasma rennin activity (>30 suggest diagnosis) (Initial screening test) вЂ“ Tx: resection [D/D: Renal artery stenosis вЂ“ BUN:Cr >20:1, abd bruits, hypokalamia is less severe than ConnвЂ™s syndrome; If Hypokalamia in ConnвЂ™s is in 2.something range, hypokalamia in renal artery stenosis is in 3.something range but less than 3.5; (3.5 вЂ“ 5.0 normal range)] вЂў Patient with HTN & hypokalemia, next step? вЂ“ plasma aldosterone and renin ratio [Dx: Primary Hyperaldosteronism (ConnвЂ™s syndrome)] п‚§ Hyperthyroidism: в†‘в†‘ T3 & T4, в†“в†“ TSH вЂ“ heat intolerance, weight loss, diarrhea, tremor, arrhythmias; Exophthalmos & dermatopathies(only in GraveвЂ™s disease) вЂ“ GraveвЂ™s disease - в†‘ RAIU (radioactive iodine uptake), antiTSH receptor antibodies. Toxic nodular goiter (single / multiple) - в†‘ RAIU. de Quarian Thyroiditis вЂ“ subacute granulomatous, giant cell - в†“ RAIU вЂ“ painful (transient hyperthyroidism). Subacute lymphocytic Thyroiditis - в†“ RAIU вЂ“ painless (transient hyperthyroidism). Ectopic thyroid tissue вЂ“ struma ovarii вЂ“ Tx: antithyroid drugs (only PTU(propythiouracil) safe in pregnancy), radioactive iodine, subtotal thyroidectomy (only indicated in pregnancy in 2nd trimester) вЂ“ Tx: subacute thyroiditis вЂ“ NSAIDs & beta-blockers п‚§ Tx of GraveвЂ™s disease in USA вЂ“ radioiodine ablation with concurrent administration of glucocorticoids to prevent worsening of ophthalmopathy п‚§ Thyroid storm: First step Propranolol (to control symptoms) & PTU and then radio active iodine or surgery for permanent treatment [Radio active iodine aggravate hyperthyroidism initially so never give it before giving PTU] п‚§ S.S.Patel. Tx of hyperthyroidism in pregnancy вЂ“ PTU; if PTU fails, surgery All rights reserved. Registered with US copy right office 22 п‚§ п‚§ п‚§ п‚§ Hypothyroidism: в†“в†“ T3 & T4, в†‘в†‘ TSH (primary) but normal / в†“ TSH (secondary / tertiary) вЂ“ cold intolerance, weight gain, amenorrhea, carpal tunnel syndrome, slow deep tendon reflexes with prolonged relaxation phase, myxedema (prolonged hypothyroidism) вЂ“ Hashimoto Thyroiditis: antimicrosomal antibody, anti-thyroglobulin antibody, lymphocytic infiltration вЂ“ associated with lymphoma in thyroid gland вЂ“ Tx: levothyroxine(T4), in secondary & tertiary first give hydrocortisone then replace thyroid hormone. вЂ“ Calcium and Iron preparation decrease absorption of levothyroxine вЂ“ Estrogen increase metabolism of levothyroxine therefore it is important to increase dose of levothyroxine in patient taking HRT вЂ“ important side effect: AF and osteoporosis Congenital Hypothyroidism: apathy, weakness, hypotonia, large tongue, abdominal bloating and umbilical hernia вЂ“ MCC of congenital hypothyroidism in USA вЂ“ thyroid dysgenesis вЂ“ Neonatal screening for Hypothyroidism: total T4 and TSH; If T4 is low and TSH is >20 U/L, next step? вЂ“ repeat free T4 and TSH on a regular blood draw to confirm diagnosis Asymptomatic Hypothyroidism: look for Anti-thyroid antibodies, abnormal lipid profile or menstrual abnormality вЂ“ If any out of three present, start treatment (levothyroxine) Myxedema: results from the accumulation of increased amounts of hyaluronic acid and chondroitin sulfate in the dermis in both lesional and normal skin вЂ“ can be seen in both hypothyroidism and hyperthyroidism вЂ“ jelly like infiltration in subcutaneous tissue, eye puffiness, non-pitting edema, drowsiness, lethargy and coma (in severe cases) вЂ“ Tx: intravenous steroids, levothyroxine п‚§ Reidle Thyroiditis: intense fibrosis of thyroid gland and surrounding structure. п‚§ Papillary CA: most common thyroid CA вЂ“ h/o radiation exposure вЂ“ Tx: surgery (small), radiation (large tumor) п‚§ Follicular CA: elderly вЂ“ spread hematogenously вЂ“ Tx: near total Thyroidectomy + post-op- radiation. п‚§ Medullary CA: parafollicular cell of thyroid gland - в†‘в†‘в†‘ п‚§ Anaplastic CA: elderly вЂ“ highly malignant with rapid and painful Calcitonin вЂ“ association with MEN type-2b вЂ“ more malignant then follicular вЂ“ Tx: Thyroidectomy enlargement of thyroid gland вЂ“ poor prognosis п‚§ S.S.Patel. Patient with Thyroidectomy due to CA should receive thyroxine life long. Check TSH on follow up visit and it should be below normal (<0.35). If it is greater than 0.35, increase dose of thyroxine All rights reserved. Registered with US copy right office 23 п‚§ Thyroid nodule: measure TSH (first step); If TSH normal, next step? вЂ“ FNAC; If TSH decreased, next step? вЂ“ Radioisotope scan; If hot nodule (increase uptake of radioisotope), next step? вЂ“ Observation п‚§ п‚§ Most common cause (MCC) of thyroid nodule вЂ“ benign colloid nodule 2nd MCC of thyroid nodule вЂ“ follicular adenoma п‚§ Parathyroid Hormone: It stimulates Osteoclast & 1-О±-hydroxylase (increase production of active form of Vit-D в†’ 1-25-(OH)2-D) вЂ“ increase Ca+2 level by bone resorption (osteoclast) & by absorption of Ca+2 from gut and kidney (Vit-D). п‚§ Vit-D: increase absorption of both Ca+2 & Phosphorus (PO4) from intestine + increase absorption of Ca+2 and decrease absorption of PO4 from kidney. п‚§ Magnesium: cofactor for adenylate cyclase вЂ“ cAMP is require for PTH activation вЂ“ therefore hypomagnesemia can cause hypocalcemia (hypomagnesemia is the most common pathologic cause of hypocalcemia in the hospital - Cause of refractory hypokalemia in alcoholics в†’ Hypomegnesemia) п‚§ Calcitonin: inhibit bone resorption п‚§ Primary Hypo- / Hyperparathyroidism: plasma calcium & phosphate levels are changing in opposite direction EXCEPT CRF which causes secondary hyperparathyroidism but in CRF there is hypocalcemia & hyperparathyroidism (moves in opposite direction) п‚§ Pseudohypoparathyroidism вЂ“ resistance to PTH on its target tissue. High PTH and High phosphorus [true Hypoparathyroidism has high phosphorus and low PTH; calcium is low in both] п‚§ Secondary Hyperparathyroidism: Increase PTH, decrease Ca+2 level & its excretion and decrease PO4 level & normal/increase its excretion п‚§ Secondary Hypoparathyroidism: Decrease PTH, increase Ca+2 level & its excretion and increase PO4 level & normal/decrease its excretion. п‚§ Hypercalcemia: primary hyperparathyroidism (one gland hyperplasia), PTHlike substance secretion from CA, Immobilization for prolong time [can cause excessive bone resorption], Sarcoidosis вЂ“ Osteitis fibrosa cystica, lytic lesions on x-rays вЂ“ serum Ca+2 level more than 10.2 mg/dl вЂ“ Tx: oral rehydration with 2-3 L/day is very effective in chronic form. Sever Hypercalcemia (вЂє15 mg/dl) is an emergency вЂ“ IV normal saline (first step), Loop diuretics, IV Pamidronate. S.S.Patel. All rights reserved. Registered with US copy right office 24 п‚§ Calcium level above 7 in patient with low albumin level (Normal вЂ“ 4 g/dl) usually doesnвЂ™t require any intervention п‚§ Hungry bone syndrome: hypocalcemia after surgical removal of a hyperactive parathyroid gland. п‚§ Hypocalcemia: tetany, muscle cramps / spasm, Chovestic sign (percussion of Facial N. leads to contraction of facial muscles), TrousseauвЂ™s sign (inflation of BP cuff on the arm of patient above SBP for more than 3 mins leads to flexion of metacarpophalangeal joints and extension of interphalangeal joint), QT prolongation on EKG вЂ“ always check for albumin level (1 gm/dl drop in albumin в†’ calcium level drop by 0.8 mg/dl) вЂ“ Tx: for acute condition вЂ“ IV calcium gluconate, for chronic condition (low Ca & low PO4 вЂ“ Vit-D deficiency) вЂ“ oral calcium 2-4 gm/day, Vit-D; If associated hyperphosphatemia (eg. CRF) вЂ“ dietary phosphate restriction, phosphate binders. п‚§ Pathogenesis of DM: Nonenzymatic glycosylation (glucose + AA) п‚§ Diagnosis of DM: symptomatic patient (polyuria, Ploydipsia, polyphagia) в†’ в†‘vessel permeability to protein and в†‘athrogenesis; Osmotic damage в†’ Aldolase reductase (glucose в†’ sorbitol) в†’ sorbitol draws water into tissues causing damage (eg. retinopathy); Diabetic microangiopathy в†’ в†‘synthesis of type-IV collagen in basement membrane & mesangium Л— Tight blood sugar control decrease the risk of development of microvascular complication (retinopathy, nephropathy, neuropathy) Л— ACE inhibitors has shown to reduce insulin resistance with random blood glucose level вЂє 200 mg/dl or fasting blood glucose вЂє 126 mg/dl on two occasion or blood glucose вЂє 200 mg/dl at 2 hrs and on at least one of the earlier samples. п‚§ HbA1c: used to follow compliance of the treatment and glucose control in patient with diabetes. п‚§ Somogyi effect: rebound hyperglycemia in the morning because of counter regulatory hormone release after an episode of hypoglycemia in the middle of night. п‚§ Dawn Phenomenon: early morning rise in plasma glucose requiring increased amount of insulin to maintain euglycemia. [dawn goes up] п‚§ п‚§ Insulinoma: increase in both plasma insulin and C peptide Exogenous insulin administration: very high insulin level but low C peptide S.S.Patel. All rights reserved. Registered with US copy right office 25 п‚§ Sulfonylureas: increase in both plasma insulin and C peptide, plasma/urine sulfonylurea (+) вЂ“ Tx of refractory hypoglycemia due to sulfonylureas overdose вЂ“ Octreotide п‚§ Management of Diabetic Ketoacidosis: 1. IV Normal saline + IV regular insulin 2. correction of electrolyte imbalance (especially K+) (D 5% in 0.45% saline with potassium after glucose level reaches 200-250 mg/dl) 3. Treatment of precipitating factor. Л— Important point to remember in Tx of DKA: Start regular insulin subcutaneously 30-60 mins before stopping IV insulin Л— Cause of hyperkalemia in DKA вЂ“ extracellular shift of K+ п‚§ Tx of Hyperosmolar Hyperglycemia: Hydration and Insulin. Hydration [hyponatremia / hypovolemic shock вЂ“ 0.9% NaCl (Normal saline); Hypernatremia вЂ“ 0.45% NaCl (half normal saline)] п‚§ Tx of Alcoholic Ketoacidosis вЂ“ Hydration (IV fluids containing Dextrose) and Thiamine вЂў Type-II Diabetic patient with low bicarbonate, normal blood sugar вЂ“ order ABG & lactic acid level вЂ“ Dx: Metabolic acidosis due to Metformin п‚§ Somatostaninoma вЂ“ diabetes (inhibits insulin release), gall stones (inhibits gall bladder motility) and Malabsorption (inhibit release of pancreatic enzymes) вЂ“ Dx: measure fasting somatostatin level (>160 pg/ml is very suggestive) п‚§ Best screening and diagnostic test for diabetic neuropathy вЂ“ nerve conduction studies (show axonal pattern of nerve damage) п‚§ п‚§ Primary Hypercortisolism (Adrenal Tumor) - в†‘ cortisol, в†“ ACTH Pituitary Cushing (CushingвЂ™s disease) - в†‘ cortisol, в†‘ ACTH. High dose Dexamethasone test - в†“cortisol, в†“ ACTH by 50% Ectopic ACTH secretion - в†‘ cortisol, в†‘ ACTH. High dose Dexamethasone test вЂ“ No suppression of ACTH п‚§ п‚§ S.S.Patel. Patient with sign & symptoms of Cushing вЂ“ low dose overnight Dexamethasone suppression test (best initial test) вЂ“ if abnormal then order 24 hrs urine-free cortisol вЂ“ if abnormal (Cushing Syndrome) вЂ“ then order high dose Dexamethasone test вЂ“ if no suppression of ACTH then order ACTH level вЂ“ if high ACTH level (Ectopic ACTH secretion вЂ“ CT chest will be the next step) вЂ“ if low ACTH level (Adrenal neoplasia) вЂ“ then order Urinary 17KS, DHEA-S & Abdominal CT вЂ“ if high level of these hormones and greater than 4 cm mass on CT (Adrenal CA) вЂ“ if low level of these hormones and less than 4 cm mass on CT (Adrenal hyperplasia) All rights reserved. Registered with US copy right office 26 п‚§ п‚§ If low dose overnight dexamethasone test is normal, Cushing is ruled out If 24-hrs urine-free cortisol test is normal, no Cushing п‚§ Young patient with HTN, diabetes, osteoporosis and hypokalamia should screen for CushingвЂ™s syndrome (order dexamethasone suppression test) Patient with central hypothyroidism [low normal TSH and low T4], next step? вЂ“ rule out adrenal insufficiency (order cosyntropin stimulation test) Tx of acute adrenal insufficiency вЂ“ IV Dexamethasone (long acting) Short-term use of glucocorticoids (less than 3 wks) even in high dose can be discontinued rapidly without causing significant adrenal insufficiency п‚§ п‚§ п‚§ вЂў Bitemporal hemianopsia & tanned skin (hyperpigmentation) following adrenalectomies for CushingвЂ™s disease вЂ“ NelsonвЂ™s syndrome п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ S.S.Patel. 17 О± Hydroxylase Deficiency: в†“ cortisol and androgen but в†‘ 11 deoxycorticosterone (weak mineralocorticoid due to which retention of sodium occur and hypertension develop) 21 ОІ Hydroxylase Deficiency: в†“ cortisol and mineralocorticoid but в†‘ androgens 11 ОІ Hydroxylase Deficiency: в†“ cortisol but в†‘ androgens and mineralocorticoid Increase ACTH in all of 3 enzymes deficiency (above) Male ambiguous genitalia (male pseudohermaphrodite) вЂ“ 17 О± Hydroxylase Female ambiguous genitalia (female pseudohermaphrodite) вЂ“ 21 & 11 ОІ Hydroxylase Female Hypogonadism вЂ“ 17 О± Hydroxylase Precocious puberty in male вЂ“ 21 & 11 ОІ Hydroxylase Dx of 21-Hydroxylase deficiency is confirmed by documenting elevated 17-alpha hydroxyprogesterone (not 17-alpha hydroxypregnenolone) MEN 1 (Wermer) вЂ“ Pancreas (ZE syndrome), Pituitary, Hyperparathyroidism (3P) MEN 2a (Sipple) вЂ“ Hyperparathyroidism, Pheochromocytoma, Medullary CA of Thyroid MEN 2b (3) вЂ“ Pheochromocytoma, Medullary CA of Thyroid, mucosal neuroma (lips/tongue) Presence of Y chromosome вЂ“ germinal tissue differentiate in to Testes. hCG + LH в†’ leyding cells в†’ testosterone в†’ wallfian duct (epididymis, ductus deference, ejaculatory duct) в†’ 5 О± reductase convert testosterone in dihydrotestosteron (DHT) which induce urogenital sinus & genital tubercle to form penis, prostate & scrotum. Sertoli cells в†’ secrete MIF (mullerian inhibiting factor) which inhibit paramesonephric duct (uterus, uterine tubes, cervix & upper part of vagina) All rights reserved. Registered with US copy right office 27 п‚§ п‚§ п‚§ п‚§ п‚§ S.S.Patel. If MIF absent вЂ“ uterus (paramesonephric duct structure) develop with normal male structure If Testosterone absent вЂ“ wallfian duct regress (male internal structure not develop) If 5 О± reductase absent вЂ“ DHT not formed. Therefore male external structures not develop but female external structures develop. Absence of Y chromosome вЂ“ germinal tissue differentiate in to Ovaries. Wallfian (mesonephric duct) regress and female genitalia develop. Testicular Feminization вЂ“ androgen receptor insensitivity, Mullerian duct structure develop in the presence of testes. (No effect of testosterone & DHT) All rights reserved. Registered with US copy right office 28 GESTROENTEROLOGY вЂў Choanal atresia вЂ“ Child become cyanotic when feeds and turn pink when cries вЂў Gastroschisis в†’ first step? в†’ Sterile wrapping of exposed bowel; next? пѓ orogasric tube to decompress stomach & IV nutrition вЂў Omphalocele в†’ shiny, thin, membranous sac at the base of the umbilical cord, cord goes to the defect, not to the baby в†’ can have multiple defects вЂў Pyloric Stenosis в†’ Projectile vomiting in infants вЂ“ hypertrophy of pyloric region в†’ Usage of Erythromycin is associated with infantile hypertrophic pyloric stenosis в†’ Diagnostic test: Abdominal USG в†’ First step in management: IV fluids & K+ supplement в†’ Tx: Pyloroplasty вЂў Infant with congenital diaphragmatic hernia, next step? пѓ orogastric tube placement * Dysphagia: п‚· Best Initial Test в†’ barium swallow / barium esophagus п‚· Dysphagia for solids not liquid в†’ Obstruction вЂ“ stricture, esophageal CA PlummerвЂ“Vinson Syndrome вЂў Dysphagia for solids and liquid в†’ Peristalsis problem вЂ“ Achalasia , Systemic Sclerosis, CREST, Polymyositis * вЂў вЂў * вЂў вЂў * вЂў вЂў * вЂў вЂў вЂў S.S.Patel. Achalasia вЂ“ Absent myenteric ganglion Most accurate test вЂ“ esophageal manometry [normal amplitude contraction with high tone of lower sphincter (failure to relax)] Best Initial Therapy вЂ“ pneumatic dilatation Esophageal spasm вЂ“ вЂњcorkscrewвЂќ pattern on barium study Most accurate test вЂ“ esophageal manometry [high amplitude contraction with normal relaxation of lower esophageal sphincter] Tx: Ca + 2 channel blockers, Nitrates Scleroderma [Progressive Systemic Sclerosis (PSS)] Most Accurate test вЂ“ esophageal manometry (absence peristalsis wave with very low tone of lower esophageal sphincter) Tx: Proton-pump inhibitors, Metoclopramide Ring & Webs : SchatzkiвЂ™s ring вЂ“ more distal & located at the squamocolumnar junction Plummer-Vinson вЂ“ more proximal & located in the hypopharynx [iron deficiency anemia] Diagnosis в†’ Barium swallow / Barium esophagus вЂ“ Tx: Dilatation All rights reserved. Registered with US copy right office 29 * вЂў вЂў Esophageal CA : Tobacco + Alcohol в†’ SCC в†’Upper 2/3 GERD & Barrett esophagus в†’ Adenocarcinoma в†’ lower 1/3 Endoscopy is mandatory. CT chest в†’ to detect degree of spread * ZenkerвЂ™s Diverticulum: out pocketing of the posterior pharyngeal Constrictor muscles at the back of the pharynx Bad breath, aspiration pneumonia / lung abscess (anaerobic) Diagnosis в†’ Barium study (Esophagography) Tx : Surgical resection (Endoscopy & placement of nasogastric tubes are C/I) вЂў вЂў вЂў вЂў Patient present with aspiration pneumonia due to ZenkerвЂ™s diverticulum started on antibiotics (treatment for pneumonia), next step? Esophagography (barium). [Not scopy] * вЂў вЂў вЂў Gastroesophageal Reflux Disease ( GERD ): Epigastric pain going under sternum , non-productive cough at night (it can cause worsening of asthma at night by irritating bronchus), bad taste in mouth Most Accurate Test в†’ 24-hrs PH monitoring Tx: PPIs (best initial), Metoclopramide, H2 вЂ“ blockers, Nissen fundoplication вЂў вЂў Sliding Hernia of esophagus вЂ“ GE junction displaced & reach above Paraesophageal Hernia вЂ“ GE junction not displaced * Л— Л— Л— Л— Barrett Esophagus: Metaplasia (squamous в†’ columnar) Repeat Endoscopy every 2-3 yrs Low grade dysplasia в†’ repeat endoscopy in 3-6 months High grade dysplasia в†’ distal esophagecomy All patients with Barrett esophagus should be on PPIs * Mallory Weiss Tear в†’ continuous retching followed by large painless bloody vomiting (mucosal tear) в†’ Hiatal hernia [predisposing factor] в†’ Endoscopy (best initial) в†’ Tx: resolve itself * Boerhaave Syndrome в†’ continuous retching followed by severe chest pain, Crepitation in the neck, air in mediastinum on CXR [Esophageal rupture вЂ“ usually in distal third, posterolateral segment (where there is no serosa) is the most common site] в†’ Tx: emergency surgical repair вЂў вЂў вЂў Esophagitis: Candida Albicans (MCC) typically in HIV patient (CD 4 < 200) Next step в†’ Empiric Tx with Fluconazole HIV positive в†’ C/O Odenophagia в†’Already on Anti-fungal в†’ Endoscopy (next step) Other causes of Esophagitis в†’ HSV, CMV, Aphthus ulcers S.S.Patel. All rights reserved. Registered with US copy right office 30 * Epigastric Pain пЈ¦ в†“ Young Patient without any S/S Suggestive of CA в†“ Empirical Treatment (PPIs / H2 вЂ“ blockers) в†“ If no improvement Endoscopy with biopsy в†“ Patient > 45 yrs. Old with / without S/S suggestive of CA (Dysphagia, wt. loss) в†“ Endoscopy with biopsy * вЂў вЂў вЂў вЂў вЂў вЂў Diagnosis of H.pylori: Serology (Best initial test), urea breath testing, stool antigen Endoscopy with biopsy & histology (Most accurate test) CLO test в†’ rapid test on biopsy which check Urease produced by H. pylori Breath & Stool Test в†’ Best post-treatment test to check eradication of H. pylori Tx: PPI + Clarithromycin + Amoxicillin If organism is eradicated & still ulcer persist в†’ check for ZE syndrome * вЂў вЂў вЂў вЂў вЂў вЂў вЂў ZollingerвЂ“Ellison Syndrome (ZE syndrome): Multiple recurrent ulcers (usually duodenum) , Steatorrhea Associated with MEN вЂ“ I (Parathyroid, Pituitary, Pancreas) в†‘в†‘в†‘ acid production inactivate pancreatic enzymes в†’ malabsorption Diagnosis: Elevated gastrin level Secretin stimulation test (if gastrin level is non diagnostic in suspected patient) Next step after Diagnosis в†’ search for metastasis в†“ Nuclear test (Somatostatin receptor scintigraphy) Tx: Localize в†’ Surgical resection, Metastasis в†’ PPIs * вЂў вЂў вЂў вЂў Gastritis: Type вЂ“ A в†’ Atrophic gastritis (Autoimmune) , Vit-B12 deficiency , в†‘в†‘в†‘ gastrin Type вЂ“ B в†’ NSAID, H. Pylori, Alcohol Tx: Vit- B12 replacement, PPIs, H2 вЂ“ blockers Increase chance of gastric CA in patient with Type-A gastritis * вЂў вЂў вЂў вЂў Gastroparesis: weak stomach Diabetes neuropathy (MCC) C/O early satiety, Postprandial nausea, в†‘ Abdominal fullness Diagnosis в†’ gastric emptying study Tx: Erythromycin / Metoclopramide S.S.Patel. All rights reserved. Registered with US copy right office 31 * вЂў вЂў вЂў вЂў Dumping Syndrome: 1St в†’ hypertonic chyme release in duodenum в†’ osmotic draw into duodenum leading to intravascular volume depletion 2nd в†’ sudden в†‘ in glucose level в†’ в†‘ insulin release в†’ Hypoglycemia Tx: Eat multiple, small meals Protein loosing enteropathy: Enlarge rugal folds вЂ“ hypertrophy of mucous cells (Menetrier disease, ZES, lymphoma) в– Inflammatory Bowel Disease: * CrohnвЂ™s Disease [CD]: Oral / Perianal involvement , palpable Abdominal mass (granuloma вЂ“ characteristic of Crohn), Transmural involvement, Skip lesions, Fistula formation * Ulcerative colitis: rectum involvement, bloody diarrhea , exclusive mucosal disease вЂ“ Patient with ulcerative colitis and pancolitis should begin surveillance colonoscopy after 8 yrs of having the disease * Best diagnostic Test в†’ Endoscopy Л— CD в†’ AntiвЂ“Saccharomyces cerevisiae antibodies (ASCA) Л— UC в†’ ANCA вЂў CD в†’ VitвЂ“B12, VitвЂ“K, Ca +2, iron deficiency в†’ elevated PT, kidney stones, Megaloblastic anemia * Tx: Mesalamine [5-Aminosalicylic acid (5-ASA)] derivatives (best initial) вЂў Infliximab в†’ CD with fistula formation / refractory to other therapy вЂў Acute Exacerbation в†’ High dose steroids (Budesonide) вЂў Young patient with bloody diarrhea, if normal rectosigmoidoscopy вЂ“ CrohnвЂ™s disease; If rectum if affected вЂ“ Ulcerative colitis * вЂў вЂў вЂў вЂў Lactose Intolerance: lactase deficiency Diarrhea associated with gas & bloating after drinking milk Never has blood / WBC in stool Diagnosis: stool osmolarity > expected osmolarity, Positive Hydrogen breath test, positive Clinitest of stool for reducing sugar Tx: Remove milk product from diet (symptoms resolve in 24-36 hrs) * вЂў вЂў вЂў вЂў вЂў вЂў Irritable Bowel Syndrome: Abdominal pain relieved by bowel movement Diarrhea alternating with constipation Tx: High вЂ“ fiber Diet. Diarrhea predominant в†’ loperamide / diphenoxylate AntiвЂ“spasmodic agent в†’ dicyclomine / hyoscyamine Alosetron в†’ Diarrhea predominant [restricted access in USA] Tricyclic Antidepressant в†’ resistant cases вЂў Tx of chronic constipation in child вЂ“ dietary modification; If it fails, use laxatives (milk of magnesia) S.S.Patel. All rights reserved. Registered with US copy right office 32 * вЂў вЂў вЂў вЂў вЂў вЂў Carcinoid Syndrome: tumors of the neuroendocrine syndrome Tip of vermiform appendix (most common site) but carcinoid tumors of terminal ileum most commonly metastasize (liver) and produce carcinoid syndrome Diarrhea, Flushing, Tachycardia and Hypotension Niacin deficiency (Serotonin & Niacin в†’ Tryptophan) Endocardial fibrosis , Tricuspid Regurgitation , Pulmonic stenosis Diagnosis : urinary 5 вЂ“ HIAA ( 5- hydroxyindolacetic acid ) Tx: Octreotide * вЂў вЂў вЂў вЂў вЂў вЂў Celiac Disease: Anti-gliadin, Anti-endomysial, Anti-transglutaminase antibodies Loss of intestinal villi (malabsorption вЂ“ diarrhea, abd distension, abd pain) Celiac disease affect PROXIMAL small bowel Function returns if patient is on gluten free diet Dermatitis herpetiformis в†’ strong association with celiac disease No wheat, rye, oat [contains gluten] * вЂў вЂў вЂў WhippleвЂ™s Disease: Tropheryma whippeli bacilli PASвЂ“positive macrophage obstruct lymphatic & reabsorption of chylomicrons Chronic diarrhea and weight loss Tx: Sulfamehoxazole / trimethoprim or Doxycyclin Г— 6 months вЂў Chronic diarrhea, WT loss, generalized lymphadenopathy but no risk factor for HIV пѓ Whipple diseases пѓ endoscopy with small bowel biopsy (PAS positive material in lamina propria) [HIV will always there in 5 choices to confuse] * вЂў вЂў вЂў вЂў вЂў Diverticulosis: Lack of fibers in the diet Right sided bleed / Left sided obstruct Most common cause of lower GI bleed. (Angiodysplasia вЂ“ 2nd MCC) Most common source of diverticular bleeding вЂ“ erosion of the artery Tx: Increase fibers in diet вЂў Patient with diverticulosis (showed on colonoscopy) present with active Bleeding & now stabilized after resuscitation, next step? labeled erythrocyte scintigraphy (to find source of bleeding) * вЂў вЂў Diverticulitis: (left sided appendicitis) CT scan (Best test) [Barium study & Endoscopy вЂ“ contraindicated] Tx: Ciprofloxacin and Metronidazole вЂў вЂў Sigmoid colon: most common site for diverticulosis, diverticulitis and polyp Rectosigmoid colon: most common site for colon CA S.S.Patel. All rights reserved. Registered with US copy right office 33 вЂў Volvulus of Sigmoid colon в†’ Elderly patient в†’ distended abdomen, similar episodes in past which resolve itself в†’ ParrotвЂ™s beak appearance (coffee bean sign / omega sign) of large gas shadow on X-ray в†’ Tx : Rigid sigmoidscopy вЂ“ Rectal tube в†’ bowel preparation & elective surgery п‚§ Ogilvie Syndrome в†’ Abdominal distension , without tenderness in elderly sedentary patient after surgery elsewhere in the body (but not Abdominal surgery) в†’ X-ray : distended colon with cut-off at splenic flexure в†’ Tx : colonoscopy (both diagnostic & therapeutic); if recur then do colonoscopy one more time; if still recur then start Neostigmine drip; Cecostomy is the last resort вЂў Mesenteric Ischemia в†’ Patient with h/o AF / atherosclerotic disease present with acute abdomen and low bicarbonate (pain out of proportion to physical findings like absent rebound tenderness, guarding, rigidity, etc) & blood in stool вЂў Mechanical Intestinal Obstruction в†’ Abdominal pain, constipation, distension & vomiting (cardinal features of obstruction) в†’ Adhesion / Indirect Inguinal Hernia Fever, leukocytosis, rebound tenderness in patient with indirect inguinal hernia suggest strangulation п‚§ Paralytic Ileus в†’ post-op-abdominal distension, without tenderness в†’ X-ray в†’ dilated small bowel, without air fluid level в†’ Absent bowel sound в†’ Tx : NPO and NG tube suctioning until peristalsis resumes п‚§ Post-op-Intestinal Obstruction: Adhesion в†’ X-ray: multiple air fluid level в†’ Barium tag: it will вЂњhangвЂќ somewhere if there is a mechanical obstruction в†’ Tx: Reoperation вЂў Meconium ileus в†’ cystic fibrosis в†’ ground glass appearance on abd x-ray в†’ Gastrografin enema (both diagnostic & therapeutic) * MeckleвЂ™s Diverticulum в†’ painless large bloody bowel movement in child (brick red stool) в†’ Technetium scan (99mTc scan) to identify ectopic gastric mucosa [rule of 2вЂ™s вЂ“ 2 ft from ileocecal valve, 2 inches long, 2 yrs of age, 2% of population; remnant of Vitelline duct (Omphalomesenteric duct)] вЂў Hirschsprung Disease (aganglionic megacolon) в†’ Rectal exam may lead to explosive expulsion of stool & flatus в†’ X-ray: distended proximal colon (normal) and вЂњnormal lookingвЂќ distal colon (aganglionic). вЂў Intussception в†’ sausage shaped mass on the right side of the abdomen, вЂњemptyвЂќ looking right lower quadrant (Dancing sign), вЂњcurrant jelly stoolвЂќ S.S.Patel. All rights reserved. Registered with US copy right office 34 вЂў п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ * вЂў вЂў вЂў вЂў * вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў Acute Abdomen: (rebound tenderness, guarding, rigidity) в†’ Exploratory laparotomy (1st step in management) [Medical treatment for Any Acute Abdomen в†’ NPO, NG suction, IV fluids] Classic presentation of Acute Appendicitis [pain start in mid epigastric region and then shifted to RLQ, positive rebound tenderness, Psoas sign, RovsingвЂ™s sign, etc], next step? в†’ Appendicectomy Above presentation, On Abdominal exploration, Appendix is normal but ileum is inflamed (CrohnвЂ™s ileitis), next step? в†’ Proceed with Appendicectomy and close the abdomen Above presentation, On Abdominal exploration, Appendix is normal but ileum & cecum are inflamed, next step? в†’ Do nothing and close the abdomen [when cecum is inflamed, Appendicular stump doesnвЂ™t heal and it can cause fecal fistula which leads a hemicolectomy] Female patient without classical presentation of appendicitis, next step? в†’ USG Classic presentation of appendicitis but 6-7 days old pain, mass on abdominal palpation, diagnosis? в†’ Appendicular mass, next step? в†’ IV fluid, bowel rest, IV antibiotics, serial examinations If above scenario, 24-hrs after starting treatment, patient is getting worse (spiking fever, tachycardia, increase in localize tenderness), next step? в†’ CT scan (Appendicular abscess) в†’ Tx : CT guided drainage Gastrointestinal Bleeding: First step в†’ Resuscitation / Treatment then find etiology FFP в†’ if PT is elevated Platelate transfusion в†’ if count < 50,000 / mm3 & Patient is acutely bleeding If h/o cirrhosis в†’ Octreotide Esophageal Varices в†’ Octreotide during acute episodes If still bleeding в†’ emergency endoscopy & place bands around the bleeding Varices If still bleeding в†’ TIPS (complication в†’ worsening of hepatic encephalopathy) Propranolol в†’ for prophylaxis of portal HTN BUN level is often elevated in patient with upper GI bleed. BUN level >40 in the presence of normal serum creatinine is very suggestive of upper GI bleed Diagnosis of GI bleed: Endoscopy (most accurate test) Nuclear bleeding scan Angiography Capsule endoscopy (to visualize small bowel) вЂў Angiodysplasia (vascular ectasia) вЂ“ Second most common cause of lower GI bleeding вЂ“ Aortic stenosis has been associated with Angiodyplasia * вЂў Colon Cancer: colonoscopy Hyperplastic polyp , Juvenile polyp , Peutz вЂ“Jeghers в†’ No malignant potential S.S.Patel. All rights reserved. Registered with US copy right office 35 вЂў Tubular polyp (most common neoplastic polyp) , villous polyp , Familial Polyposis , Turcot syndrome , Gardner syndrome в†’ malignant potential * вЂў вЂў вЂў Hereditary Non-Polyposis Syndrome (HNPCC) (Lynch Syndrome): Mis-match base repair defect Colonoscopy every 1-2 yrs start at age of 25 yrs Very high incidence of ovarian and endometrial cancer * вЂў вЂў вЂў Familial Adenomatous Polyposis: APC gene confers 100 % penetrance for the development of adenomas by the age of 35 & colon cancer by the age of 50 Flexible sigmoidoscopy every 1-2 yrs start at age of 12 As soon as polyps are found в†’ colectomy. * вЂў Cowden Syndrome: Hemartomas, rectal bleeding in a child * вЂў Gardner Syndrome: Colon CA + multiple soft вЂ“ tissue tumors (osteoma, lipoma, fibrosarcoma) * вЂў Turcot Syndrome: Colon CA + CNS malignancy * вЂў Peutz вЂ“ Jeghers Syndrome: Hemartomatous polyp + Hyperpigmented spots (lips, buccal mucosa, skin) вЂў Hemorrhoids / Anal fissure в†’ Proctosigmoidscopy [1st examination then Tx] [Any anorectal problem (even abscess) в†’ rule out cancer 1st by appropriate examination and then treatment] * Acute Pancreatitis: Mid epigastric pain classically radiates straight to the back. Amylase & lipase (most specific) are extremely elevated CT scan (Most accurate test) RansonвЂ™s criteria: within first 48-hrs [age(>55yrs), WBCs (>16,000), LDH (>350 IU/L), Blood sugar (>200 mg/dl), AST (>250 IU/L)] After 48-hrs [Po2 (<60 mmHg), Calcium (<8 mg/dl), BUN increases (>5mg/dl), Hematocrit decrease (>10%), Albumin (<3.2 mg/dl), estimated fluid deficit (> 4 L)] Billiary & Pancreatic ductal pathology в†’ ERCP (most accurate test) Tx: Supportive (NPO, IV fluids, etc). ERCP (sometimes) to remove stone in the pancreatic duct or to dilate a stricture h/o Acute Hemorrhagic pancreatitis + fever & leucocytosis в†’ Pancreatic Abscess в†’ Tx : Require Drainage Pseudopancreatic cyst of less than 6 weeks в†’ observe Pseudopancreatic cyst of greater than 6 weeks в†’ intervene оЂЊ оЂЊ оЂЊ оЂЊ оЂЊ оЂЊ оЂЊ оЂЊ оЂЊ S.S.Patel. All rights reserved. Registered with US copy right office 36 * вЂў вЂў вЂў вЂў вЂў вЂў оЂЊ * * вЂў вЂў Chronic Pancreatitis: Chronic abdominal pain in chronic alcoholics; h/o recurrent acute pancreatitis Calcification of pancreas on x-ray / CT scan (initial test) Stool elastase; Secretin Test в†’ low trypsin level (most accurate test) D-Xylose Test в†’ to differentiate b/w celiac disease / chronic pancreatitis If no Absorption of DвЂ“Xylose в†’ Celiac Disease Tx of Steatorrhea due to chronic pancreatitis вЂ“ diet modification (low fat diet); If it fails, give pancreatic enzyme supplements Pancreatic head cancer: Palpable gallbladder without significant tenderness Billirubin: Senescent RBC в†’ Heme в†’ unconjugated Bilirubin (lipid soluble вЂ“ can accumulate in tissue so large amt in blood can cause problem) в†’ bind with albumin and goes to liver в†’ conjugated in liver [water soluble вЂ“ easy for our body to excrete] в†’ secreted in bile в†’ 80% excreted in feces & 20% extrahepatic circulation [90% liver and 10% renal (in urine)] Jaundice: в†‘ unconjugated [more hemolysis, liver unable to pick up (Gilbert syndrome вЂ“ jaundice with fasting), liver unable to conjugate (Crigler-Najjar syndrome вЂ“ deficient enzyme)] в†‘ conjugated [liver unable to excrete in bile (Dubin-Johnson syndrome вЂ“ black liver) (OCP), в†“ extrahepatic bile flow (gall stone, CA of head of pancrease)] в†‘ Both [liver dysfunction (hepatitis)] Pruritus in Billiary disease is due to bile salt which deposits in skin For Jaundice, we test billirubin in urine with strip test not urobillinogen (UBG). UBG is normally present in urine. UBG is absent in obstructive jaundice but billirubin (conjugated вЂ“ water soluble) is present in urine in obstructive jaundice в– Neonatal Jaundice: - Within first 24-hrs вЂ“ Pathological [ABO incompatibility (most common cause), Rh incompatibility, Sepsis, Spherocytosis, G6PD, etc], >13 mg/dl (indirect / direct hyperbilirubinemia) - Between 24-36 hrs вЂ“ Physiological [resolved by 7-10 days, monitor bilirubin level to prevent Kernicterus (lethargy, irritability, hypotonic seizure) (rare)], rarely exceed 12.9вЂ“15 mg/dl (indirect hyperbilirubinemia) - After 1 week вЂ“ Breast milk [diagnosis of exclusion] - Tx: Phototherapy for indirect hyperbilirubinemia to prevent kernicterus вЂў Billiary Atresia в†’ Persistent, Progressively increasing jaundice in newborn в†’ HIDA scan after week of Phenobarbital в†’ If no bile reaches the duodenum after Phenobarbital stimulation в†’ surgery * вЂў вЂў вЂў вЂў S.S.Patel. Cirrhosis: Best initial test to assess liver cell damage вЂ“ serum Transaminases Liver biopsy (most specific test) AST/ALT - >2 is quite specific for alcoholic liver disease. (increase GGT is also seen in alcoholic liver disease) AST - >500 вЂ“ should suspect viral / toxins / ischemia (AST level in alcoholic liver problem is usually below 450) All rights reserved. Registered with US copy right office 37 вЂў вЂў Portal HTN, Esophageal Varices, Ascites, Peripheral edema, elevated PT, low Albumin, spider angioma, palmar erythema, asterixis Serum : Ascites albumin gradient ( SAAG ): SAAG > 1.1 в†’ portal HTN SAAG < 1.1 в†’ cancer, Infection вЂў Ascitic patient, already on furosemide, spironolactone, protein & salt restriction diet, has passed little urine, diagnosis? пѓ Hepato-renal syndrome; next step? пѓ careful volume loading and stop diuretics вЂў Respiratory distress due to abdominal distension in cirrhotic patient, next step? пѓ Paracentesis (diagnostic & therapeutic) вЂў Management of Ascitic patient: 1. Sodium and water restriction 2. Spironolactone [Anti-androgenic action helps decreasing effect of estrogen] 3. Loop diuretics (furosemide) 4. Frequent abdominal tapping * вЂў вЂў вЂў Spontaneous Bacterial Peritonitis (SBP): Culture of the fluid (most specific test) Total WBC > 500 / mm3 / > 250 /mm3 neutrophils в†’ Infection is present Cefotaxime / Ceftriaxone (drug of choice) вЂў Subacute bacterial peritonitis in Ascitic patient, next step? пѓ paracentesis; not diagnostic peritoneal lavage which is done in blunt abdominal trauma * вЂў вЂў вЂў вЂў Primary Billiary Cirrhosis: Anti-mitochondrial antibody Granulomatous destruction of bile ducts in portal triad Middle вЂ“ aged women, very less elevation of bilirubin, strong association with other auto-immune diseases в†’ Sjogren Syndrome, RA, Scleroderma Diagnosis: Transaminase are often normal в†‘в†‘ Alkaline Phosphatase & Оі - glutamyl transpeptidase в†‘ risk for hepatocellular carcinoma [HCC] Tx: Bile acid sequesters (cholestyramine, Ursodeoxycholic acid), UV light for Pruritus Ursodeoxycholic acid slows the progression of Primary Billiary Cirrhosis (PBC). * вЂў вЂў вЂў вЂў вЂў Primary Sclerosing Cholangitis: Obliterative fibrosis of intrahepatic & extrahepatic bile ducts Strong association with Ulcerative colitis Sx вЂ“ same as primary billiary cirrhosis ( Pruritus; etc) Anti-mitochondrial Antibody в†’ negative в†‘ risk for cholangiocarcinoma (CA of bile duct) вЂў вЂў вЂў S.S.Patel. All rights reserved. Registered with US copy right office 38 * вЂў вЂў вЂў вЂў вЂў вЂў * вЂў вЂў вЂў Hemochromatosis: Most common inherited genetic disease Over absorption of iron [Ferritin (major storage protein) store iron in macrophage in bone marrow and hepatocytes, circulate in small amt in serum (в†“ in iron deficiency anemia); Hemosiderin degradation product of Ferritin in cell, (doesnвЂ™t circulate) golden brown granules in tissue & blue with Prussian blue] Intracellular iron produce hydroxyl ions which damage parenchymal cells Cirrhosis, Restrictive cardiomyopathy, Arthralgia, skin hyperpigmentation, diabetes, Hypogonadism в†‘ infection with Vibrio vulnificus, Yersinia & L.monocytogens Most appropriate treatment of Hemochromatosis вЂ“ therapeutic phlebotomy Wilson Disease: Autosomal recessive disease в†“ copper transport into bile and в†“ ceruloplasmin synthesis leads to в†“ excretion of copper from body & в†‘ free Cu +2 in the body which deposited in various tissue and produce damage Basal ganglia dysfunction (choreoathetoid movements), Kayser-Fleischer ring (Slit вЂ“ lamp Examination), Fanconi Syndrome вЂў Ruptured Hepatic Adenoma в†’ young woman on birth control pills present with abdominal pain, low hemoglobin, hypovolemic shock в†’ CT Scan в†’ Surgical removal вЂў Amebic liver abscess в†’ h/o Travel to Mexico в†’ jaundice, weight loss, right upper quadrant pain, diarrhea в†’ Serology for amebic titers в†’ Metronidazole ( if not cured with Metronidazole в†’ Aspiration ) вЂў Liver Problem в†’ в†‘в†‘в†‘ transaminase Billiary Problem в†’ в†‘в†‘в†‘ Alkaline Phosphatase Alcoholic liver Problem в†’ в†‘в†‘в†‘ GGT (ОівЂ“glutamyl transferase ) * * Choledochal cyst вЂ“ congenital benign dilatation of bile ducts CaroliвЂ™s Syndrome вЂ“ congenital cystic dilatation of the intrahepatic biliary tree вЂ“ associated with polycystic kidney disease вЂ“ Cholangitis & Cholangio CA * вЂў вЂў вЂў Gall stone (Cholelithiasis): Cholesterol (80%) (radiolucent) - в†‘cholesterol in bile and в†“bile salt & lecithin Pigment stone (20%) (radio-opaque) вЂ“ calcium bilirubinate [Sickle cell anemia] Billiary colic в†’ colicky right upper quadrant pain, radiate to the right shoulder, often aggravated after ingestion of fatty food / Anti-cholinergic drug в†’ USG [presence of gall stones, no thickening of GB wall] Acute Cholecystitis в†’ Female , Forty , Fertile , Fatty в†’ colicky right upper quadrant pain, radiate to the right shoulder, often aggravated after ingestion of fatty food в†’ USG [presence of gall stones, thickening of GB wall, pericholecystic fluid] вЂў S.S.Patel. All rights reserved. Registered with US copy right office 39 Л— Typical acute Cholecystitis presentation but negative USG в†’ HIDA scan Л— Tx : If patient present within 3 days of onset of pain в†’ Emergent laparoscopic cholecystectomy; If patient present after 3 days of onset of pain в†’ medical treatment as of acute abdomen followed by elective cholecystectomy; but if medical treatment fail в†’ Emergent open laparotomy Л— Air in GB wall on X-ray в†’ emergent laparotomy Л— Air in GB, stone in terminal ileum (small bowel obstruction) в†’ GB-enteric fistula Л— Air within Billiary tree after ERCP в†’ billiary enteric fistula Л— Any patient with symptomatic gall stones / gall stones complications (Pancreatitis, Cholangitis, Obstructive jaundice) should not go home without cholecystectomy Л— Post-cholecystectomy pain в†’ sphincter of oddi dysfunction, CBD stone or functional pain вЂў Obstructive Jaundice в†’ USG (Best next / 1st step in management) Л— if it is due to gall stone в†’ ERCP (remove gall stone) then laparoscopic cholecystectomy Л— If it is due to CA (wt. Loss, Asymptomatic Jaundice) в†’ CT scan в†’ Percutaneous biopsy в†’ Tx Л— If it is due to CA (wt. Loss, Asymptomatic Jaundice) в†’ CT scan (negative) в†’ ERCP в†’ Tx * вЂў Gallbladder adenocarcinoma: Risk factors вЂ“ cholelithiasis, porcelain gallbladder [calcification of GB wall] п‚§ Infant on milk formula вЂ“ necrotizing enterocolitis вЂ“ transmural necrosis пЃ® - Reye Syndrome: Encephalopathy and microvesicular steatosis in Liver Recent viral URI, [Varicella, Influenza + Aspirin use]. Ammonia, Transaminases are markedly elevated Liver biopsy в†’ non-inflammatory fatty infiltration, mitochondrial injury вЂў Hep A vaccine should be given to all unimmunized patient with underlying chronic liver disease Almost all patients with Hep C infection who undergo transplantation have a documented recurrence of Hep C infection in the transplant liver. Tx of acute Hep B (patient already have symptoms) вЂ“ supportive care Tx (prophylaxis) of Hep B exposure (patient has exposed but doesnвЂ™t have symptoms, eg. Needle prick) вЂ“ Immunoglobulin + Hep B vaccine вЂў вЂў вЂў S.S.Patel. All rights reserved. Registered with US copy right office 40 HEMATOLOGY * Anemia: low Hb (<13 in M & <12 in F) / low hematocrit (<40 in M & <37 in F) в†“ Next step: determine MCV Microcytic ( MCV < 80 ) Normocytic Macrocytic ( MCV > 96 ) вЂў Iron deficiency вЂў Hemolytic anemia вЂў B12 deficiency вЂў Thalasemia вЂў ACD вЂў Folate deficiency вЂў Sideroblastic Next step? вЂ“ Reticulocyte count в†‘ Reticulocyte count Hemolysis в†“ Reticulocytes вЂ“ bone marrow failure вЂў Lead poisoning вЂў Anemia of chronic diseases ( ACD ) * MCHC : Mean corpuscular Hb concentration (Avg. Hb concentration in RBC) в†“ MCHC в†’ central area of pallor вЂ“ Microcytic в†‘ MCHC в†’ No central area of pallor вЂ“ Spherocytosis N MCHC в†’ Megaloblastic Anemia вЂў вЂў Hb A - О± , ОІ Hb F - О± ,Оі Hb A2 - О± , Оґ Daily requirement of Iron вЂ“ 1 mg/day in M, 2-3 mg/day in F * вЂў вЂў вЂў Iron Deficiency Anemia: Low serum ferritin, serum iron , high TIBC Blood loss (menstruation), dietary deficiency Most specific test: Bone marrow biopsy * вЂў вЂў вЂў ACD (Anemia of Chronic Diseases): Normal / elevated serum ferritin Both serum iron & TIBC в†’ Low In treatment of ACD, if erythropoietin level is normal, then periodic blood transfusion is the best choice to improve anemia * вЂў вЂў вЂў вЂў Sideroblastic Anemia: Normal serum ferritin Very high transferrin saturation High serum iron & Low TIBC Most specific test: Prussian Blue stain of RBC in the marrow will show ringed sideroblast Vit-B6 deficiency: в†“ Protoporphyrin & в†“ Оґ ALA вЂў S.S.Patel. All rights reserved. Registered with US copy right office 41 вЂў вЂў Iron deficiency: в†‘ Protoporphyrin & N Оґ ALA Lead poisoning: в†‘ Protoporphyrin & в†‘ Оґ ALA * вЂў вЂў вЂў Thalassemia: Underproduction of alpha/beta globin chain very low MCV, very low RBC count compare to iron deficiency Target cells, Normal serum iron & RDW Electrophoresis пЈ¦ в†“ Normal в†“ О± - Thalassemia trait в†“ All Hb requires О± chain for synthesis So Hb concentration decrease but Relative proportion of the normal Hb remains the same в†“ Abnormal в†“ ОІ - Thalassemia в†“ в†‘ Hb A2 в†‘ Hb F - Thalassemia traits of both О± & ОІ do not require specific treatment Thalassemia major ( ОІ ) requires periodic blood transfusion Tx of Iron overload (due to periodic blood transfusion) в†’ Deferoxamine п‚§ вЂњCrew haircutвЂќ on skull X-ray is distinctive radiological change seen most often in patient with Sickle cell anemia & Thalassemia major. в™¦ Macrocytic Anemia в™¦ пЈ¦ в†“ в†“ Vit вЂ“ B12 deficiency Folic acid deficiency вЂў Peripheral Blood smear: Hypersegmented Neutrophils seen in Both вЂў How to differentiate: Low B12 level в†‘ Methylmalonic acid level is seen in only B12 вЂў вЂў вЂў вЂў вЂў вЂў S.S.Patel. Low RBC folic acid level Schilling Test is occasionally used to determine etiology of B12 def Schilling Test: Oral Administration of Radioactive Vit вЂ“ B12 Reabsorption в†’ Pure vegan B12 + IF в†’ Reabsorption в†’ Pernicious Anemia B12 + Antibiotics в†’ Reabsorption в†’ Bacterial over growth B12 + Pancreatic extract в†’ Reabsorption в†’ chronic Pancreatitis. All rights reserved. Registered with US copy right office 42 вЂў вЂў вЂў Important: Vit-B12 and folic acid require for DNA synthesis in all cells so their deficiency affect all bone marrow cells, not just RBC. Treatment : Vit вЂ“ B12 IM Folic acid orally Diamond-Blackfan Syndrome (congenital hypoplastic anemia) вЂ“ intrinsic defect of erythroid progenitor cells which results in increased apoptosis вЂ“ megaloblastic anemia without hypersegmented neutrophils [In Vit-B12 and Folic acid deficiency, megaloblastic anemia with hypersegmented neutrophils] в†“ Extravascular hemolysis - в†‘ Unconjugated bilirubin - в†‘ LDH - Spherocytosis - Sickle-cell - Immune-hemolytic anemia Hemolytic Anemia в†“ в†“ Intravascular Hemolysis - в†‘plasma & urine Hb - Hemoglobinuria - Hemosiderinuria - в†“Serum Haptoglobin - в†‘ LDH - PNH (Paroxysmal Nocturnal Hemoglobinuria) - G6PD в– Sickle- cell-Anemia: Autosomal-recessive - Substitution of Valine for glutamic acid at sixth position on ОІ- globin chain - Sickle-cell trait в†’ Asymptomatic. Significant manifestation of trait is the renal concentrating defect - Screening test: Sickle prep test to diagnose traits - Most specific test: Hemoglobin electrophoresis - Treatment: Acute crisis в†’ fluid, Analgesics, O2 - Fever & leucocytosis with/without infection в†’ Antibiotics в‡’ ceftriaxone, cefotaxime - Severe (Acute chest syndrome, CNS, priapism) в†’ RBC transfusion - Hydroxyurea в€’Prophylaxis в€’ prevent vaso occlusive crisis - Folic acid supplementation вЂ“ prevent Aplastic anemia - Most common complication of Sickle cell disease вЂ“ splenic sequestration (not splenic infarction) вЂў Sudden fall in Hb concentration and virtual absence of reticulocytes on the peripheral blood smear in sickle cell disease patient вЂ“ Parvo virus B-19 (slapped cheek appearance) пѓ Aplastic crisis в– S.S.Patel. Hereditary Spherocytosis: Autosomal dominant RBC membrane protein defect [spectrin, ankyrin] Osmotic Fragility test & в†‘ MCHC Treatment: Splenectomy All rights reserved. Registered with US copy right office 43 в– - Autoimmune Hemolytic Anemia: CoombвЂ™s test Warm вЂ“ antibody (IgG), Cold вЂ“ antibody (IgM) Drug вЂ“ induced в†’ Penicillin, Quinidine, О± - Methyldopa в– - Paroxysmal Nocturnal Hemoglobinuria (PNH): Loss of anchor for DAF ( Decay Accelerating Factor ) more complements bind to RBC & intravascular hemolysis occur Presentation: Hemoglobinuria in first morning urine Sign / Symptoms of major venous structure thrombosis Treatment: Corticosteroid in severe disease for unclear reason Anticoagulation for thrombosis вЂў в– - G6PD deficiency: в†“ Synthesis of NADPH & GSH (glutathione) X-linked recessive Hemolysis in the presence of Oxidant Stress Oxidant Stress в†’ Infection , fevabeans, Drugs (Sulfa, Dapsone, Primaquine, Quinidine, Nitrofurantoin, INH) Diagnosis в†’ Heinz bodies, bite cells, G6PD level Treatment в†’ Avoid oxidant stress в– Pyruvate kinase deficiency: в†“ Synthesis of ATP - Autosomal recessive - PK gives 2 ATPs вЂ“ its deficiency produce membrane damage вЂ“ RBC with thorny projection (echinocytes) в™¦ Leukemia в™¦ ALL AML CML вЂў Children вЂў 15-39 yrs вЂў 40-60 yrs ( < 14 yrs ) вЂў Thrombocytopenia вЂў Thrombocytopenia вЂў Thrombocytopenia Thrombocytosis in 40% вЂў Pre-B cells вЂў t (15; 17) in M3 вЂў Philadelphia CALLA, CD10 & chromosome, TdT positive t (9 ; 22) вЂў T cells - CD10 & вЂў DIC in M3 вЂў Bcr-Abl fusion вЂ“ TdT negative в†‘Abl kinase activity вЂў t (12; 21) вЂ“ good вЂў Auer rods in M2 & prognosis M3 вЂў CNS in M4 & M5 вЂў Basophillia CALLA (common M3- Premyelocytic ALL antigen) M4-myelomonocytic S.S.Patel. All rights reserved. CLL вЂў > 60 yrs вЂў Thrombocytopenia вЂў CD19 Antigen вЂў Predominantly B lymphocytes. вЂў вЂњSmudge cellsвЂќ вЂў Stages : 0 вЂ“lymphocytosis 1-lymphadenopathy Registered with US copy right office 44 M5- monocytic вЂў VitвЂ“A вЂ“ useful in Premyelocytic ( M3) * 2-spleenomegaly 3-Anemia 4-thrombocytopenia Diagnosis: Bone marrow biopsy в†’ > 30 % blast в†’ Acute leukemia < 30 % blast в†’ Chronic leukemia Treatment Treatment вЂў Daunorubicin вЂў Cytosine arabinoside вЂў daunorubicin / Idarubicin вЂў Vincristine вЂў Prednisone вЂў Vit-A in Premyelocytic (M3) Treatment Treatment вЂў Imatinib вЂў Stage 0, 1 в†’ No Treatment вЂў if it fails, then вЂў Advanced stage Bone marrow в†“ Fludarabine вЂў Imatinib: вЂў Autoimmune selective inhibitor of hemolysis & Bcr-Abl tyrosine Thrombocytopenia kinase в†“ Prednisone вЂў Asparaginase вЂў Intrathecal Methotraxate for CNS Prophylaxis * - Adult T-cell lymphoma: HTLV-1 [Human T-cell leukemia virus вЂ“ 1] Activation of TAX gene вЂ“ inhibits TP53 suppressor gene в†‘ CD4 T-cells Skin infiltration and lytic bone lesion [lymphoblast release osteoclast activating factor] (hypercalcemia) Negative TdT * - Hairy cell leukemia: only leukemia without lymphadenopathy B-cell leukemia Positive TRAP stain (tartrate-resistant acid phosphate) Treatment: Pentostatin / 2-Chlorohydroxy adenosine * Infectious Mononucleosis: EBV вЂ“ CD21 receptor on B-cells вЂ“ heterophile antibody (IgM to sheepвЂ™s RBC) вЂ“ danger of rupture of spleen вЂ“ When patient receive ampicillin, Ampicillin-associated maculopapular rash is well known phenomenon in patient with mononucleosis [Tx: discontinue drug, supportive treatment and observation] * Sudden increase in WBC in patient with severe infection or inflammation with high leukocyte alkaline phosphatase score, diagnosis? пѓ Leukemoid reaction S.S.Patel. All rights reserved. Registered with US copy right office 45 * Myeloid stem cells: RBC, granulocytes, mast cells and platelets вЂ“ Polycythemia vera affect myeloid stem cells so increase everything in Polycythemia vera вЂ“ myelofibosis (tear drop cells, extramedullary hematopoesis) * Aplastic Anemia: Pancytopenia [Everything is decreased including RBCs, Platelets and WBCs] Л— Bone marrow biopsy вЂ“ most specific test Л— Treatment вЂ“ Allogenic bone marrow transplant (best treatment for < 50 yrs old) вЂ“ Antithymocyte globulin, cyclosporine, Prednisone * * * * Relative Polycythemia вЂ“ increased RBCs count due to decrease plasma volume Appropriate absolute Polycythemia (secondary) вЂ“ increase RBCs count due to increase in erythropoietin (EPO) level due to hypoxia [eg. COPD, high altitude]; next step would be measure oxygen saturation (decrease Sao2) in these patients Inappropriate absolute Polycythemia (Polycythemia vera and ectopic EPO secretion) вЂ“ Polycythemia vera вЂ“ clonal expansion of the trilineage myeloid stem cells [increase RBCs, Granulocytes, Mast cells and Platelets]; decrease EPO (increase Sao2 inhibits EPO secretion) Ectopic EPO secretion вЂ“ Renal cell CA, Hepatocellular CA вЂ“ increase RBCs and increase EPO, normal Sao2 and plasma volume Neonatal Polycythemia вЂ“ hematocrit >65% - If increase on hematocrit on heel prick sample, next step? вЂ“ Recheck sample from peripheral blood (hematocrit 515% lower than heel prick) вЂ“ If symptomatic (hypoglycemia, hyperbilirrubinemia, cardiac or respiratory compromise), next step? вЂ“ hydration and partial exchange transfusion в™¦ Plasma Cell Disorder в™¦ Multiple Myeloma Monoclonal Gammopathy of Uncertain Significance вЂў Bone pain , Infection, Anemia , Renal failure вЂў Electrophoresis вЂ“ IgG monoclonal spike вЂў No systemic manifestation like multiple myeloma вЂў IgG monoclonal spike on electrophoresis вЂў X-ray вЂ“ punched out lytic lesion (osteoclast activating factor) вЂў Hypercalcemia вЂў Bence-Jones Protein (Acidification of urine is required to test BJ protein) вЂў Bone marrow biopsy (most specific) вЂ“ >10% plasma cells вЂў First step вЂ“ serum protein electrophoresis, plain radiographic skeletal survey & assessment of renal function вЂў No lytic bone lesion on X-ray S.S.Patel. All rights reserved. вЂў Normal lab test (creatinine, calcium) вЂў Bone marrow - < 5 % Plasma cells Registered with US copy right office 46 вЂў Whole body X-ray should be done once a patient has been diagnosed with multiple myeloma. Bone scans are not useful for these patients. в– Treatment - Younger в†’ Bonemarrow transplant Older в†’ Melphalan & Prednisone Hypercalcemia в†’ Hydration & loop diuretics and then with Bisphophonates * WaldenstromвЂ™s macroglobulinemia (Lymphoplasmatic lymphoma): M spike with IgM, BJ protein вЂ“ no lytic lesions like multiple myeloma п‚§ BJ proteins вЂ“ kappa or lambda light chains в™¦ Lymphomas в™¦ * Stage of Lymphoma (HodgkinвЂ™s & NonвЂ“HodgkinвЂ™s): Л— A вЂ“ Constitutional Symptoms Absent Л— B вЂ“ Constitutional Symptoms Present (fatigue, Wt .loss, night sweat, etc.) пЃ® Stages: 1-Single lymphnode / single extralymphatic organ one side of 2-Two / more lymphnode / contiguous extralymphatic organ diaphragm 3-Involvement of lymphnode/extralymphatic organ 4-Multiple disease foci in extralymphatic organ (eg. liver, Bone marrow) S.S.Patel. All rights reserved. both side of the diaphragm Registered with US copy right office 47 Hodgkin Lymphoma вЂў Reed-Sternberg cells Non-Hodgkin Lymphoma вЂў Reed-Sternberg cell Absent вЂў Lymphadenopathy is more common (cervical, Supraclavicular, Axillary) вЂў B-cells lineage involve вЂў Extralymphatic involvement is more common (Spleen, liver, stomach) вЂў Both B & T cell lineages involve вЂў Lymphocyte Predominant (Best Prognosis) вЂў HIV, EBV в†’ Burkitt lymphoma, t (8;14 ) вЂў Mixed celluarity вЂў H.pylori в†’ gastric lymphoma (mucosa associated lymphoid tissues in stomach) вЂў Nodular Sclerosing (Female) в†’ lacunar cells вЂў CNS involvement is more common in HIVpositive patient вЂў Lymphocyte depletion (worst prognosis) вЂў Follicular lymphoma вЂ“ t (14;18 ), over expression of BCL2 anti-apoptosis gene вЂў RS cells вЂ“ CD15, CD 30 positive вЂў Sjogren syndrome вЂ“ salivary gland & GI - B-lymphocyte with somatic hypermutation lymphoma вЂў HashimotoвЂ™s thyroidits вЂ“ thyroid malignant lymphoma вЂў Diagnosis: Excisional lymphnode biopsy вЂў Diagnosis: Excisional lymphnode biopsy Next step в†’ determine the extent of disease by Next step в†’ Staging CXR, Chest CT, Abdominal CT/MRI вЂў Bone marrow biopsy is best initial staging tool вЂў Staging laparotomy for NHL вЂў Bone marrow biopsy вЂў Treatment вЂў Treatment : same as Hodgkin for stages IA , II A, CNS lymphoma пЈ¦ в†“ в†“ вЂў B, III , IV в†’ Chemotherapy I A , II A B , III , IV в†“ Cyclophophamide в†“ в†“ Radiation Chemotherapy Hydroxy вЂ“ Adriamycin Oncovin [vincristine] Adriamycin(doxorubicin) Bleomycin Prednisone Vinblastine вЂў CD 20 antigen expression в†’RITUXIMAB Dacarbazine п‚§ Mycosis fungoides вЂ“ cutaneous (begins in skin) T-cell lymphoma (not a fungal infection) в™¦ Bleeding Disorders в™¦ вЂў вЂў Tissue thromboplastin в†’ Factor 7 в†’ Extrinsic Pathway (PT) в†’ Warfarin Subendothelial collagen, HMWK в†’ Factor 12 в†’ Intrinsic Pathway (PTT) в†’ Heparin Common final pathway в†’ Factor 10 , 5 , 2 , 1 [ 2-Prothrombin ] Heparin в†’ вЉ• AT III в†’ neutralize 9 , 10 , 11 , 12 , Prothrombin & thrombin вЂў вЂў S.S.Patel. All rights reserved. Registered with US copy right office 48 вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў Thrombin в†’ convert fibrinogen into fibrin monomers [fibrin monomers then aggregate which are soluble] Thrombin в†’ activate fibrin stabilizing factor (13) [once fibrin monomers aggregate, factor-13 stabilize them by making them insoluble] Plasmin в†’ cleaves insoluble fibrin monomers and fibrinogen into fibrin degradation products [FDP] D-Dimers в†’ fragments of cross-linked insoluble fibrin monomers Protein C & S ( Vit .K dependent ) в†’ inactivate 5 & 8 в†’ enhance fibrinolysis tPA вЂ“ synthesized by endothelial cell , TxA2 вЂ“synthesized by Platelates n VWF вЂ“ synthesized by endothelial cell & Platelates вЂ“ Func. в†’ Platelate Adhesion & Prevent degradation of factor VIII:C Platelate Storage в†’ VWF & Fibrinogen (1) Platelate receptors в†’ glycoprotein (gp) 1b вЂ“ VWF ; GP2b:3a вЂ“ Fibrinogen Platelate Factors в†’ PF3 в†’ Prothrombin complex (V, Xa, PF3, Ca+2). PF4 в†’ Heparin neutralizing factor в– Hemostasis in small vessel injury: injury в†’ tissue thromboplastin (activates extrinsic pathway) & exposed collagen (activates intrinsic pathway) в†’ Endothelial cells synthesize vWF so injury makes it expose to platelets and platelets start attaching to them and ADP from platelets help aggregating them (temporary plug) в†’ platelets have fibrinogen at gp2b:3a в†’ activated thrombin (by intrinsic & extrinsic pathway) convert fibrinogen to fibrin monomers which aggregate and make soluble plug в†’ thrombin also activate factor-13 which convert soluble plug into insoluble plug в†’ bleeding stop в†’ tPA activates plasmin which dissolve fibrin monomer & blood flow reestablish to the tissue Idiopathic Thrombocytopenic Purpura: Sign of bleeding from superficial areas of body Absent spleenomegaly, Prolong Bleeding time Idiopathic Antibody (IgG) Production to the Platelets receptors (gp2b:3a) Diagnosis : Anti-platelate Antibody Bone-marrow в†’ megakaryocytosis вЂ“ indicate problem with platelate destruction, not with production * Treatment: Prednisone (initial therapy) Л— Corticosteroid should be given if platelets count <30,000/ mm3, otherwise observe patient Л— If count fails to raise after Prednisone therapy в†’ IV Immunoglobulin / Rhogam Л— Platelate transfusion (if above fails to raise count) в– - пЃ® Thrombotic Thrombocytopenic Purpura (TTP) вЂ“ deficiency in vWF cleaving metalloprotease in endothelial cells leads to в†‘в†‘vWF в†’ more platelets attach to vWF leads to thrombosis and thrombocytopenia (due to platelet consumption in thrombosis) вЂ“ microangiopathic anemia, Renal & CNS involvement вЂ“ schistocytes (fragmented RBCs), Helmet-shaped cells S.S.Patel. All rights reserved. Registered with US copy right office 49 пЃ® Hemolytic Uremic Syndrome (HUS) вЂ“ microangiopathic anemia, thrombocytopenia and renal involvement [no CNS involvement] п‚§ Schistocytes вЂ“ TTP, DIC, Aortic stenosis в– Giant platelates, greater than expected bleeding for the degree of thrombocytopenia, Normal vWF level, subnormal ristocetin assay, diagnosis? пѓ Bernal-Soulier Syndrome; defect? пѓ glycoprotein Ib в– - Von Willebran Disease ( VWD ): Autosomal вЂ“ Dominant Sign of bleeding from superficial areas of body Low level of VWF , factor VIII : C Ristocetin Platelate Aggregation Test : Abnormal Elevated PTT, Normal PT Treatment: Desmopressin Acetate, VWF replacement в– - Hemophilia: Autosomal вЂ“ recessive Hemophilia A вЂ“ factor 8 deficiency, Hemophilia B вЂ“ factor 9 deficiency Sign of deep bleeding в†’ hemarthrosis, hematoma, GI bleeding may become apparent at the time of circumcision Diagnosis : elevated PTT , Normal PT вЂњMixing StudyвЂќ : 50 % PatientвЂ™s blood + 50 % Normal blood в†’ PTT corrected в†’ Hemophilic в†’ If PTT is not corrected в†’ Antibody inhibition of the factor Treatment: Desmopressin, Specific factor replacement в– - VitвЂ“K Deficiency: в†“ Production of Factor 2, 7, 9, 10 Both PT & PTT are elevated Diagnosis : correction of PT & PTT after giving Vit-K Treatment: Fresh Frozen Plasma in severe bleeding в– - Liver Disease : в†“ Production of All factors except VWF & Factor 8 Both PT & PTT are elevated Diagnosis: H/O liver disease & No correction of PT & PTT after giving Vit-K Treatment : Fresh Frozen Plasma в– DIC (Disseminated Intravascular Coagulation): - Platelates, в†‘в†‘ PT & PTT, в†‘ D- dimmers & FDPS, Low fibrinogen level schiztocytes on peripheral blood smear - Treatment: Fresh Frozen Plasma, Platelate transfusion пЃ® Л— Л— Л— S.S.Patel. SLE: Anti-phospholipids antibody, false VDRL/RPR positive Elevated PTT, all other parameters normal, h/o recurrent abortion Tx: Heparin & Aspirin All rights reserved. Registered with US copy right office 50 INFECTIOUS DISEASES * Best Initial Antibiotics for different Organisms : п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ S.S.Patel. Staph Aureus: Dicloxacillin, Oxacillin [Penicillins] / Cefadroxil, Cefalaxin [1st generation Cephalosporins] If patient is allergic to above groups вЂ“ Macrolide, newer fluoroquinolone If patient has MRSA вЂ“ Vancomycin / Linezolid Streptococcus: Penicillin (if sensitive) / Ceftriaxone / levofloxacin Strep Pneumonia: Penicillin G / Ceftriaxone / levofloxacin Strep Viridans: Penicillin G / Ceftriaxone Strep Pyogens: Ampicillin / Ampicillin + Sulbactam Strep Meningitis: Ceftriaxone Listeria Monocytogens: Ampicillin Legionella Pneumonia: Erythromycin Rickettsia in children: Chloramphenicol, Erythromycin Rickettsia in adults: Doxycycline Lyme disease in children < 9yrs of age: Amoxicillin Lyme disease in children >9 yrs of age and adults: Doxycycline Lyme disease in Pregnant women: Amoxicillin Disseminated Lyme disease [BellвЂ™s palsy, Cardiac involvement, CNS involvement]: Ceftriaxone Syphilis: Penicillin G Gonococcus: Ceftriaxone Chlamydia, Mycoplasma: Macrolides / Doxycycline C.Jejunii: Erythromycin H Influenzae: 2nd or 3rd generation cephalosporin E coli: Ciprofloxacin / Ampicillin Pseudomonas: Piperacillin, Ticarcillin [Anti-pseudomonal penicillin] Klebsiella: 2nd or 3rd generation cephalosporin Cryptococcus: Amphotericin B (severe), Fluconazole (prophylaxis) Candida: Fluconazole Dermatophytes: Terfinabine (oral) / Meconazole (local) PCP: Trimethoprim + Sulfamethoxazole Actinomycetes: Penicillin Nocardia: Sulfonamides Anaerobes: Metronidazole / Clindamycin Penicillin and Aminoglycosides have synergistic effects so combination of both (Penicillin + Aminoglycosides) is used in Enterobacteraceae and Pseudomonas infections All rights reserved. Registered with US copy right office 51 пЃ® Meningitis: infection of covering of brain вЂ“ fever, headache, stiff neck and focal neurologic deficiet вЂў Causes: Л— New born (< 1 month ) в†’Group B Streptococci, E.coli, L. monocytogen Л— 1 month вЂ“ 18 yrs Old в†’ N.meningitides Л— >18 yrs Old в†’ Strep Pneumoniae Л— Staph Aureus в†’ recent neurosurgery Л— L.monocytogens в†’ Immunocompromised (Neonates & elderly Patients) Л— Cryptococcus в†’ HIV positive, CD4+ < 100 cells Л— RMSF в†’ rash on wrist, ankle в†’ spread towards body Л— Neisseria в†’ Petechial rash Л— Cause of viral meningitis in pediatric population in US вЂ“ Arbovirus and Enterovirus Л— CN вЂ“ 8 deficits is more common long-term neurological deficit вЂў Sign & Symptoms of meningitis but appear less toxic вЂ“ Aseptic (Viral) meningitis [ECHO virus вЂ“ belongs to enterovirus вЂ“ Picornavirus family] вЂў вЂў вЂў вЂў вЂў Management: Л— Lumbar Puncture (Next Best Step / First step in management) Л— CT scan of the Head (If Papilledema, focal motor deficit, confusion, coma) then Lumbar puncture (LP) Л— Culture of the CSF (most accurate test) Л— Lyme , RMSF , Syphilis в†’ Serologic test Л— Cryptococcus в†’ India ink test, cryptococcal antigen test CSF findings of Bacterial Meningitis вЂ“ low glucose (<40 mg/dl), increase WBC count [Neutrophils] CSF findings of Viral Meningitis вЂ“ increase WBC (lymphocytes), normal glucose CSF findings of Cryptococcal Meningitis вЂ“ low WBC count (<50 cells/L) (Lymphocytes) , low glucose Treatment: Л— Ceftriaxone (Best initial empiric therapy) Л— Ceftriaxone + Ampicillin ( if L.monocytogens is suspected) Л— Amphotericin B (Best initial therapy for Cryptococcus) Л— RMSF в†’ Chloramphenicol, Erythromycin (children) / Doxycycline (Adults) Л— Tx of tubercular meningitis вЂ“ at least 12 months пЃ® Encephalitis: infection of parenchyma of brain вЂ“ fever, headache, stiff neck and altered mental status вЂў Causes: Л— HSV (temporal lobe) (most common) вЂў Management: Л— Lumbar Puncture (Next Best Step / First step in management) S.S.Patel. All rights reserved. Registered with US copy right office 52 вЂў * вЂў вЂў вЂў вЂў вЂў * * * вЂў вЂў вЂў вЂў вЂў * * оЂЊ оЂЊ оЂЊ оЂЊ оЂЊ оЂЉ оЂЉ S.S.Patel. Л— CT scan / MRI of the Head Л— PCR for HSV Treatment: Л— IV Acyclovir for HSV Л— Ganciclovir / Foscarnet for CMV Brain Abscess: Headache, fever, focal neurologic deficit CT scan with contrast (Best initial test) Biopsy of the lesion + gram stain + culture (most accurate test) Treatment: HIV positive в†’ Toxoplasmosis / Lymphoma (90% of cases) в†’ Pyrimethamine + Sulfadiazine for 14 days followed by CT scan to check lesion regress or not. If not regress, most probably lymphoma (Tx: radiation) Combination of Antibiotics (gram (+), gram(-), Anaerobes) Transverse Myelitis вЂ“ rapidly progressing lower extremity weakness following URI, accompanied by sensory loss and urinary retention вЂ“ Dx: MRI Epidural abscess вЂ“ patient with h/o IV drug abuse Otitis Media: Strep. Pneumoniae (35-40 %) , H. influenzae (25-30%),Moraxella(15-20%) Earache, fever, decrease hearing, red, bulging tympanic membrane with loss of light reflex, immobility of the membrane on insufflations of the ear with air Amoxicillin (Best initial therapy) / Amoxicillin + clavulanate (if recent use of Amoxicillin) Azithromycin / newer fluoroquinolone (Alternatives) Complications: The most common complication after an episode of otitis media is another episode of otitis media вЂ“ Acute mastoditis [pinna displaced inferior & laterally вЂ“ X-ray of skull, mastoid вЂ“ IV antibiotics + surgical debridement (mastodectomy may require)] Middle ear effusion [A normal appearing tympanic membrane with decrease movement of pneumatic otoscopy in patient treated for recent acute otitis media вЂ“ follow up after 4-6 wks; If not resolved, tympanostomy may require; referral to ENT] Malignant Otitis Externa: Diabetes Mellitus + foul smelling ear discharge & granulation пѓ Pseudomonas Tx: IV Ciprofloxacin Sinusitis: Headache (worse on leaning forward), facial pain, nasal discharge Maxillary sinus x-ray (best initial test) Uncomplicated в†’ decongestant , Analgesic Complicated (discolored nasal discharge) в†’ Antibiotics Pharyngitis: Viruses, group A streptococci (15-20%) Exudates covering is highly suggestive of Strep.pyogen All rights reserved. Registered with US copy right office 53 RM Centor at el criteria for Mx of Pharyngitis вЂ“ fever, tonsillar exudates, tender cervical lymphadenopathy and absence of cough вЂ“ If 1 or 2 present, rapid antigen test. If test is positive, give antibiotics; If 3 or 4 present, give empiric antibiotics Rapid Strep test (Best initial test / next step in management) Tx: Penicillin (1st choice), Macrolide / 2nd generation Cephalosporin orally Benefit of antibiotic therapy in patient with Strep. Pyogens pharyngitis вЂ“ it can prevent Rheumatic fever (not glomerulonephritis) оЂЉ оЂЉ оЂЉ оЂЉ Influenza: fever, headache, myalgias, fatigue Rapid antigen detection method of swab or washing of nasopharyngeal secretion Viral culture (most accurate test) Oseltamivir / Zanamivir (active against both A & B) в†’ within 48 hrs of onset of Symptoms Vaccination (C/I in patients allergic to eggs) оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ Л— Л— Л— Л— оЂЉ Л— Л— Л— Л— Л— Л— Л— Л— Л— Л— Л— Л— Л— Л— Lung Abscess: chest X-ray (Best initial test) Biopsy of lesion & culture (most accurate test) Clindamycin (Best initial Therapy) Alcoholic, Extremely bad odor ( like decomposing dead animal) Pneumonia: Following flu в†’ Stap. Aureus ( abscess ) HIV positive ( CD4+ < 200 cells ) в†’ PCP California , desert of Arizona в†’ Coccidiomycosis Young ( school children ) в†’ Mycoplasma Alcoholics в†’ Klebsiella Smoker, COPD в†’ H. influenzae Elderly pt, CXR вЂ“ lobar consolidation в†’ Strep. Pneumoniae Neutropenia, Steroid use, cavitatory lesion, part of the lesion moves on CXR when patient change position в†’ Aspergilloma (fungus ball) Exposure to animal at the time of giving birth в†’ Coxiella Burnetti ( Q-fever ) Birds, Triad of pneumonia, spleenomegaly and meningoencephalitis [fever, dry cough and headache] in immunocompetent host в†’ Chlamydia Psittaci Pneumonia вЂ“ Tx: Doxycycline (follow-up on out patient basis) Old , smoker , AirвЂ“conditioning в†’ Legionella Pneumonia & diarrhea in transplant patient пѓ CMV Immunocompromised patient, gram (+) branching rods & partially acid-fast пѓ Nocardia (Trimethoprim-Sulfamethoxazole) Recurrent pneumonia in chronic smoker, next step? пѓ CT chest to rule out lung CA which may obstruct bronchus and produce recurrent pneumonia вЂў Best diagnostic test for patient with recurrent pneumonia [most probable cause S.S.Patel. All rights reserved. Registered with US copy right office 54 will be an endobronchial obstruction] вЂ“ Flexible bronchoscopy [If question is вЂњwhat is the best next step in Mx of patient with recurrent pneumonia?вЂќ вЂ“ order chest CT scan] Empiric Therapy Community вЂ“ Acquired (out-patient) Macrolide (1st choice) Community вЂ“ Acquired (Inpatient) Hypoxia(<70 PO2) Newer Fluoroquinolones O2 Saturation ( < 94 % at room ) PR > 24 Macrolide / Doxycycline + New fluoroquinolone / 2nd generation cephalosporin/ B-lactam + B вЂ“ lactamase inhibitor combination оЂЉ - оЂЉ Л— Л— Л— Л— Л— Л— Л— Л— Л— Л— Л— S.S.Patel. Hospital вЂ“ Acquired Pneumonia (48-hrs after Hospitalization) 3rd generation Cephalosporin Carbapenem B-lactam / B- lactamase inhibitor combination PPD: PPD is Positive if induration after 48 hrs is > 5 mm в†’ HIV Positive , recent exposure to TB , Immunocompromised > 10 mm в†’ High risk group ( Health workers , recent immigrant , Homeless) > 15 mm в†’ Low risk group PPD is Best screening Test (not diagnostic test) to check TB exposure PPD positive, CXR negative вЂ“ INH + Vit-B6 for 9 months; PPD become positive again on routine test and CXR negative вЂ“ reassurance and no Tx require Prophylaxis for only INH resistant TB вЂ“ Rifampin for 4-months Prophylaxis for both INH and Rifampin resistant TB вЂ“ Pyrazinamide and Ethambutol or levofloxacin Diarrhea: TravelerвЂ™s diarrhea вЂ“ E.coli Undercooked hamburger meat вЂ“ E.coli 0157 : H7 ( associated with HUS ) Giardia lamblia вЂ“ camping, contaminated water source HIV Positive, CD4 < 50 cells, Acid fast oocyst вЂ“ Cryptosporidium Ingestion of unrefrigerated meat вЂ“ Cl. Difficile Fired rice вЂ“ Bacillus Cerius Contaminate Shellfish вЂ“ V. parahaemolyticus Severe liver disease patient вЂ“ V.vulnificus Diagnosis: Presence of blood in stool by methylene blue test (1st step) Stool culture (most accurate) Patient with chronic diarrhea вЂ“ stool microscopic examination (first step) [not stool culture] All rights reserved. Registered with US copy right office 55 Л— Л— Л— Л— Л— Л— Treatment: Ciprofloxacin (Best initial empiric therapy) Cryptosporidiosis в†’ raise CD4+ count Scombroid в†’ Histamines в†’ Symptoms in few mins в†’ Tx вЂ“ Anti-Histamines Salmonella enteritidis diarrhea вЂ“ supportive care (rarely use antibiotics) Giardia & Cl. difficile в†’ Metronidazole For Giardiasis, only symptomatic carries are treated вЂ“ Asymptomatic carriers are not treated except for few circumstances like pregnant woman in house, immunocompromised individual, cystic fibrosis - Tx of Giardiasis in pregnant women вЂ“ Paromomycin оЂЉ Hepatitis B : Acute в†’ HBsAg, IgM to HBV HBsAg в†’ Active disease + Persistent disease Anti вЂ“ HBcAb в†’ 1st Ab to appear in Hepatitis B HBeAg в†’ Active viral production + Infectivity Anti - HBeAb в†’ Appear after viral no longer detectable Anti вЂ“ HBsAg в†’ Protective Ab, Immunization Window Period ( equivalence zone of Ab production ) в†’ Anti вЂ“ HBcAb, AntiHBeAb Treatment : Interferon / Lamivudin (chronic Hep B) Needle stick injury with HBsAg positive в†’ HB immunoglobulin + HB vaccine Tx of HBV post-exposure in a patient who already had Hep B vaccine but did NOT seroconvert after Hep B vaccination вЂ“ Hep B immunoglobulin Л— Л— Л— Л— Л— Л— Л— вЂў Л— Л— Л— Diagnosis of Acute HAV, HDV, HEV в†’ Presence of IgM antibody оЂЉ Л— Л— вЂў Л— Hepatitis C: Blood transfusion Dx: PCR HCV RNA Treatment в†’ Interferon + Ribavirin Advance stage (cirrhosis) в†’ Liver Transplant оЂЉ Pelvic Inflammatory Disease: Л— Lower Abdominal and pelvic pain, fever, leucocytosis, discharge (vagina) Cervical motion tenderness Л— Gram stain & culture of discharge Л— USG (to exclude ovarian cyst / Tubo-ovarian abscess) Л— Tx: Single dose Ceftriaxone + Doxycycline for two weeks (out patient) Л— Inpatient (high grade fever / в†‘в†‘ WBCs) в†’ Doxycycline + IV Cefoxitin Л— Goal of Tx of severe PID is to obtain high blood concentration of antibiotics as soon as possible so all therapy should be intravenous. use IV Cefoxitin + Oral Doxycycline or IV ceftriaxone + oral Doxycycline S.S.Patel. All rights reserved. Registered with US copy right office 56 Fever, urinary frequency, urgency, burning Purulent Discharge No Discharge Urethritis (Tx: Ceftriaxone + Azithromycin) Cystitis Gonococcus gram stain Chlamydia Serology Culture Ligase chain reaction Tx : Ceftriaxone IM Tx Doxycycline(7 days ) Azithromycin Azithromycin(single) Ciprofloxacin Mucopurulent discharge Cefixime Dx of Chlamydia is costlier than Tx so all patient with gonorrhea receive Azithromycin (1g single dose) Purulent discharge вЂў вЂў вЂў вЂў - E.coli (>80%) Urinary stasis / Foreign body Predisposes Suprapubic tenderness Urine Analysis looking for WBCs ( Best Initial ) Nitrates в†’ Nitrites (gram(-) organism) Urine culture (> 100000 colonies) Tx: If uncomplicated, Trimethoprim / Sulfamethoxazole or Quinolones X 3 days In DM в†’ 7 days Tx Chlamydia infection in pregnancy вЂ“ Erythromycin 500mg PO four times a day for 7 days Asymptomatic bacteruria is common in elderly, and doesnвЂ™t require treatment if WBC count is less than 20 / HPF. [reassure and repeat urine culture again after 12 months] Treatment of UTI /Asymptomatic bacteriuria in pregnancy пѓ Nitrofurantoin or Ampicillin for 7-10 days Uncomplicated UTI can be treated by prescribing TMP-SMX over the phone в™¦ Chancre в™¦ Painless 10 Syphilis (Treponema Pallidum) - Dark field exam (best initial for primary) - VDRL & RPR (Best initial for 20 & tertiary) - FTA-ABS, MHA-TP (most specific test) S.S.Patel. All rights reserved. Painful Chancroid ( H . ducreyi ) - Genital ulcer, enlarged tender inguinal lymphnode - Gram stain, culture, PCR - Tx: Azithromycin (single dose) Ceftriaxone 250 mg 1M(single) Registered with US copy right office 57 20 Syphilis в†’ Cutaneous rash (symmetrical) в†’ Condylomata lata (infectious) Л— Condylomata Acuminata (HPV): verrucous, papilliform, skin color lesion Л— Condylomata late (Secondary Syphilis): flat or velvety lesion * Tertiary Syphilis в†’ gumma, Tabes dorsalis , Argil-Robertson pupils * Treatment: Penicillin (Benzathin) вЂ“ 2.4 million units / Week IM for 10 (1 week) & 20 (3 weeks) Tertiary вЂ“ 10-20 million units / day IV for 10 days If Allergic to Penicillin вЂ“ Doxycycline for 10 & 20 If Allergic to Penicillin - Desensitization in tertiary & pregnancy. * * * вЂў No effective prevention is available for Jarisch-Herxheimer reaction (fever, chills) occur within 24-hrs after starting therapy to syphilis пЃ® Lymphogranuloma Venerum: - Chlamydia trachomatis (L1,L2,L3) - Small, transient , non indurated lesion that ulcerates & heals quickly; unilateral enlargement of inguinal lymphnodes, multiple draining sinuses, buboes - Diagnosis в†’ serological tests. Isolation of Chlamydia from pus in buboes - Tx в†’ Doxycycline в– Л— Л— Л— Л— Л— Granuloma Inguinale : Donovania granulomatis, calymmatobacterium granulomatis Painless red nodule that develops into an elevated granulomatous mass Slow healing & scar formation occur Giemsa / wright stain , Punch biopsy Tx в†’ Doxycycline. пЃ® Л— Л— Л— Л— Л— Genital warts: caused by HPV Condylomata acuminate warm , moist surface in the genital areas Cauliflower appearance Tx: Imiquimod ( immune stimulant ) , Cryotherapy, Podophyllin ,Laser removal пЃ® - Acute Bacterial Pyelonephritis: E.coli (MCC) , Proteus , Klebsiella, Enterococcus Costovertebral angle tenderness Diagnosis : Dysuria, flank pain, urine culture (> 100000 Colonies) USG ( to rule out obstruction ) Tx: Ciprofloxacin for 10-14 days (Any gram (-) Coverage antibiotics) S.S.Patel. All rights reserved. Registered with US copy right office 58 пЃ® - Perinephric Abscess: Persistent of pyelonephritis вЂ“ like symptoms after treatment of pyelonephritis Urinanalysis & Urine culture ( best initial test ) USG (best initial scan) Biopsy & culture ( most accurate test for etiology of organism ) Tx: gram (-) coverage + Drainage пЃ® Л— Л— Л— Molluscum Contagiosum: Caused by Poxvirus Papules with central umbilication, Giemsa stain в†’ inclusion body D/D: Cutaneous Cryptococcosis вЂ“ red colored papules with central umbilication (resembles Molluscum Contagiosum) вЂ“ Dx: biopsy of lesions Л— Tx: Freezing, electrocautery, curettage пЃ® Л— Л— Л— Л— Л— Л— Л— Л— Л— Л— Л— Osteomyelitis: Pain erythema, swelling, tenderness over the infected bone, draining sinus tract Hematogenously Spread Steph Aureus (MCC) Salmonella in pt with Sickle cell disease. [Order Hb electrophoresis to confirm diagnosis of sickle cell disease if not previously diagnose of sickle cell disease] Best initial test в†’ X ray в†’ Periosteal elevation Earliest test в†’ MRI / Bone Scan Most accurate test в†’ Bone biopsy & culture Best empiric Treatment в†’ Oxacillin / Neficillin + Aminoglycosides Chronic osteomyelitis в†’ 12 weeks followed by 8-12 weeks orally ESR в†’ useful to follow during treatment MRI вЂ“ diagnosis of osteomyelitis in vertebrae and diabetic foot (most accurate test) [C/I in patient with implanted pacemaker, defibrillators вЂ“ Bone scan is useful in those patient] вЂў Low-grade fever, elevated ESR, low backache, tenderness to gentle percussion over the spinous process, diagnosis? Osteomyelitis of vertebrae; best test? MRI оЂЉ вЂў вЂў вЂў вЂў вЂў Gas Gangrene: Caused by Cl. Perfringens Deep , necrotic wound , without exit to the surface Crepitation over the site X вЂ“ ray в†’ gas bubbles Tx в†’ High does penicillin / clindamycin в†’ Surgical debridement / Amputation оЂЉ Tetanus: Л— caused by Cl. Tetani S.S.Patel. All rights reserved. Registered with US copy right office 59 Л— Lockjaw , Respiratory arrest , dysphagia , flexion of arms & extension of lower extremities * Tetanus Prophylaxis: (TIG вЂ“ Tetanus Immunoglobulin) Л— Two Important factors вЂ“ Vaccination history & Wound (clean or dirty) Л— Last TT dose (< 10 yrs) & clean wound вЂ“ No need to give tetanus prophylaxis Л— Last TT dose (> 5 yrs) & dirty wound вЂ“ Give TT prophylaxis Л— No previous vaccination & clean wound вЂ“ Give TT prophylaxis Л— No previous vaccination & dirty wound вЂ“ Give TT + tetanus immunoglobulins оЂЉ оЂЉ оЂЉ оЂЉ Blastomycosis в†’ Broad base bud , rooting woods, skin lesions (crusted, heaped up, warty lesion with violaceous hue) вЂ“ Tx: Amphotericin B Histoplasmosis в†’ Soil enriched with bird / bat feces, No skin lesions, found in RES cells Coccidioides вЂ“ California, spherules, skin lesions (Erythema multiforme and Erythema nodosum) Aspergillosis в†’ Neutropenia / steroid use / Cytotoxic drug, cavitatory lesion, CXR вЂ“ Abnormal, Sputum вЂ“ Aspergillus, Acute 450 branching оЂЉ Babesiosis вЂ“ hemolytic anemia with jaundice, no rash, tick-bite вЂ“ Tx: quinine + clindamycin / atovaquone + azithromycin оЂЉ Ehrlichiosis вЂ“ tick-bite, вЂњspotless вЂ“ Rocky Mountain spotted feverвЂќ, fever, malaise, headache, nausea and vomiting вЂ“ Tx: Doxycycline оЂЉ Trichinellosis вЂ“ Swelling around eye, severe muscle pain, splinter hemorrhage, eosinophilia, no murmur оЂЉ Acquired Immuno Deficiency Syndrome (AIDS): Prophylaxis in AIDS в†’ CD4+ < 200 cells в†’ Trimethoprim / Sulfamethoxazole (Pneumocystis Carinii - PCP) + CD4 < 50 cells в†’ Azithromycin once a week for MAC (Mycobacterium Avium Complex - MAC) Indication corticosteroid in PCP вЂ“ alveolar-arterial O2 ratio >35 mmHg on room air and/or Pao2 <70 mmHg on room air Start Antiretroviral therapy when CD4+ < 350 / viral load > 55000 Post exposure prophylaxis вЂ“ AZT + Lamivudin + PI (4-wks) Tx of HIV-associated thrombocytopenia вЂ“ Zidovudine HIV virus load after initiation of HAART вЂ“ within 4-wks [<5000 copies/ml], 816 wks [<500 copies/ml], after 4-6 months [<50 copies/ml] After that viral load can be reevaluated every 3 months CD4 count & HIV viral load monitoring for patient not on HAART вЂ“ once every 3-4 months вЂў вЂў вЂў вЂў вЂў вЂў вЂў S.S.Patel. All rights reserved. Registered with US copy right office 60 вЂў Best indicator of immune status of HIV(+) patient вЂ“ CD4 count вЂў Mx of Esophagitis in HIV positive вЂ“ empiric Fluconazole; If doesnвЂ™t get better, endoscopy вЂў Fluctuation of CD4 count and viral load in patient on HAART is common and is self-limited вЂў CT scan of AIDS patient shows multiple hypodense, non-enhancing lesion with no mass effect in the cerebral white matter, diagnosis? Progressive multifocal leukoencephalopathy (PML) (JC virus) вЂ“ without treatment, majority of patient die within 3-6 months after onset of symptoms вЂ“ Tx: HAART вЂў Dx of HIV during window period вЂ“ measuring viral load by HIV RNA PCR assay or by confirming presence of p24 antigen вЂў Pregnant Patient в†’ AZT start at 14 wks. If serious disease ( very low CD4 / very high viral load ) в†’ start 3 drugs вЂў Only efavirenz is teratogenic вЂў AZT в†’ Severe Anemia вЂў Didanosine (DDI ) & Zalcitabine (DDC) в†’ Pancreatitis вЂў Stavudine в†’ Peripheral neuropathy вЂў Indinavir в†’ Nephrolithiasis вЂў All Protease Inhibitors в†’ Central obesity, hyperlipidemia (Tx: Gemfibrozil), hyperglycemia вЂў Nucleoside reverse transcriptase inhibitors can cause lactic acidosis by reduced O2 utilization by tissue вЂў Disseminated Histoplasmosis in HIV пѓ IV amphoterecin B, followed by lifelong itraconazole вЂў Contact of saliva of patient with HIV has never shown to transmit infection so If patient present with human bites from HIV-infected person, give standard wound care and prescribe Ampicillin + Sulbactam п‚§ Any newborn (<4 wks of age) with fever >38 C should be admitted to the hospital and thoroughly evaluated (blood, urine & CSF culture) and start antibiotics в– TORCH Infection: п‚§ Toxoplasmosis вЂ“ more serious in first trimester в†’ intracranial calcifications, IUGR, microcephaly, blindness вЂ“ First trimester пѓ Spiramycin / elective abortion; In second & third trimester пѓ pyrimethamine + sulfadiazine вЂ“ Newborn вЂ“ Pyrimethamine and Sulfonamide & leucovorin. п‚§ Other (Syphilis) вЂ“ can be transmitted to fetus at any stage of pregnancy в†’ fever, anemia, failure to thrive, maculopapular rash, hepatosplenomegaly (<2yrs) в†’ Hutchinson teeth, saber skin, saddle nose, clutton joints (late manifestation) п‚§ Rubella вЂ“ IUGR, Cataract, PDA (Patent Ductus Arteriosus), Deafness, blueberry muffin lesions. п‚§ CMV вЂ“ IUGR, Chorioretinitis, Periventricular calcification. п‚§ Herpes вЂ“ infection occur due to passage through an infected birth canal в†’ first time infection in mother has high rate of transmission в†’ local (5-14days), disseminated (5-7 days), CNS (3-4 wks). вЂ“ Tx: Acyclovir, delivery by c-section. S.S.Patel. All rights reserved. Registered with US copy right office 61 п‚§ Varicella вЂ“ neonatal (Perinatal) disease is treated with VZIG if mother develop varicella 5 days before to 2 days after delivery, Acyclovir in all perinatal disease. в– - Pertussis: Whooping cough в†’ forceful inspiratory gasp (whoop) after a paroxysmal cough. Children < 5 yrs of age. Catarrhal stage (infectious stage), paroxysmal stage, convalescent stage. Leukocytosis caused by absolute lymphocytosis. Direct fluorescent antibody testing of nasopharyngeal secretion (Rapid test). Tx: Supportive/Erythromycin. ALL contacts should receive prophylaxis with Erythromycin regardless of their age & immunization status. в– - Cat Scratch Disease: Bartonella Hensalae. Chronic regional lymphadenitis, fever, headache, malaise. Resolve spontaneously in 2-4 months or Azithromycin for 5 days Bacillary Angiomatosis in AIDS patient. в– - Erythema Infectiosum: Fifth disease, Parvovirus B19 вЂњSlapped cheekвЂќ appearance (due to erythematous rash) Aplastic crisis in sickle cell anemia patient Infectious before the appearance of rash в– - Roseola (Exanthema Subitum): HHV (Human herpes Virus-6) High grade fever в†’ resolve by 3-4 day в†’ maculopapular rash appear. Supportive therapy. в– Measles (Rubeola): - Cough, coryza, conjunctivitis, koplik spots (grayish white dots on the buccal mucosa), rash appear on face and spread towards trunk (rash pattern is same in Rubella but patient looks more ill in Measles compare to Rubella) - Supportive therapy, Vit-A supplementation. в– - Mumps: Contagious 1 day before and 3 days after the swelling. Swelling of the parotid gland, orchitis. Elevation of serum amylase вЂ“ Tx : Supportive treatment в– - Rubella (German Measles): Contagious 2 days before the rash begin and 5 days after the rash Retroauricular, Posterior cervical & Postoccipital lymphadenopathy Supportive treatment. S.S.Patel. All rights reserved. Registered with US copy right office 62 - complication of Rubella вЂ“ Arthritis, thrombocytopenia and encephalitis в– Herpes Simplex: - Burning rash followed by generalized eruption after 2-3 weeks, Vesicles on red erythematous base вЂ“ Tx: Acyclovir в– Varicella (Chickenpox): - Contagious 2 days before the rash begin and until all the lesions are crusted. - Pruritic rash consisting of papules, vesicles, pustules and crusted lesions in crops in various stages - Varicella Zoster Immunoglobulin (VZIG), Acyclovir - Varicella Post exposure prophylaxis пѓ VZLG / Acyclovir (within 72 hrs only) - Transmission of varicella from vaccinated individual to the organ transplant household member is not typical. It can occur if rash appear after vaccination. If rash appear, isolate the vaccinated person. в– Scarlet Fever: - Group A ОІ-hemolytic Streptococci. - вЂњStrawberryвЂќ tongue, circumoral pallor, maculopapular/sandpaper rash, pastia lines, military sudamina (small, vesicular lesions over the hands, abdomen and feet), bilateral cervical lymphadenopathy - Tx: Penicillins (DOC), Erythromycin, 1st generation cephalosporin. в– Kawasaki Disease: - Child with unilateral cervical lymphadenopathy, fever, desquamating rash on palm, sole & mouth, Strawberry tongue, fissured lips - Echocardiography (Best screening test вЂ“ Coronary artery aneurism) - Tx : IV Immunoglobulin (first step in treatment), High dose Aspirin [If need arise to give continuous Aspirin, patient should receive influenza vaccine to prevent ReyeвЂ™s syndrome] в– - Hand, foot and mouth disease: Coxsackie A 16 virus Vesicular rash involve hand, foot & mouth. Supportive treatment в– - Scabies: Permethrin 5% cream / 1% lindane (>2 months of age). 6% sulfur in petroleum (<2 months of age) All family member & care taker should be treated. в– Laryngotracheobronchitis (Croup): в—Џ Parainfluenza virus в—Џ Cold symptoms followed by brassy, barking cough & intermittent inspiratory stridor в—Џ X-ray: вЂњsteepleвЂќ signs indicate a narrow subglottic space. S.S.Patel. All rights reserved. Registered with US copy right office 63 в—Џ Tx: steam from a vaporizer, continuous humidification (mild cases). Stridor at rest в†’ epinephrine and corticosteroids systemically. give trial of epinephrine before intubation в– - Acute Epiglottitis: H.influenzae type B Acute onset of symptoms вЂ“ high grade fever, stridor, drawling. Younger child sit in tripod position with neck hyperextended. X-ray: вЂњthumb printвЂќ sign Tx: Intubation (1st step) (regardless of degree of respiratory distress), Antibiotic в– - Bronchiolitis: infection of lower respiratory tract Respiratory syncytial virus (RSV) around 6 months of age H/O URTI, rhinorhea, sneezing RSV infection increase the risk of asthma in later life CXR: hyperinflation of the lungs Antigen detection in nasopharyngeal secretion/culture. Criteria for hospitalization: premature, younger than 3 months old, RR>60/min, PO2 <60 mmHg on room air , feeding difficulties Tx: respiratory isolation and supportive therapy (like IV fluids Antipyretics, Humidified air and bronchodilators), Aerosolized epinephrine (no corticosteroids), Ribavirin (aerosolized) (reserved for serious cases), Intubation. в—Џ в—Џ - пЃ® Rocky-Mountain Spotted Fever: headache, fever, rose-red maculopapular rash (rosettes) on palms & soles в†’ Tx: Doxycycline (benefits out weigh the risk) (In Lyme, course of treatment is long thatвЂ™s why we use Amoxicillin in children less than 9 yrs of age) пЃ® Lyme Disease: erythema chronicum migrans вЂ“ The risk of acquiring Lyme disease after tickвЂ™s bite is less than 1.5%. The most common complication of tick bite is local inflammation and infection вЂ“ Tx of classic early-localize Lyme disease вЂ“ oral Doxycycline for 28-days [It is clinical diagnosis, doesnвЂ™t require serological test] вЂ“ Dx of Lyme arthritis вЂ“ ELISA antibodies in synovial fluid вЂ“ Prognosis of Lyme arthritis is good and more than 90% of patients are diseasefree one year after treatment вЂ“ Early-disseminated-Lyme вЂ“ do CSF examination вЂ“ If positive Lyme serology on CSF, give IV antibiotics вЂ“ When pregnant patient exposed to tick bite, prophylaxis of Lyme is necessary вЂ“ give Amoxicillin orally [All other patient doesnвЂ™t require any prophylaxis; Tick must be attached for more than 24-hrs to transmit Lyme disease] пЃ® Tx of UTI in newborn вЂ“ fever, increase WBC, >20 hpf вЂ“ Ampicillin + Gentamicin (both IV) S.S.Patel. All rights reserved. Registered with US copy right office 64 в– Eye discharge in Neonate: - 1-3 day of life вЂ“ Physiological - 3-5 day of life (Mucopurulent discharge) вЂ“ Gonococcal Conjunctivitis (Tx : topical antibiotics) - Few days after birth вЂ“ Mucoid discharge вЂ“ Chlamydia Conjunctivitis (Tx : Oral (systemic) antibiotics to prevent pneumonia) в– - Eye discharge in child: Purulent discharge, crusting in the morning вЂ“ Bacterial conjunctivitis Clear watery discharge (usually bilateral, h/o URTI) вЂ“ Viral Conjunctivitis follicular conjunctivitis вЂ“ trachoma (chlamydia) оЂЉ Otitis Media (infant is irritable) in patient with h/o conjunctivitis (red eye) is caused by non-typable H. Influenzae so use Amoxicillin + Clavulanate acid (not Amoxicillin alone) в– вЂў - IMMUNIZATION: Live Attenuated Vaccine : Viral: MMR, Yellow fever, Varicella Bacterial: BCG, oral typhoid вЂў - Inactivated Vaccine : Viral: Polio, Rabies, Hepatitis A ( whole) Fractional Protein based вЂ“ Subunit в†’ Hep B, Influenza, acellular Pertussis Toxoid в†’ Diphtheria, Tetanus Polysaccharide based вЂ“ Pure в†’ Pneumococcal, Hib, meningococcal Conjugate в†’ Hib, Pneumococcal вЂў Influenza & Yellow fever vaccines are contraindicated in persons with hypersensitivity to egg. IPV & MMR are contraindicated in persons with hypersensitivity to neomycin/ streptomycin. вЂў вЂў - S.S.Patel. Contraindications to vaccines : severe allergic reaction to prior doses of vaccine ( or) to a component Encephalopathy following Pertussis vaccine Immunocompromised state & pregnancy Only MMR is contraindicated in HIV infected patient with severe immunocompression & Symptomatic. All other vaccine can be given in HIV positive symptomatic person. Previous febrile illness is not a contraindication (C/I) for giving MMR C/I to MMR are: pregnancy, severe immunodeficiency (asymptomatic HIV is not a C/I), recent immunoglobulin administration, allergy to neomycin All rights reserved. Registered with US copy right office 65 вЂў вЂў - S.S.Patel. Immunization: Usually vaccines [DTaP, Hib, PCV, IPV] and Hep B (if not given at birth) are given at 2, 4, 6 months and then boosters if appropriate. All pre-term infants should receive vaccines according to their chronological age, not their gestational age. Hep B vaccine should be administer at birth (Wt should be >2 kg) Hep-B vaccine в†’ at birth. DTaP, Hib, PCV, IPV в†’ Started at 2 months of age. MMR, Varicella в†’ started at 12 months of age. Influenza в†’ started at 6 months of age. DTaP в†’ 2nd & 3rd dose 4-6 weeks apart, 4th dose 6 months after 3rd dose. Hib в†’ All doses 4-6 weeks apart, if first dose is given after 15 months of age then no need for other doses, booster b/w 12-15 months of age PCV в†’ All doses 4-6 weeks apart, no doses for healthy child of в‰Ґ 24 months of age, booster b/w 12-15 months of age MMR в†’ 2nd dosesв†’ 4-6 weeks after 1st dose/at 4-6 yrs f age. Meningococcal Vaccine (serotypes A, C, Y, W - 135) в†’ not protective for those < 2 yrs of age. That means give vaccine after 2 yrs of age If mother is HBsAg positive в†’ HBIG + HB Vaccine at birth. OPV (oral polio vaccine) is not used in USA Immunization in internationally adopted child without written documentation вЂ“ Give all necessary immunization according to recommendation for unimmunized child + screen for Hep B, Hep C, HIV, Syphilis and TB All rights reserved. Registered with US copy right office 66 MUSCULOSKELETAL SYSTEM пЃ® New born в†’ Uneven gluteal folds в†’ easy posterior dislocation of hip with вЂњclickвЂќ and returned to normal position with вЂњsnappingвЂќ в†’ positive family history в†’ Developmental dysplasia of Hip в†’ sonogram of the Hip (not X-ray) Л— Tx: Abduction splinting with Pavlik harness оЂЉ оЂЉ оЂЉ оЂЉ Legg calve Parthes disease в†’Avascular necrosis of the capital femoral epiphysis в†’ around 6 yrs of age в†’ Tx: keep femoral head in acetabulum by casting and crutches Slipped capital femoral epiphysis в†’ obese, around 13 yrs of age в†’ sits with the sole of the foot on the affected side pointing toward the other foot в†’ Tx : Pin the femoral head in place Osgood вЂ“ Schlatter Disease в†’ Osteochondrosis of the tibial tubercle в†’ Persistent pain over tibial tubercle, aggravated by contraction of quadriceps в†’ Teenagers в†’ Tx: immobilization of the knee in an extension (or) Cylinder cast for 4 вЂ“ 6 weeks. Spondylolisthesis в†’ development disorder characterized by a forward slip of vertebrae вЂ“ palpable вЂњstep offвЂќ on examination if the disease is severe оЂЉ Flexible Kyphosis вЂ“ postural round back which is correct on voluntary hyperextension, no angular deformity and no neurological problem вЂ“ it is a common finding in adolescent and usually has no long term deformity оЂЉ Scoliosis вЂ“ angularity in the thorax region on forward bending вЂ“ Tx: Milwaukee brace and spinal muscle exercises Joint aspiration Cell count в†“ п‚§ < 2,000 вЂ“ OA, Traumatic п‚§ Up to 50,000 вЂ“ Inflammatory (RA, Gout, Pseudogout) п‚§ >75,000 (without crystal) вЂ“ Septic S.S.Patel. в†“ Gram stain в†“ (+) organism (-) organism Staph Aureus All rights reserved. N. Gonorrhea в†“ Microscopic Polarization Needle-shaped / (-) birefringent вЂ“ Monosodium Urate (Gout) Rhomboid / (+) birefringent вЂ“ Calcium Pyrophosphate (Pseudogout) Registered with US copy right office 67 вЂў - Rheumatoid Arthritis (RA) вЂў Osteo Arthritis (OA) Polyarticular Symmetric - Monoarticular Asymmetric Inflammatory synovitis - Non-inflammatory bone erosions - Non-erossive MCP & PIP involvement - PIP& DIP involvement Swan-neck deformity - Osteophytes & unequal joint space Boutonniere deformity - BouchardвЂ™s node (PIP) radial deviation of the wrist with ulnar - HeberdenвЂ™s node (DIP) deviation of the digits - Tx: Acetoaminophen (NSAID) - Tx: NSAID, Glucocorticoids, methotraxate, Capsaicin cream,joint Arthroplasty Gold, Sulfasalazine, Infliximab, Hydroxychloroquine вЂў вЂў вЂў Best initial drug for Rheumatoid Arthritis (RA) вЂ“ NSAID Best initial DMARDs вЂ“ Methotraxate Patient on hydroxychloroquin вЂ“ require frequent eye exam в– Ankylosing Spondylitis: Positive HLA B-27, M>W, 2nd - 3rd decade - chronic lower back pain, morning stiffness >1 hrs improve with exercise - Anterior uveitis, Aortic insufficiency, 3rd degree heart block, Apical Pulmonary fibrosis, Restrictive lung disease and Ig A nephropathy - Best initial test: X-ray of lumbar spine в†’ sacroilitis and eventual fusing of the sacroiliac joint, bamboo spine вЂ“ Repeat X-ray after 3-months & ESR (used to monitor disease activity) - Tx: NSAID, Physical therapy, exercise вЂў Next step in management of patient with low back pain who do NOT respond to 6-wks of conservative therapy вЂ“ ESR; If increase in ESR, next step? вЂ“ Imaging studies (MRI / CT scan) в– Reactive Arthritis: infectious diarrhea (C.Jejunii) + Arthritis - Urethritis (Chlamydia) / conjunctivitis + Arthritis в†’ Reiter Syndrome [Strong association with HLA-B27 (80%); No HLA-B27 = no Reiter syndrome; negative rheumatoid factor] - Early Antibiotic use in urethritis decrease the chances of Reiter Syndrome в– Psoriatic Arthritis: DIP joint + pitting of nail + skin lesions в– Enteropathic Arthritis (Ulcerative colitis/ CrohnвЂ™s disease): - Inflammatory Bowel disease + Arthritis + Pyoderma gangrenosum + erythema nodosum в– Gout: deposit of uric acid crystals in joints вЂ“ most common site first toe (podagra) вЂ“ precipitating factors are alcohol, steroid withdrawal, diuretics, Pyrazinamide, Ethambutol, following anti-cancer treatment S.S.Patel. All rights reserved. Registered with US copy right office 68 в– Pseudo Gout: deposit of calcium pyrophosphate in joints вЂ“ most common site knee joints вЂ“ pre-existing joint damage is a precipitating factor вЂ“ causes: Hyperparathyroidism, Hemochromatosis, Hypophosphatemia, Hypomagnesemia в– Septic arthritis: Gonococcal: migratory polyarthropathy, Tenosynovitis (inflammation of tendon sheath) Staphylococci: pre-existing joint damage (eg. RA patient) вЂ“ First step in Mx of septic arthritis вЂ“ immediate surgical drainage followed by IV antibiotics пЃ® Inflammatory myopathies (polymyositis, Dermatomyositis, Inclusion body myositis) в—Џ Difficulty with task involving proximal muscles (lifting, combing hair, etc) в—Џ в†‘в†‘ Creatinine Kinase & Aldolase (most sensitive test) в—Џ EMGв†’short duration, low amplitude myopathic potentials в—Џ Muscle Biopsy (most specific test) в—Џ Tx : Steroidsв†’ polymyopathies & Dermatomyositis в—Џ Inclusion body myositisв†’ Resistant to immunosuppressive therapy вЂў Fibromyalgia: chronic widespread pain (multiple tender points), fatigue, women (20-50 yrs) вЂ“ Tx: TCA or Cyclobenzaprine вЂў Polymyalgia rheumatic: pain & stiffness (at least 30 min) of the shoulder and pelvic girdle, >50 yrs of age, ESR >50 mm/hr вЂў Polymyositis: c/o proximal muscles weakness (combing hair, difficulty raising from chair) в– SLE: anti-nuclear Ab, Anti-smith & Anti-ds-DNA Ab (most specific) - non-erosive arthritis, malar rash, photosensitivity, Renal, CVS (Libman-Sack endocarditis вЂ“ sterile vegetation on MV), CNS involvement (psychosis) - Anti-phospholipids antibody вЂ“ anticoagulant, recurrent abortion - Anti-cardiolipin antibody вЂ“ give false VDRL & RPR test - Absent of SLE symptoms, mildly high titer of ANA вЂ“ No further work up require - All SLE patients with renal involvement (Hematuria, Proteinuria) should have renal biopsy to guide the treatment - Tx of arthritis in SLE вЂ“ NSAID - Tx of SLE rash вЂ“ cortisone cream - Tx of Lupus Nephritis вЂ“ pulse Cyclophosphamide пЃ® Rosacea: facial rash same distribution as SLE, but rosacea has pustules & papules & flushing of this rash by hot drinks вЂ“ Tx: Metronidazole в– Scleroderma: excessive collagen deposition вЂ“ RaynaudвЂ™s phenomenon (blue discoloration of fingers on exposure to cold), skin thickening, dysphagia вЂ“ Antiscl-70 Ab; Tx of Scleroderma induce HTN вЂ“ ACE inhibitors S.S.Patel. All rights reserved. Registered with US copy right office 69 в– CREST Syndrome: Anti-centromere antibody Л— Calcinosis, RaynaudвЂ™s phenomena, Esophagus (dysphagia), Scleroductly (clawlike finger), Telangiectasia (dilated blood vessels) [Calcinosis is a feature of CREST syndrome, not Scleroderma] в– RaynaudвЂ™s Disease вЂ“ RaynaudвЂ™s phenomena occurs independently without connective tissue involvement вЂ“ Tx: Nifedepine / Amlodepine в– Sjogren syndrome: anti-Ro (SS-A) & Anti-La (SS-B) Antibodies, Dry eye (constant sensation of foreign body in eye), dental caries, parotid enlargement (lymphatic infiltration of glands вЂ“ lip biopsy - most specific) вЂ“ also gives positive RA factor вЂ“ Malignancy associated with Sjogren Syndrome вЂ“ HodgkinвЂ™s Lymphoma (B-cell lymphoma) пЃ® Juvenile Rheumatoid Arthritis: salmon pink evascent rash в†’ Tx : NSAIDs with monitoring of liver enzymes пЃ® Adult StillвЂ™s disease вЂ“ evanescent, salmon colored maculopapular rash that involves the trunk & extremities typically develops with fever; Arthralgia / Arthritis, significant leucocytosis вЂ“ Tx: NSAID with monitoring liver panel пЃ® Osteoporosis: hypogonadism, h/o taking steroids for long time вЂ“ normal ALP [Osteomalacia, PagetвЂ™s disease of bone вЂ“ increase ALP] вЂ“ Dx: Bone densitometry [T-score: > -2.5 вЂ“ osteoporosis; -1.5 to -2.5 вЂ“ osteopenia] вЂ“ Tx: Ca & Vit D (best initial drugs), Bisphosphonates, Calcitonin, Estrogen [only when perimanopausal (c/o hot flashes) + osteopenia] Only Bisphosphonates has shown to reduce incidence of hip fracture. вЂў Breast feeding increase bone resorption in women during post-partum period. Breast feeding increase the level of PTHrP which is important for transferring calcium in breast milk. вЂў Osteochondroma в†’ most common benign tumor в†’ Metaphysis вЂў Osteoma в†’ Facial bones в†’ associated with GardnerвЂ™s Polyposis Syndrome вЂў Giant cell tumor в†’ Epiphysis в†’ Females вЂў Osteogenic Sarcoma в†’ Metaphysis of distal femur , Proximal tibia вЂњsunburstвЂќ appearance on X-ray в†’ Male ( 10-25 yrs ) в†’ Familial retinoblastoma вЂў EwingвЂ™s Sarcoma в†’ Diaphysis & metaphysis of proximal femur, ribs, pelvic bones в†’ вЂњonion skinвЂќ appearance on X-ray вЂў ErbвЂ™s Palsy вЂ“ upper trunk (C5, C6) в†’ Axillary N. & Musculocutaneous N. в†’ muscles of shoulder & arm в†’ Arm: medially rotated & adducted в†’ Forearm: S.S.Patel. All rights reserved. Registered with US copy right office 70 extended & pronated (вЂњwaiterвЂ™s tipвЂќ) в†’ Prognosis of obstetrical ErbвЂ™s palsy is good, with 80% chance of full or near-full recovery вЂў KlumpkeвЂ™s Palsy вЂ“ lower trunk (C8, T11) в†’ loss of muscles of Hand вЂў Open fracture вЂ“ stabilization (eg. cast) + delayed primary closure (dressing of wound for few days to prevent risk of osteomyelitis) вЂў Green stick fracture: one cortex break & another is intact Torus fracture : impaction injury leads to buckling of cortex of long bones but on breach in continuity Plastic deformation: bones simply bend without any break in cortex Physeal Injury: fracture across the growth plate of bone. оЂЉ оЂЉ оЂЉ оЂЉ Supracondylar Fracture в†’ require appropriate casting / traction в†’ tense & tender forearm after casting в†’ Volkmann contracture (complication) в†’ immediate fasciotomy Fracture Clavicle в†’ junction of middle and distal thirds в†’ Tx: figure of 8 device for 4 вЂ“6 weeks. Anterior dislocation of shoulder вЂ“ held arm close to the body вЂ“ risk of Axillary nerve damage в†’AP & Lateral view в†’ Tx: reduction Posterior dislocation of shoulder в†’ high-voltage electric burns, severe muscle contraction (generalized seizure) в†’ Axillary (or) Scapular view оЂЉ Frozen shoulder пѓ joint stiffness & restriction of movement in all direction (both active and passive) оЂЉ Rotator cuff tear or tendinitis пѓ severe pain during mid arc abduction (passive movement is normal); lidocaine injection пѓ if movement improve пѓ tendinitis; if movement doesnвЂ™t improve пѓ tear; diagnostic test? пѓ MRI of shoulder оЂЉ оЂЉ оЂЉ S.S.Patel. Humeral shaft fracture в†’Radial nerve injury в†’ Tx: reduction (If S&S of radial nerve injury persist after reduction of the fracture в†’ open reduction & remove entrapped nerve) Colles Fracture в†’ Osteoporosis, fall on outstretched hand в†’ вЂњ dinner fork deformity в†’ Tx: close reduction and long arm cast Monteggia fracture в†’ direct blow to the ulna (eg. Someone hits you by stick) в†’ diaphyseal fracture of the proximal ulna , with anterior dislocation of the radial head в†’ Tx: close reduction of the radial head & open reduction and internal fixation of the ulnar fracture All rights reserved. Registered with US copy right office 71 оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ S.S.Patel. Galeazzi fracture в†’ fracture of the distal radius and dorsal dislocation of the radioulnar joint в†’ Tx: open reduction and fixation of the radius & casting of the forearm in supination to reduce dislocated joint. Carpel tunnel Syndrome: chronic use of hands (typist), DM, Hypothyroidism, Acromegaly вЂ“ eliciting pain by compressing median nerve at wrist / ask patient to do forced and prolong flexion of the wrist [best initial test] вЂ“ electromyography [most accurate test] вЂ“ Hand splinting at night [best initial treatment] вЂ“ PO prednisone has shown to improve symptoms Trigger finger в†’ Acutely flexed finger в†’ Tx: steroid injection / surgery de Quervain Tenosynovitis в†’ young mother, holding babyвЂ™s head with hand в†’ pain occur when asking her to hold her thumb in her closed fist & forcing the wrist into ulnar deviation в†’ Tx: splint & NSAIDs / steroid injection Dupuytren contracture в†’ contracture of the palm of the hand & palmar fascial nodule on palpation (hand can not placed on a flat table) в†’ Tx: surgery Felon в†’ abscess in the pulp of a fingertip в†’ throbbing pain в†’Tx: urgent surgical drainage is require Scaphoid fracture в†’ fall on outstretched hand в†’ tenderness over anatomic snuff вЂ“ box в†’ Tx: Thumb spica cast, with follow-up X-ray 3 weeks later (high rate of nonunion). If displaced and angulated fracture of the scaphoid в†’ open reduction & internal fixation Gamekeeper Thumb в†’ ulnar collateral ligament injury by forced hyper extension of the thumb в†’ collateral laxity at the thumb metacarpophalangeal joint в†’ Tx: cast Jersey finger в†’ flex finger is forcefully extended вЂ“ injury to flexor tendon в†’ Tx: splint Mallet finger в†’ Extended finger is forcefully flexed в†’ injury to extensor tendon в†’ Tx: splint Compartment Syndrome в†’ intense & persistent pain few hours after casting в†’ excruciating pain with passive movement of muscles в†’ Tx: fasciotomy Fourth & fifth metacarpal neck fracture в†’ hitting the wall with closed fist в†’ Tx: closed reduction & ulnar gutter splint (if mild) (or) K-wire / plate fixation (if severe) All rights reserved. Registered with US copy right office 72 оЂЉ оЂЉ оЂЉ Externally rotated short leg в†’ Hip fracture, femoral neck fracture, intertrochanteric fracture в†’ Tx: femoral head replacement with prosthesis in femoral neck fracture & open reduction and pinning in intertrochanteric fracture Internally rotated short leg в†’ Posterior dislocation of the Hip в†’ typically in car accident where knee hit the dashboard в†’ Tx: emergency reduction (to avoid avascular necrosis of femoral head) Medial collateral ligament injury: pain on direct palpation over the medial aspect of the knee в†’ Affected leg abducted more than normal leg (valgus stress test) в†’ MRI в†’ Tx: Hinged cast / repair пЃ® Avascular necrosis of femoral head: progressive hip pain with normal range of movements вЂ“ Causes: chronic steroid therapy, alcoholism, hemoglobinopathies вЂ“ diagnostic test? MRI of hip вЂ“ Tx: Core decompression (stage 1 or 2 вЂ“ positive radiograph without femoral head collapse) / Total hip replacement (stage 4 вЂ“ flattening of femoral head with joint space narrowing) пЃ® Lateral collateral ligament Injury: pain on direct palpation over the lateral aspect of the knee в†’ Affected leg Adducted more than normal leg (varus stress test) в†’ MRI в†’ Tx: Hinged cast / repair оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ оЂЉ S.S.Patel. Anterior Cruciate ligament Injury : leg can be pulled anteriorly (positive drawer sign) в†’ MRI в†’ Tx: immobilization / repair Meniscal Tears в†’ catching & locking that limit knee motion and a вЂњ click вЂќ when the knee is forcefully extended в†’ Tx: repair (complete meniscectomy в†’ degenerative arthritis) Posterior dislocation of knee в†’ popliteal artery injury в†’ Tx: reduction . Ankle fracture в†’ falling on an inverted / everted foot в†’ both malleoli break в†’ AP, lateral & mortise X-ray в†’ Tx: open reduction and internal fixation Achilles tendon rupture в†’ loud вЂњpopвЂќ sound (like a gunshot) в†’ palpation of Achilles tendon reveals an obvious defect right beneath the skin в†’ Tx : casting in equines position for several months / open surgical repair Metatarsal Stress Fracture в†’ H/O rigorous walking, marching Lumbar Spinal Stenosis: either the spinal canal (central stenosis) or vertebral foramen (foraminal stenosis) becomes narrowed вЂ“ compression of the nerves вЂ“ Elderly patient walking вЂњlike drunken sailorвЂќ, pain improves when lean forward (bending forward increases the space in the spinal canal and vertebral foramen) and gets worse by bending backward, best test? вЂ“ MRI of spine All rights reserved. Registered with US copy right office 73 оЂЉ Spinal Nerve root irritation (Radiculopathy) вЂ“ nerve may be inflamed or pinched (spinal stenosis, osteoarthritis of spine, herniated lumbar disk, osteoporosis, etc) вЂ“ eg. Sciatica вЂ“ shooting pain, weakness, numbness вЂ“ pain increased by bending forward and straining; decrease by lying down вЂ“ SLR test is positive at 60o or less оЂЉ Lumbargo (lower back pain): stiff & tender lower back muscles on palpation вЂ“ h/o recent heavy exercise вЂ“ Tx: NSAIDs, apply heat & early mobilization оЂЉ оЂЉ оЂЉ оЂЉ S.S.Patel. Herniated Lumbar disk в†’ L4 вЂ“L5 / L5 вЂ“ S1 в†’ Severe back pain (h/o heavy weigh lifting recently) without neurological deficit & intact perianal area; positive straight leg raising test, diagnosis? пѓ Herniated disc; Treatment? пѓ NASIDs & early mobilization Cauda Equina Syndrome в†’ Loss of bowel & bladder control & loss of sensation in perianal area в†’ Emergency decompression Motor Neuroma в†’ inflammation of the common digital nerve at the 3rd interspace between 3rd & 4th toes в†’ pointed , high heel shoes в†’ Tx: NSAIDS / surgical excision Traumatically Amputed Digits в†’ cleaned with sterile saline , wrapped in a saline вЂ“ moistened gauze , placed in a sealed bag (plastic) and then bag placed on ice в†’ surgically reattached All rights reserved. Registered with US copy right office 74 Nephrology п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Pre-renal Azotemia - в†‘ BUN but creatinine near normal (N) (0.6 вЂ“ 1.2) Post-renal Azotemia - в†‘ BUN & в†‘ creatinine Renal Azotemia вЂ“ BUN/Cr в‰¤ 15 ( bcoz more в†‘в†‘ in creatinine) Cortical necrosis of both kidney sparing medulla вЂ“ DIC Sickle cell anemia вЂ“ affect medulla most severly вЂ“ can cause Papillary necrosis Renal papillary necrosis (SADD) вЂ“ Sickle cell anemia, Acute pyelonephritis, Drugs (Aspirin + acetaminophen), Diabetes * Nephritic type Glomerular Disease : (moderate proteinuria & RBC cast) п‚§ IgA glomerulonephritis (BergerвЂ™s disease) (BuergerвЂ™s disease вЂ“ thromboangitis obliterans вЂ“ male вЂ“ smoking cigarettes) (both are different disease) вЂ“ episodic bouts of hematuria 1-3 days following URTI, slow progression to CRF (40-50%), mesangeal IgA deposit with granular immunoflurocence п‚§ Post-streptococcal вЂ“ Hematuria 1-3 weeks following group A Strep Pyogens infection - Skin infection - в†‘ anti-DNAase B titer - Pharynx infection - в†‘ ASO titer - Diffuse proliferative (usually resolve, CRF is uncommon) п‚§ Diffuse Proliferative (SLE) вЂ“ subendothelial Immune Complex (IC) (anti-ds DNA Ab) deposit with granular IF, вЂњwire loopingвЂќ of capillaries (CRF most common cause of death in SLE) п‚§ Rapidly Progressive вЂ“ crescent formation, associated with Goodpasture syndrome (linear IF) (Lower Resp Tract involvement, Hemoptysis followed by ARF), Polyarteritis nodosa (p-ANCA) (GIT involvement вЂ“ mesenteric artery, bowel ischemia, bloody diarrhea), WegnerвЂ™s granulomatosis (cANCA) (Upper & Lower Resp Tract involvement, perforation of nasal septum) * Nephrotic type Glomerular Disease: (proteinuriaвЂє 3.5 g/24 hrs & fatty cast) п‚§ п‚§ п‚§ п‚§ п‚§ S.S.Patel. Minimal change disease вЂ“ children вЂ“ EM show fusion of podocytes (selective proteinuria вЂ“ Albumin not globulin), negative IF Focal Segmental вЂ“ HIV, Heroin IV abuse, NSAID, HodgkinвЂ™s lymphoma вЂ“ negative IF, non-selective proteinuria Diffuse Membranous вЂ“ Adults вЂ“ captopril, HBV, malaria, syphilis вЂ“ subepithelial deposit with granular IF вЂ“ вЂњspike and domeвЂќ pattern Type-1 MPGN вЂ“ HCV, HBV, cryoglobulinemia вЂ“ subepithelial deposit вЂ“ EM show вЂњtram trackвЂќ (progress to CRF) Type-2 MPGN вЂ“ C3 nephritic factor (C3NeF), Ab binds to C3 convertase & prevent its degradation & sustain activation of C3 leads to very low C3 level. вЂњdense deposit diseaseвЂќ (progress to CRF) All rights reserved. Registered with US copy right office 75 п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Only in SLE, there is subendothelial deposit. In all others, there is subepithelial deposit Only in Goodpasture syndrome, there is linear deposit. In all others, there is granular deposit. Patient with Nephrotic syndrome has accelerated athrogenesis Palpable purpura, hematuria, Proteinuria, Hepatitis C вЂ“ diagnosis? пѓ Mixed essential cryoglobulinemia вЂ“ Tx: Mix cryoglobulinemia associated with Hep C вЂ“ Anti-viral drugs (Ribavirin and alpha-interferon) Polyarteritis nodosa вЂ“ p-ANCA вЂ“ HbsAg (+) in 30% of cases Lichen planus вЂ“ association with Hep. C Glomerular nodule вЂ“ DM / Amyloid (chronic disease) both show red on H&E stain but with Congo red stain вЂ“ Amyloid вЂ“ вЂњapple-greenвЂќ birefringence nodule. DM nodule is composed of Type-4 collagen & protein п‚§ Pathogenesis of DM: Nonenzymatic glycosylation (glucose + AA) в†’ в†‘vessel permeability to protein and в†‘athrogenesis; Osmotic damage в†’ Aldolase reductase (glucose в†’ sorbitol) в†’ sorbitol draws water into tissues causing damage (eg. retinopathy); Diabetic microangiopathy в†’ в†‘synthesis of type-IV collagen in basement membrane & mesangium Л— Tight blood sugar control decrease the risk of development of microvascular complication (retinopathy, nephropathy, neuropathy) Л— ACE inhibitors has shown to reduce insulin resistance п‚§ Screening test for nephropathy in DM: Random urine for microalbumin / creatinine ratio (goal <30 mg/g) п‚§ Earliest renal abnormality seen in diabetic nephropathy: glomerular hyper filtration п‚§ п‚§ п‚§ Ethylene glycol poisoning вЂ“ Metabolic acidosis (в†‘ anion gap) + oxalate crystalluria Cystinuria вЂ“ staghorn calculi, positive nitropruside cyanide test Staghorn calculi вЂ“ Proteus infection * Acid-Base Disturbances п‚§ (Na + K) вЂ“ (HCO3 + Cl) = 8-14 (normal anion gap) п‚§ Only chronic acidosis/alkalosis is compensated not acute п‚§ Chronic Resp. Acidosis вЂ“ compensated by Metabolic Alkalosis (HCO3 вЂ“ 22-28) PCo2 - вЂє 45 mmHg, HCO3 - в‰¤ 30 mEq/L вЂ“ Acute Resp. Acidosis HCO3 - вЂє 30 mEq/L вЂ“ Chronic Resp. Acidosis п‚§ Chronic Resp. Alkalosis вЂ“ compensated by Metabolic Acidosis PCo2 - вЂ№ 33 mmHg, HCO3 - в‰Ґ 18 mEq/L вЂ“ Acute Resp. Alkalosis HCO3 - вЂ№ 18 mEq/L (butвЂє12 mEq/L) вЂ“ Chronic Resp. Alkalosis S.S.Patel. All rights reserved. Registered with US copy right office 76 * Renal Tubular Acidosis (Normal Anion gap Acidosis) п‚§ Type-1 вЂ“ secondary to autoimmune diseases, Lithium, analgesics, sickle cell disease Inability to secrete H+ in Urine, Urine pH - вЂє 5.4 Patient usually gets Renal stone Acid load Test вЂ“ After giving ammonium chloride, urine pH still remain elevated (normally it should be decreased) п‚§ Type-2 вЂ“ Renal threshold for absorbing HCO3 is lowered from normal of 24 mEq/L to 15 mEq/L Initially pH вЂє 5.5 and then it goes back to вЂ№5.5 Patient usually get bone lesion (osteomalacia, rickets) Both Type-1 & Type-2 get Hypokalamia. п‚§ Type-4 is the only renal tubular acidosis which produce hyperkalamia due to destruction of JG apparatus - в†“ rennin - в†“ aldosterone Causes вЂ“ hyaline arteriosclerosis in afferent arteriole in DM, LegionaireвЂ™s disease п‚§ Intake of salt = output of salt (95% renal & 5% sweat) * п‚§ п‚§ п‚§ Hyponatremia вЂ“ Na вЂ№135 in the absence of hyperglycemia SIADH вЂ“ Oral hypoglycemic & Carbamazapine Diagnosis вЂ“ Urine osm вЂє Serum osm (Urine osm вЂє 40 is typical) Treatment вЂ“ fluid restriction (hyponatremia due to volume overload like in CHF), loop diuretics & normal saline, hypertonic saline, Lithium/Demeclocycline in SIADH. Rapid correction of hyponatremia results in Central Pontine Myelinosis вЂ“ destruction of brain stem present with paraparesis, dysarthria or Dysphagia. Hypernatremia вЂ“ loss of hypotonic fluids (sweating, burns, fever), central DI & Nephrogenic DI вЂ“ Best initial Tx of hypovolemic hypernatremia вЂ“ normal saline (0.9%) Hypokalamia вЂ“ вЂњUвЂќ wave on ECG, Alkalosis, в†‘ Aldosterone Hyperkalemia вЂ“ peaked T wave on ECG, Acidosis, в†“ Aldosterone вЂ“ IV calcium gluconate (first step) вЂ“ In Hyperkalemia, removal of k+ from the body can be achieved by dialysis, cations exchange resins (kayexalate) or diuretics. Hypercalcemia вЂ“ IV normal saline infusion (first step). (except hypercalcemia is due to Metastasis in which bisphosphonates preferred) Hypophosphatemia вЂ“ continuous infusion of glucose is the leading cause of hypophosphatemia; muscles weakness * * * * * * вЂў вЂў вЂў вЂў S.S.Patel. Hypercalcemia вЂ“ Loop diuretics / Hypercalciuria вЂ“ Thiazide diuretics Any time in question, hypokalamia, first step вЂ“ IV potassium Any time in question, hyperkalemia, first step вЂ“ IV calcium gluconate Hypocalcemia вЂ“ hyperactive deep tendon reflexes Hypermagnesemia вЂ“ loss of deep tendon reflexes All rights reserved. Registered with US copy right office 77 * Pheochromocytoma п‚§ a neuroendocrine tumor of the medulla of the adrenal glands п‚§ Signs & Symptoms of sympathetic hyperactivity (increase HR, HTN, etc) п‚§ Diagnosis: urinary vanillylmandelic acid (VMA) п‚§ CT scan/MRI вЂ“ to localize tumor п‚§ Confirmation of diagnosis with biochemical tests (24-hrs urine measurement of VMA, metanephrines & catecholamine) is required before imaging studies. Once diagnosis is confirmed, start alpha-blockers. п‚§ Treatment вЂ“ Alfa-blockade followed by surgical removal * Renal Artery Stenosis Л— Best screening test вЂ“ US abdomen (renal Doppler US) / captopril renogram (best non-invasive method) Л— Most accurate method вЂ“ MRI / CT angiography Л— Best initial treatment вЂ“ percutneous transluminal angioplasty * MCC of secondary HTN in children вЂ“ fibromuscular dysplasia (20% of all cases of renal HTN) вЂ“ angiogram typically show a вЂњstring of beadвЂќ appearance to the renal artery * п‚§ п‚§ п‚§ Important Urinary Cast Waxy, broad cast вЂ“ signs of End Stage Renal Disease WBC cast вЂ“ Acute pyelonephritis, acute tubulointerstitial nephritis (drug) Renal tubular cell cast (muddy brown granular cast) - ATN вЂў вЂў First step in management of patient with hematuria вЂ“ urine analysis Microscopic / gross painless hematuria, next step? вЂ“ CT urogram / IVP (to look kidney & ureter) and Cystoscopy (to look bladder) Most common cause of painless hematuria in adults in USA пѓ Bladder mass then Renal cell CA вЂў вЂў Patient with BPH doesnвЂ™t have hematuria. Presence of hematuria in a patient with BPH present with irritative or obstructive voiding symptoms вЂ“ always suspect bladder CA вЂў Chronic pyelonephritis: h/o vasicoureteral reflux + bilateral focal parenchymal scaring & blunted Calyces on IVP пЃ® вЂў вЂў вЂў вЂў Chronic Renal Failure (CRF): Diabetic nephropathy, HTN and glomerulonephritis [These 3 = 75% of cases] Volume overload leads to HTN and CHF Uremia (Pericarditis and encephalopathy) Hyperkalemia, Hyperphosphotemia and Hypocalcemia (Vit-D3 deficiency) (renal osteodystrophy) Metabolic acidosis and accelerated atherosclerosis Causes of bleeding in CRF вЂ“ platelet dysfunction вЂў вЂў S.S.Patel. All rights reserved. Registered with US copy right office 78 вЂў вЂў вЂў вЂў вЂў Increase Bleeding Time due to renal disease вЂ“ IV Desmopressin (first choice) CRF patient should receive erythropoietin & iron supplementation Anemia in patient with end-stage renal disease is due to erythropoietin deficiency. If there is no or minimal increase in hematocrit level after 4-6 wks treatment with EPO, iron saturation, ferritin level and TIBC should be measure [Side effects of Erythropoietin Therapy вЂ“ worsening of hypertension, Headache, flu-like syndrome, Red cell aplasia] Vitamin-D3 and calcium supplements, ACE inhibitors have been found to slow the progression of CRF Protein restriction improve prognosis in CRF оЂЉ Kidney stones вЂ“ plain X-ray KUB (first step); If KUB is normal but S&S suggestive of renal stone, next step? вЂ“ order CT scan without contrast; Pregnant patient with S&S of renal stone, next step? вЂ“ order U/S of abdomen оЂЉ Urethral Diverticulum вЂ“ h/o frequent UTI, tender suburethral mass вЂ“ classical triad of dribbling, dyspareunia and Dysuria вЂ“ order Cystourethrogram or Urethroscopy * п‚§ п‚§ п‚§ Juvenile Polycystic Kidney Disease Autosomal recessive Bilateral enlarged kidney at birth Maternal oligohydramnios * п‚§ п‚§ п‚§ п‚§ Adult Polycystic Kidney Disease Autosomal dominant Bilateral enlarged kidney around 20-25 yrs of age Cyst also present in liver (40%) Intracranial berry aneurysm (10-30%) (present with subarachnoid hemorrhage), HTN, sigmoid diverticulosis (Before doing peritoneal dialysis in a patient with APKD, patient should undergo colonoscopy first), MVP Follow up plan for patient with APKD вЂ“ regular BP measurement Screening test for APKD вЂ“ U/S п‚§ п‚§ * Multiple contrast filled cyst on IVP, diagnosis? пѓ Medullary Cystic kidney (Autosomal dominant, No Hypertension) * Unilateral flank mass in child > 3 yrs пѓ WilmвЂ™s tumor Bilateral flank mass in child пѓ polycystic kidney (infantile) Unilateral flank mass in Adults пѓ Renal cell CA Bilateral flank mass in Adults пѓ polycystic kidney (adult) * п‚§ п‚§ Renal cell CA (clear cell CA, hypernephroma, Grawitz tumor) Derived from proximal tubule (PT) Risk factors вЂ“ smoking, Von Hipple-Lindau syndrome, Adult polycystic kidney disease (APKD) S.S.Patel. All rights reserved. Registered with US copy right office 79 п‚§ п‚§ Hematuria, flank mass & CVA tenderness Metastasize to lung [cannonball appearance on x-ray], Lt sided varicocele * - Wilms Tumor: Derived from mesonephric mesoderm (unilateral flank mass) Histology: abortive glomeruli, & tubules, primitive blastemal cells, rhabdomyoblasts Hypertension in child, Autosomal Dominant (chromosome-11) WAGR Syndrome (Wilms tumor, Aniridia, Genital abnormalities, Retardation) * - Neuroblastoma: N-myc gene amplification. Small blue cell tumors (Ewing sarcoma, Lymphoma, Neuroblastoma, small cell CA of Lung). Composed of malignant neuroblast, presence of Homer-Wright rosettes. Neurosecretory granules on electron microscopy. Hypertension in child. в†‘в†‘в†‘ Urinary VMA (vanillylmandelic acid), HVA, metanephrines. п‚§ PotterвЂ™s syndrome вЂ“ absent of both kidney вЂ“ oligohydroamnios вЂ“ failure of ureteric buds to develop вЂў - Alport syndrome: X-linked dominant disorder Asymptomatic hematuria , Sensorineural hearing loss Renal biopsy: Glomerular Sclerosis , thickened basement membrane tubular atrophy fibrosis and foam cells. вЂў - - Hemolytic Uremic Syndrome ( HUS ): E.Coli (0157 : HS) в†’ produce Vero toxin в†’ endothelial cell injury Endothelial injury of the kidney results in localized clotting в†’ RBCs and intrarenal platelate damage causes microangiopathic anemia and thrombocytopenia Approximately 1 week after E.Coli ( 0157 : H7 ) infection , patient develop oliguria, sign & symptoms of anemia в†“ Hb level m drop in platelate count, Hematuria , proteinuria , Helmet cells on peripheral smear D/D: TTP which involves CNS whereas HUS involves kidney. вЂў - HenochвЂ“Schonlein Purpura: IgA вЂ“ mediated vasculitis of small vessels Non-thrombocytopenic purpura in children Usually follows an URI. Tetrad of Abd. Pain, Rash, Renal involvement & Thrombocytopenia. Palpable purpuric rash on buttocks Intussusception is more common in patient with HSP Tx: Corticosteroids - S.S.Patel. All rights reserved. Registered with US copy right office 80 вЂў Combination of obstruction & Infection of Urinary tract : Patient who is being allowed to pass a ureteral stone spontaneously & who suddenly develop chills , fever & flank pain. It is a medical emergency due to high chances of sepsis so order CT scan without contrast to establish diagnosis after starting IV antibiotics and hydration Л— Tx : IV antibiotics & immediate decompression of the urinary tract above the obstruction by either percutaneous nephrostomy or ureteral stent placement в– Post- op вЂ“ Zero urinary output в†’ plugged / kinked catheter. в– Post вЂ“ op вЂ“ Low urinary output with good perfusing pressure (SBPвЂє 90 mm Hg) в†“ в†“ в†“ Insufficient Fluid intake Acute Renal Failure в†“ в†“ Fluid challenge Test в†’ 500 ml of RL/NS IV in 10-20 mins в†“ в†“ в†‘ urinary output no change in urinary output в†“ в†“ + Na concentration в†’ < 20 mEq / L > 40 mEq /L S.S.Patel. All rights reserved. Registered with US copy right office 81 NEUROLOGY пЃ® CSF: Lat ventricle в†’ foramen of Monro в†’ 3rd ventricle в†’ Aqueduct of Sylvius в†’ 4th ventricle [obstruction to CSF flow will give hydrocephalus] п‚§ Arnold-Chiari malformation вЂ“ herniation of cerebellum вЂ“ hydrocephalus п‚§ Arnold-Chiari malformation type 2 вЂ“ syringomyelia, myelomeningocele п‚§ Syringomyelia is associated with Arnold-Chiari malformation in most cases. п‚§ Headache, 6th CN palsy, bilateral papilledema, empty sella & no mass lesion on MRI, Diagnosis? пѓ Pseudothmor cerebri (benign idiopathic intracranial hypertension); next step? пѓ Lumbar puncture (reduce intracranial pressure to prevent vision loss); Tx: Acetazolamide пЃ® Subfalcine herniation: cingulate gyrus herniates under falx cerebri [compress anterior cerebral artery] пЃ® Uncal herniation: medial portion of temporal lobe herniates through tentorium cerebellli [compress midbrain & posterior cerebral artery] пЃ® Tonsillar herniation: cerebellar tonsils herniates through foramen magnum [produce cardiorespiratory arrest] в– - Guillain вЂ“ Barre Syndrome: Auto immune destruction of myelin Begins in lower extremities and move upward Patient usually c/o pain / tingling Associate with C.Jejunii Best Initial test: lumbar puncture for protein and cell count Most Accurate test: Electromyography (EMG) Treatment: IV immunoglobulin / plasmaphersis вЂў Progressive ascending paralysis with normal CSF finding вЂ“ Tick-born paralysis в– - S.S.Patel. Myasthenia Gravis: Antibodies produce against Ach receptors C/o diplopia , ptosis , difficulty swallowing Symptoms are improved with rest Best Initial Test: Ach receptor antibody test Most Accurate Test: EMG в†’ decremental decrease in muscle fiber contraction on repetitive never stimulation Treatment : Anticholinesterase Drugs Glucocorticods / Azathioprine (take more time to reach peak action) Thymectomy (post-pubertal) Eaton-Lambert syndrome пѓ Antibodies to voltage gated Ca+2 channels in the presynaptic nerve terminal (pre-synaptic membrane damage), absent or diminished reflex, в†‘в†‘ muscle strength after repetitive task Myopathy in Lang CA пѓ muscle membrane damage, Normal reflex All rights reserved. Registered with US copy right office 82 в– - Huntington Disease: Autosomal Dominant Affect Caudate nucleus CAG trinucleotide repeat expansion Chorea & behavioral disturbance Onset in 4th or 5th decade Diagnosis в†’ Southern blot test to check DNA repeat expansion в– - Parkinson Disease: Degeneration of substantia nigra (в†“ dopamine) Imbalance b/w dopamine (в†“в†“) and cholinergic (в†‘в†‘) transmitters Bradykinesia , Cogwheel Rigidity , Resting tremor (pill rolling) , postural instability Shy-Drager Syndrome вЂ“ Parkinsonism + orthostatic hypotension Diagnosis в†’ clinical Treatment в†’ 1st step is evaluating the patientвЂ™s functional status Intact functional status (less Bradykinesia) вЂ“ Anticholinergics (Benztropin, Trihexyphenidyl) (< 60 yrs of age) / Amantidine (> 60 yrs of age) Compromised functional status (significant Bradykinesia) вЂ“ Carbidopa / levodopa (best initial drugs), Selegiline (only drug which arrest progression of disease), Surgery (pallidotomy, thalamotomy) Visual hallucination, confusion and agitation after starting treatment with Levodopa is highly suggestive of Lewy body dementia в– - S.S.Patel. Multiple Sclerosis: Focal areas of demyelination Optic neuritis, scanning speech, intention tremor, nystagmus Bilateral internuclear ophthalmoplegia [demyelination of MLF] [pathognomic] Blurry vision and double vision в†’ common initial manifestations of the disease в†’ resolve spontaneously CSF show oligoclonal bands (70 вЂ“90 %) Best initial Test : MRI of the Brain Best Diagnostic Test : MRI of the Brain Relapsing вЂ“ remitting disease в†’ IFN-B1a, IFN-B1b, glatiramer acetate Secondary вЂ“ progressive Disease в†’ IFN-B1b, Mitoxantrone (cardiotoxicity ) Primary вЂ“ Progressive Disease в†’ No approved therapy exist at this time Acute Exacerbation в†’ IV steroid X 3 days followed by oral medication X 4 weeks plasma Exchange Patient with exacerbation of MS вЂ“ IV steroids (study has shown that oral should not be offered in patient with MS who present with optic neuritis) then start interferon / glatiramer acetate (both are teratogenic) and follow-up with MRI after 3-months; optic neuritis as an initial presentation of MS has a good prognosis Spasticity в†’ beclofen ; Nocturnal Spasticity в†’ Diazepam , Tizanidine Trigeminal neuralgia в†’ carbamazapine ; Fatigue в†’ Amantidine / fluoxetine Bladder ltyperactivity в†’ Oxybutynin; Urinary retention в†’ Bethanechol All rights reserved. Registered with US copy right office 83 в– Marcus-Gunn phenomenon вЂ“ dilatation of Rt. Pupil with dilatation of Lt. Pupil occur (Paradoxical dilatation) in patient with Rt. Optic neuritis/ Rt. retinal detachment пЃ® Alzheimer Disease: defect in degradation of ОІ-Amyloid protein by secretase leads to accumulation of Amyloid protein in neuron and damage neurons вЂ“ mutation in Tau protein (maintain microtubule in neuron) leads to formation of neurofibrillary tangles вЂ“ Microscopic [senile plaques (Amyloid protein) and neurofibrillary tangles] вЂ“ Problem in memory [affect hippocampus (old brain) short term memory loss] & visuospatial abilities (early); Hallucination & Personality change (late) вЂ“ diagnosis of exclusion [rule out reversible cause of dementia (first step) so order CBC, electrolytes, Ca+2,Creatinine, LFTS, Glucose, TSH, Vit-B12 ,RPR, HIV вЂ“ Tx: Donepezil (discontinue if no improvement within 3-6 months of treatment) пЃ® Pick Disease: frontotemporal dementia в†’ Pick body (Intracytoplasmic spherules composed of paired helical filaments) в†’ Present with Personality change пЃ® Lewy body dementia: Lewy body (Intracytoplasmic spherules that stain brightly eosinophilic) в†’ fluctuating cognitive impairment [confusion and agitation] which can be confused with delirium пЃ® Creutzfeldt Jacob Disease (CJD): Shorter & more aggressive course, present with dementia & myoclonus пЃ® Vascular Dementia: h/o multiple strokes вЂ“ multi-infarct dementia пЃ® Extrapyramidal symptoms + dementia = subcortical dementia (Parkinsonism) пЃ® Binswanger Disease: involve subcortical white matter, slow course пЃ® Normal pressure hydrocephalus: dementia, gait abnormality and Urinary incontinence вЂў Appearance of CT scan in different kind of dementia: 1. Enlargement of ventricle without cortical atrophy вЂ“ normal pressure hydrocephalus 2. Hypodense images involving different brain regions вЂ“ Multi-infarct 3. Marked atrophy of the frontal and temporal cortices вЂ“ Pick 4. Diffuse cortical and subcortical atrophy вЂ“ Alzheimer пЃ® KrabbeвЂ™s Disease: presence of вЂњgloboidвЂќ cells (multinucleated histiocytic cells) in degenerating white matter in brain вЂ“ galactocerebrosidase deficiency пЃ® Л— Л— Л— Cerebrovascular Accident : Sudden onset of focal neurologic deficit Hypertensive (Lacunar stroke) пѓ limited / mild neurologic deficit Large artery atherosclerosis / cardiac emboli пѓ severe clinical manifestation Yong patient with TIA (neurologic deficit resolve in 24 hrs), cause? пѓ Emboli (MCC) investigation? пѓ transthoracic echo (other cause vasculitis, dissection, hypercoaguble state) Л— Elder patient with TIA, cause? пѓ Atherosclerosis & Emboli; investigation? пѓ Carotid Doppler ultrasonography Л— Next step in management of patient with stroke whose carotid Doppler imaging is normal вЂ“ echocardiography S.S.Patel. All rights reserved. Registered with US copy right office 84 Л— Best Initial Test / Next Step: Non-contrast CT Scan of the Head (detect blood in the brain) Л— Most Accurate Test : Diffusion вЂ“ weighted MRI ( Detect ischemia) Л— Treatment : tPA(within 3 hrs of onset of Symptoms if not contraindicates) Heparin (if not contraindicated) Anti-platelate Therapy (Aspirin / clopidogrel) Carotid endarterectomy (Occlusion > 70 % & Symptomatic) п‚§ Anti-hypertensive drugs should only be used in patient with hвЂ™gic stroke if BP is over 220/120 п‚§ First step in management of patient with unconsciousness or Seizures or focal neurologic findings [after stabilization] пѓ CT scan without contrast п‚§ Serum glucose level should be measured in all patients present with CNS dysfunction [CNS dysfunction due to hypocalcemia, next step? - measure serum glucose] пЃ® Spinal Cord compression: neurologic emergency Л— Pain which в†‘ by action that в†‘ intrathoracic pressure в†’ в†‘ CSF pressure Л— H/O cancer, fever, bowel / bladder incontinence, UMN lesion sign below compression Л— High вЂ“ dose dexamethasone в†’ immediately once diagnosis is suspected Л— X-ray Spine ( Best initial test ) Л— MRI Spine ( Most Accurate test ) Л— CT myelogram (when MRI is C/I) Л— Surgical Decompression в†’ Herniated disk, epidural abscess , Hematoma Л— Hyperextension injuries in elderly patients with degenerative changes in cervical spine вЂ“ Central Cord Syndrome (Acute cervical spine cord injury) вЂ“ characterized by disproportionately greater motor impairment in upper compared to lower extremities, bladder dysfunction, and variable degree of sensory loss below the level of injury в– Subacute Combined Degeneration: Vit вЂ“ B12 deficiency - deficit of vibration & proprioception в– Ant Spinal artery Infarct: - Acute onset of flaccid paralysis that evolves into a spastic paresis over days to weeks - Loss of pain & temp. Sensation with Sparing of vibration and position sense. пЃ® Tabes Dorsalis: dorsal column of spinal cord вЂ“ tertiary syphilis пЃ® Seizures & Epilepsy : Л— Postictal Symptoms (c/o disorientation, sleepiness, muscle aches) for mins to hrs differentiate Seizure from Syncope S.S.Patel. All rights reserved. Registered with US copy right office 85 Л— ToddвЂ™s paralysis: Acute onset of paralysis in patient gaining consciousness after short period of unconsciousness and resolvement of paralysis after few hours, Cause? вЂ“ Seizure Л— Child with febrile seizures have slightly increase risk of epilepsy in future compare to general population Л— Absence Seizures (Petitmal): Sudden, brief loss of consciousness without loss of postural tone (stare in the space). EEG will show generalized, Symmetric 3-Hz spike and wave discharge pattern Л— EEG( electroencephalogram ) в†’ best diagnostic test for epilepsy Л— Treatment : Acute attack в†’ Secure A,BC (first step ) Л— Lorazepam or Diazepam (IV) в†’ Best initial drug Л— Phenytoin / fosphenytion в†’ Phenobarbitol в†’ Midazolam / Propofol Л— Generalized tonicвЂ“clonic (grandmal) в†’ Valpronic acid / lamotrigine Л— Absence Seizure в†’ Ethosuximide or Valproic acid Л— Partial Seizures (Simple / complex) в†’ carbamazapine / phenytoin Л— Myoclonic Seizures в†’ Valproic acid Л— Atonic Seizures в†’ Valproic acid вЂў Patient with epilepsy which is well controlled with anti-epileptic drug comes to you at 13-15 wks of pregnancy for first time for routine care, next step? вЂ“ continue her anti-epileptic drug and offer alpha-feto protein. [If there is a chance of any malformation, pregnancy can be terminated] Breast feeding is not C/I when patient is on anti-epileptic drugs пЃ® Л— Л— Л— Vertigo & Dizziness : Vertigo в†’ c/o room (environment ) is spinning around Presyncope в†’ c/o lightheadedness / feeling like IвЂ™m going to black out Central Vertigo (Cerebellar lesion/ Brain stem lesion) в†’ gradual onset, Absent tinnitus, no hearing loss, signs of CN lesions, vertical nystagmus Л— Peripheral Vertigo в†’ Sudden onset, tinnitus & hearing loss present, horizontal nystagmus. пЃ® Meniere disease в†’ Tinnitus , Hearing loss & Episodic Vertigo Л— Symptoms wax & wane as endolymphatic pressure rise & fall Л— Tx: low salt diet & Diuretics [Syphilis can cause Meniere disease] пЃ® Benign Paroxysmal Positional Vertigo в†’ Head movements lead to vertigo (1060 sec) вЂ“ Tx: Positional maneuvers пЃ® Labyrinthitis в†’ severe vertigo, hearing loss & tinnitus follows an URI Л— Tx : meclizine & diazepam оЂЉ S.S.Patel. Temporal Arteritis (Giant Cell Arteritis) вЂ“ vasculitis of superficial temporal artery and ophthalmic artery в†’ Unilateral pounding headache, Visual changes, All rights reserved. Registered with US copy right office 86 Jaw claudication, scalp tenderness, ESR >60 mm/hr в†’ Tx: в†’ High dose Prednisone (1st step) оЂЉ - Migraine Headache в†’ unilateral throbbing, 4-72 hrs Scintillating scotoma в†’ Migraine with Aura Tx : Acute attack в†’ Sumatriptan / Ergotamine Prophylaxis (> 3 headache / month) в†’ Propranolol / Valproic acid / methysergide Tension вЂ“type Headache в†’ bilateral tight band like headache with tightness of posterior neck muscles . Л— Tx : Relaxation Activities, Acetaminophen (NSAID), muscle relaxant оЂЉ пЃ® Cluster Headache в†’ excruciating , periorbital with rhinorhea , reddening of the eye , nasal stuffiness , peaking in intensity in 5 min usually last 45-90 min , 1-3 times/day for 4-8 weeks Л— D/D: Acute angle closure glaucoma & cluster headache may confuse, but halos around light & fixed dilated pupils are seen in acute angle closure glaucoma. Cluster headache are recurrent Л— Tx : Acute attack в†’ 100 % O2 Prophylaxis в†’ Prednisone, lithium, Verapamil, methysergide, ergotamine пЃ® - Tuberous Sclerosis: Infantile spasm (Tx : ACTH & Prednisone) Rhabdomyoma of Heart (echocardiography) Ash leaf spots (Hyperpigmented lesions), shagreen patches (вЂњorange-peelвЂќ lesions), sebaceous adenomas. Angiofibroma on the face Angiolipomas in the kidney Astrocyte proliferations in subependyma (Look like вЂњcandlestick drippingsвЂќ in the ventricles) пЃ® Neurofibromatosis (NF): - CafГ© au lait spots (tan/light brown flat lesion), Axillary freckling, Lisch nodules, optic nerve gliomas, Acoustic Neuroma (CN-8)(feature of NF-2)(all other NF-1). - Association with Pheochromocytoma, Wilms tumor пЃ® - Duchenne Muscular Dystrophy: Pseudohypertrophy of the calves. Gower Sign (child places hands on the knees for help in standing). Deficiency of dystrophin. пЃ® Becker Muscular Dystrophy: - Defective dystrophin - Less serious than Duchenne muscular dystrophy S.S.Patel. All rights reserved. Registered with US copy right office 87 пЃ® Warding вЂ“ Hoffman Disease: - Infantile spinal muscular atrophy. - Atrophy of anterior horn cells in the spinal cord and of motor nuclei in the brainstem. - Severe hypotonia and absent tendon reflexes. - Legs tend to lie in a Frog leg position. пЃ® Л— - Charcot-Marie Tooth: Hereditary motorвЂ“sensory neuropathy. Peroneal muscular atrophy. Peroneal & Tibial nerve most commonly affected. Wasting of the lower legs giving them stork like appearance. Sural nerve biopsy в†’ вЂњonion bulbвЂќ formation (interstitial hypertrophic neuropathy) пЃ® - Friedreich Ataxia: Expanded GAA triplet repeats Autosomal recessive male child with gait disturbance & dysarthria (speech difficulty) Cardiomyopaty (90% of cases) пЃ® Ataxia Telangiectasia: - Mutation in DNA repair enzyme, thymic hypoplasia - Cerebellar ataxia, Telangiectasia of skin & eye пЃ® CNS tumors: Adult (above tentorium cerebelli) Children (below tentorium cerebelli) Most common [Adult вЂ“ glioblastoma multiforme, meningioma; Children вЂ“ Astrocytoma, Medulloblastoma] * * * * * * * * * * S.S.Patel. Oligodendroglioma вЂ“ вЂњfried egg cellвЂќ round nuclei & clear cell вЂ“ cerebral hemisphere Choroid plexus papilloma вЂ“ papillary growth in ventricle Ependymoma вЂ“ pseudorosettes & structure resembling ependymal canal Glioblastoma multiforme вЂ“ hemorrhagic tumor (multiple area of necrosis & cystic degeneration) Pilocytic Astrocytoma вЂ“ bipolar cells вЂ“ cerebellum of young children Medulloblastoma вЂ“ most common in children вЂ“ only CNS tumor with both neural & glial components вЂ“ affect granular cell layer of cerebellum Meningioma вЂ“ associated with neurofibromatosis вЂ“ parasagital location Craniopharyngioma в†’ remnant of RathkeвЂ™s pouch (Resembles to Amblioblastoma) в†’ calcified lesion above the sella on X-ray в†’ bitemporal hemianopsia Pineal gland tumor в†’ loss of vertical gaze (вЂњsunset eyesвЂќ) [Parinaud Syndrome] Acoustic neuroma пѓ MRI with gadolinium пѓ surgery All rights reserved. Registered with US copy right office 88 в– CNS bleeds: * * * * * Epidural: skull fracture вЂ“ rupture middle meningeal artery Subdural: tear of bridging veins вЂ“ fluctuating levels of consciousness Atherosclerotic stroke: usually pale infarct (since no reperfusion) Embolic stroke: hemorrhagic infarct extends to surface of the brain Intracerebral bleed: hypertension most common cause вЂ“ rupture of lenticulostriate Charcot-Bouchard aneurysms вЂ“ hematoma (not an infarct) вЂ“ globus pallidus/putamen area most common sites Л— Hemorrhage in brain parenchyma: Л— Putamen hemorrhage (most common site) вЂ“ almost always involve internal capsule. Hemiparesis, hemi-sensory loss, homonymous hemianopsia, stupor and coma Л— Pontine hemorrhage вЂ“ pinpoint pupil, deep coma and paraplegia within few min. There is decerebrate rigidity Л— Cerebellar hemorrhage вЂ“ ataxia, occipital headache, gaze palsy and facial weakness (no hemiparesis) Л— Subarachnoid hemorrhage вЂ“ вЂњworst headache of my lifeвЂќ * Subarachnoid bleed: ruptured congenital berry aneurysm [junction of communicating branch with anterior cerebral artery] вЂ“ severe occipital headache вЂ“ common in patient with polycystic kidney disease вЂ“ Major cause of morbidity & mortality in a patient with subarachnoid hemorrhage (SAH) пѓ Vasospasm following SAH; Nimodipine is used to prevent vasospasm following SAH Л— High RBC count without xanthochromia вЂ“ traumatic LP Л— RBC with xanthochromia and discoloration of centrifuged CSF due to Hb breakdown вЂ“ Subarachnoid hвЂ™ge * Syncope (Loss of Consciousness): it may be acute or gradual вЂ“ EKG (Best initial test / first step in management) вЂ“ causes of acute syncope [Arrhythmia, Mitral valve prolapsed, Hypertrophic obstructiove cardiomyopathy and seizure] gradual syncope [hypoglycemia, hypoxia, hypovolemai, vaso-vagal attack, barbiturates, etc] вЂ“ early regaining of consciousness is seen in syncope due to heart problem which is helpful in differentiate acute syncope due to seizure (gradual regaining of consciousness) * Vasovagal syncope вЂ“ Recurrent syncopal attacks provoked by emotions preceded by prodrome (light headedness, blurred vision) вЂ“ upright tilt table test S.S.Patel. All rights reserved. Registered with US copy right office 89 PSYCHIATRY вЂў IQ : Average(90 вЂ“ 109) (50 % of population ) - Add 10 for High average , Superior , Very Superior (2.5 % population ) (110-119) (120-129) (over 130) - Autistic children в†’ IQ less than 70 вЂў Commonly used IQ test: - WAIS вЂ“ R вЂ“ Adult ( > 17 yrs ) - WPPSI вЂ“ Pre вЂ“ School & Primary ( 4 вЂ“ 6 yrs ) - Stanford вЂ“ Binet Scale вЂ“ children ( 2 вЂ“ 6 yrs ) - WISC вЂ“ R вЂ“ children ( 6 вЂ“17 yrs ) в– Mood Disorders: Depression or Mania (hyper) вЂў Dysthymia (non-psychotic depression) в†’ chronic (atleast 2 yrs) вЂў - Unipolar (Major depression) в†’ Symptoms for atleast 2 weeks Early morning waking, pseudodementia in elderly в– Cyclothymia (non-psychotic bipolar) в†’ chronic (atleast 2 yrs) - ego-syntonic в– Bipolar вЂ“ Manic Symptoms + Major depression (bipolar-1) (bipolar-2) - Presence of maniac symptoms put patient in bipolar category в– Schizophrenia: Delusion, illusion, hallucination (Auditory 75 %), Blunted affect, loose associations. в– Differential: Schizophrenia - > 6 month of symptoms Schizopheniform - < 6 month of symptoms Brief Psychotic - < 1 month of symptoms Schizoid Personality вЂ“ life long pattern of social withdrawal seen by others as eccentric, isolated Schizotypal personality вЂ“ Magical thinking, illusions, ideas of Reference, very odd, strange, weird Schizoaffective вЂ“ Alteration in mood is present during substantial Portion of illness + Sx of Schizophrenia S.S.Patel. All rights reserved. Registered with US copy right office 90 вЂў Subtypes of Schizophrenia: - Paranoid вЂ“ Delusions of persecution (or) grandeur, hallucinations (voice) - Catatonic вЂ“ Rigidity of posture, Complete stupor, may be mute - Disorganized вЂ“ uninhibited, unrecognized (disorganized) behavior & speech, poor personal appearance, little contact with reality. - Undifferentiated вЂ“ psychotic symptoms but doesnвЂ™t fit in above three category. - Residual вЂ“ Previous episode but no prominent psychotic symptoms at evaluation, some negative symptoms. вЂў Positive Symptoms: Delusion, hallucination, bizarre behavior (dopamine) вЂў Negative Symptoms: Flat affect, apathy, mutism (muscarinic receptor) вЂў Brain structural and Anatomic abnormalities: - Large ventricle size, cortical atrophy, smaller volume of left hippocampus and amygdala, Atrophy of temporal lobe. - Limbic system seen as the primary pathology site for schizophrenia * Delusional Disorders: No hallucination, Delusions are not bizarre (like IвЂ™m a millionaire (believable - could be possible) whereas in Schizophrenia bizarre delusion like IвЂ™m a king of Moon (not believable) and patient is not functioning. п‚§ п‚§ п‚§ вЂў вЂў вЂў вЂў Somatoform Disorder: Production of somatic symptoms (like abdominal pain, headache, etc) вЂ“ extensive diagnostic procedures fail to show any pathology вЂ“ constantly change their primary physicians вЂ“ unconscious symptom production Factitious Disorder: intentional symptoms production but there is no motivation to produce symptoms means symptom production is not for any gain Malingering: Intentional symptoms production for gaining something Munchausen Syndrome: Factitious disorder вЂ“ usually medical field person (nurse) Munchausen Syndrome by proxy: mother produce symptoms in her baby Conversion Disorder: One (or) more NEUROLOGIC symptoms (eg. paralysis of half of the body) without any real organic cause. Loss of functioning is real & unfeigned Body dysmorphic disorder: persisten subjective feeling of ugliness or physical defect в– Post-Traumatic Stress Disorder: Flashback, Avoidance of associated stimuli вЂ“ Symptoms must be exhibited for > 1 month after passing few months of traumatic event вЂў Acute stress Disorder: symptoms for < 1 month soon after traumatic event. вЂў Adjustment disorder вЂ“ Symptoms must occur within 3 months of stressors and must not last more than 6 months вЂ“ Tx: Supportive Psychotherapy S.S.Patel. All rights reserved. Registered with US copy right office 91 в– Substance Abuse: - Cocaine, amphetamine, cannabis, hallucinogens and PCP (phencyclidine) в†’ paranoia (Schizopheniform delusion disorder) - Cannabis, inhalants, hallucinogens and PCP do not have withdrawal reactions. - Toxicology screening вЂ“ urine immunoassay qualitative screen [rapid and inexpensive] * Cocaine вЂ“ Intoxication в†’ Paranoia, arrhythmias Withdrawal в†’ Depression * Amphetamine вЂ“ Intoxication в†’ Paranoia, pupillary dilatation Withdrawal в†’ Depression * Cannabis вЂ“ Slowed reaction time, social withdrawal, injected conjunctiva * Hallucinogens вЂ“ Ideas of reference, brilliant color hallucination, pupillary dilatation. Withdrawal вЂ“ flash back (feels same as they are on drug without drug) * Opiates вЂ“ Intoxication в†’ pupillary constriction, respiratory arrest, bradycardia, hypotension (due to vasodilation) Withdrawal в†’ flu-like symptoms Tx: Clonidine, methadone вЂў IV drug abuser admitted in hospital and after few hours develop dilated pupils and other symptoms (like piloerection, rhinorhea, abdominal cramps, etc) вЂ“ opioid withdrawal [Tx: Methadone / other opioids] * * PCP вЂ“ violence, vertical nystagmus вЂ“ Next step in management of patient with PCP intoxication вЂ“ put patient in low-sensory environment Benzodiazepines вЂ“ Intoxication в†’ inappropriate sexual / aggressive behavior Withdrawal в†’ seizures - Sleep: Sleep cycle is regulated by super chiasmatic nucleus. Non-REM в†’ 1st half, REM в†’ 2nd half (REM cycle в†’ 90 mins) Adults wake out of REM / 2nd stage of non-REM REM в†’ Rapid eye movement вЂ“ eye is a part of brain so brain is active & body is inactive (в†‘ eye movement, в†“ muscle tone) NREM в†’ Opposite to REM means brain is inactive & body is active (в†‘muscle tone) REM sleep is requiring for memory Melatonin в†’ inhibited by Daylight (responsible for jetlag) Elderly в†’ REM вЂ“ 20 % (constant), stage - 4 (delta) вЂ“ Vanish (в†“) вЂў - Neurotransmitters: Serotonin вЂ“ initial sleep ACh вЂ“ REM sleep Norepinephrine - в†“ in REM sleep Dopamine вЂ“ Arousal & wakefulness в– - пЃ® Sleep Waves: Л— Awake в†’ low-voltage fast waves вЂ“ Beta waves S.S.Patel. All rights reserved. Registered with US copy right office 92 Л— Л— Л— Л— Л— Drowsy в†’Alpha waves (8-12 cps) (closing the eyes) Stage -1в†’Theta waves (3-7 cps) Stage -2в†’ Sleep spindles & K complexes. (12-14 cps) Delta sleep в†’Delta waves (1/2 вЂ“2 cps) [Delta вЂ“ D means Deep sleep] REM sleep в†’low-voltage fast with saw tooth waves. п‚§ Somnambulism: sleep walking вЂ“occur during stage-4 (Delta sleep). п‚§ Nightmare: REM sleep вЂ“ person often awake вЂ“ dreaming (dreams of frightening nature) вЂ“ can recall п‚§ Night terror: NREM sleep (stage 4) вЂ“ person often impossible to fully awaken вЂ“ canвЂ™t remember п‚§ - Narcolepsy: excessive daytime sleepiness and abnormalities of REM sleep greater than 3 months. REM sleep occurs in less than 10 mins Cataplexy в†’ Sudden loss of muscle tone Sleep paralysis Falling asleep quickly at night Tx: Psychostimulant; If cataplexy present в†’ Antidepressants вЂў - Modeling / Observational Learning ( Social Learning ): victim of abuse is now abusing his/her own child в– Defense Mechanisms: вЂў Projection вЂ“ Paranoid behavior [Do not trust anyone!] вЂў - Introjection вЂ“ try to act like someone eg: Students act like the resident вЂў - Denial вЂ“ denies every thing eg: I know I donвЂ™t have cancer [even though patient have cancer] вЂў - Isolation of Affect вЂ“ no reaction on their face! Blunted affect (Schizophrenia) вЂў - Splitting вЂ“ split their behavior! Sometime very good Sometimes very bad Borderline personality вЂў Blocking вЂ“ Transient block on our memory! Л— eg: Oh! WhatвЂ™s his name? I canвЂ™t seem to remember his name S.S.Patel. All rights reserved. Registered with US copy right office 93 вЂў - Repression вЂ“ nonretrievable forgetting eg: sexually abuse girl in her childhood doesnвЂ™t remember anything in her adulthood вЂў - Suppression вЂ“ Forgetting is reversible eg: I went out for movie before the day of my anatomy exam but after coming back I again started my reading [remembered that I have exam tomorrow!] вЂў вЂў Л— Regression вЂ“ Returning to an earlier stage of development eg: Enuresis in previously toilet train child after birth of another baby Somatization вЂ“ productions of somatic symptoms [somatoform disorder] eg: Just thinking of six flag I get Butterflies in my stomach вЂў - Displacement вЂ“ source stays the same, target changes eg: My elder brother angry at me and I angry at my younger one вЂў - Acting out вЂ“ doing something to hide emotion! eg: Whistling in dark who is afraid of going in darkness вЂў - Rationalization вЂ“ Explanations are used to justify unacceptable behavior eg: USMLE step 1 exam was hard thatвЂ™s why I failed [Not coz he / she didnвЂ™t read!] вЂў - Reaction formation вЂ“ Unacceptable transformed into its opposite eg: Feel love but show hate вЂў - Undoing вЂ“ Action to symbolically reverse the unacceptable eg: I need to wash my hands whenever I touch something Obsessive- compulsive behavior вЂў - Dissociation вЂ“ Separating self form oneвЂ™s own experience eg: Raped victim describe event as she is watching whole things form the roof Fugue, depersonalization вЂў - Humor вЂ“ A pleasant release from anxiety eg: Laughter hides the pain вЂў - Sublimation вЂ“ Unacceptable action transform into acceptable action eg: person who likes pornography becomes director of sensor board вЂў Altruism вЂ“ Individual provides a helpful, gratifying service to others as a means of quelling their own anxiety. Eg: Alcoholic cirrhotic patient works as a volunteer for a non profit organization that assist patient with alcoholic cirrhosis S.S.Patel. All rights reserved. Registered with US copy right office 94 в– Personality Disorders: вЂў - Paranoid Personality вЂ“ suspicious in nature Coldness & distance in most relationship Very hostile reaction to other peopleвЂ™s innocent or even positive act. вЂў Narcissistic personality вЂ“ Person feels вЂњentitledвЂќ to recognition. They often believe they are so special that others are jealous of their talents. They do not tolerate perceived rejection well. вЂў вЂў Histrionic Personality вЂ“ excessive emotion and attention seeking Seductive behavior - Women > Men Borderline Personality вЂ“ mood swings, unstable relationship, recurrent suicidal behavior - Women > Men вЂў - AntiвЂ“social Personality вЂ“ continuous antisocial (or) criminal acts Men > Women вЂў Avoidant Personality вЂ“ Social inhibition, fear of rejection, shy вЂў Dependent Personality вЂ“ Submissive, gets others to assume responsibility, canвЂ™t express disagreement. вЂў ObsessiveвЂ“Compulsive Personality вЂ“ Perfectionist, orderly, rigid, inflexible вЂў Obsessive-Compulsive anxiety disorder (OCD) вЂ“ has Obsession (persistent thoughts) and Compulsion (repetitive acts). Obsession and compulsion that are focal and acquired and have functional impairment whereas obsessive-compulsive personality disorder is life-long and less functional impairment в– Impulse Control Disorders: вЂў Intermittent Explosive Disorder вЂ“ Failure to resist aggressive impulses that result in serious assaultive acts вЂў - Kleptomania вЂ“ Failure to resist impulses to steal objects that the patients does not need Associated with Eating disorder, mood disorders, OCD вЂў Pyromania вЂ“ characterized by deliberate fire setting on more than one occasion вЂў Trichotillomania вЂ“ characterized by pulling oneвЂ™s own hair, resulting in hair loss patient may eat hair, resulting in bezoars stomach, obstruction & malnutrition S.S.Patel. All rights reserved. Registered with US copy right office 95 вЂў Oppositional defiant disorder вЂ“ frequently loss their temper, disobey their elders, deliberately annoy others, no violation of rules вЂў Conduct Disorders вЂ“ disregard rights and rules of the society, вЂ№18 yrs of age вЂў Anti-Social вЂ“ disregard rights and rules of the society, вЂє 18 yrs of age вЂў Selective Mutism вЂ“ speak normally in other situation or at home вЂў Autism вЂ“ вЂ№3 yrs of age, repetitive behavior, marked hearing impairment вЂў Childhood disintegrative disorder вЂ“ period of normal development for atleast 2 yrs followed by loss of previously acquired skills like expressive or receptive language, social skill, play & motor skills. вЂў Undetected hearing impairment вЂ“ hereditary, repeated ear infection, symptoms are same as autism but detected at later age compare to autism * Kubler-Ross Stage of adjustment in dying patient: 1. 2. 3. 4. 5. Denial Anger Bargaining Depression Acceptance ( DABDA) вЂў Any stage can occur first. (this order is not necessary) вЂў Fuge: Person has an abrupt change of geographic location (or) identity without alteration in consciousness (or) memory change * Delirium вЂ“ Acute onset, impaired cognitive functioning, fluctuating & brief вЂ“ Reversible вЂ“ Mx: order basic laboratory wok-up вЂ“ Benzodiazepine for 3-5 days вЂ“ usually Haloperidol is not recommended as first-line drug but If patient become more combative then use Haloperidol before applying restraints * Suicide rate is high in native American adolescence вЂў Anorexia Nervosa вЂ“ distorted body image (perception problem вЂ“ even with very thin body, patient looks herself as a fatty person), 15-20 % loss of body weight вЂ“ Complication: osteoporosis (Ca+2, estrogen deficiency), elevated carotene and Cholesterol, euthyroid sick syndrome, amenorrhea (estrogen deficiency), small for gestational age baby, Cardiac arrhythmia (due to Hypokalemia) (hypomagnesemia is another electrolyte abnormality) вЂ“ First step in management of patient with Anorexia Nervosa вЂ“ Hospitalization (weight gain & prevent complication) вЂ“ Refeeding syndrome вЂ“ develop in second-third week after initiation of feeding in patient with anorexia nervosa вЂ“ patient develop edema and heart failure вЂ“ should be treated with Phosphate supplementation S.S.Patel. All rights reserved. Registered with US copy right office 96 в– Bulimia Nervosa вЂ“ Binge & purge вЂ“ looks healthy compare to anorexia вЂ“ presence dental caries, excoriation on knuckle of hand due to repeated vomiting вЂ“ Level of serotonin is decreased in Bulimia thatвЂ™s why SSRIs are good choice for Bulimia. в– Eating disorder, not otherwise specified: features suggestive of both anorexia and bulimia, but doesnвЂ™t meet the criteria for one specific eating disorder ANTIPSYCHOTIC MEDICATIONS в– Mood Disorders : в– - Lithium ( DOC ) (C/I in pregnancy), Divalproex (2nd choice ) In pregnancy в†’ Clonazepam, Gabapentin M/A: в†“ PIP 2 в†’ в†“IP3 & DAG в†’ в†“ c AMP [ renal v2 receptor coupled to cAMP] AE : Goiter & Hypothyroidism, Teratogenic Nephrogenic DI [Treat with Amiloride (K+ sparing) not Thiazide] Low Na + в†’ enhance toxicity Concentration >4 mEq/L require emergent dialysis в– Anti- Depressants : ( Depression в†’ Functional deficiency of NE/5HT in Brain ) вЂў - MAO Inhibitors: (Phenelezine, Tranylcypromine) AE: Hypertensive crises with Tyramine Severe drug reaction with Meperidine вЂў - TCA s (Tricyclic Antidepressants ): MA : inhibit re-uptake of 5- HT and NE Amitriptyline, Imipramine, Clomipramine, Amoxepin, Doxepin USES: Major depression, Phobic & panic anxiety state, Neuropathic pain, Enuresis (Imipramine), OCD (clomipramine) similar to Phenothiazines (M block , О± block , sedation , в†“ seizure threshold) Cardiotoxicity (вЂњquinidine likeвЂќ), SIADH вЂў вЂў S.S.Patel. SSRIs: (Fluoxitine, Sertraline, Citalopram) It takes 5 wks to reach steady state & 6-8 wks to show adequate response Uses : PMS, Bulimia, OCD, Alcoholism AE : Agitation (need sedatives ), Weight loss (but regained after 12 months treatment), Seizure , Sexual dysfunction (always discuss with patient) Serotonin Syndrome: diaphoresis, rigidity, hyperthermia, ANS instability, myoclonus вЂ“ occurs when it takes with MAOI. Pt on SSRI c/o sexual dysfunction, appropriate other drug? вЂ“ Bupropion (MAOI & TCA Can cause sexual dysfunction) Bupropion (Atypical): block DA reuptake вЂ“ USE: Smoking Cessation All rights reserved. Registered with US copy right office 97 вЂў Buspirone: partial agonist at 5HT 1A - USE: Generalize Anxiety Disorder вЂў Trazadone : block 5HT reuptake , 5HT2 antagonist вЂ“ AE: Priapism вЂў Nefazodone: block both NE & 5HT reuptake вЂў Venlafaxime: similar to SSRI вЂў Mirtazapine: similar to TCAs в– Anti-Psychotic Drugs : вЂў Phenothiazines (Typical): DA (dopamine ) receptor - Chlorpromazine (rarely use now), Fluphenazine, Thioridazine. M block,О±-block, Sedation, в†“Seizure threshold. Quinidine like cardiotoxicity (Thioridazine) Tardive dyskinesia (TD) в†’ Extrapyramidal dysfunction оЂЉ Butyrophenone (Typical): DA receptor antagonist. Haloperidol Dystonia (torticolis вЂ“ spasm of sternocleidomastoid, Occulogyric crisis вЂ“ eyes rolled upwards) (Within first few day) [Tx: Benztropine / Diphenhydramine] TD (Tardive dyskinasia) [involuntary rhythmic movements of jaw upper extremity, lip, tongue protrusion, etc] (after long term use) [Tx: discontinue drug and start atypical antipsychotic] [Akathisia вЂ“ continuous restlessness and inability to stay calm] в†‘ Prolactin в†’ gynecomastia Riversible pseudo-parkinsonism Neuroleptic Malignant Syndrome [Tx: Dentroline] [Benztropine is C/I because anti-cholinergic drugs retain heat] вЂў - вЂў - Clozapine, Olanzepine, Risperidone [Atypical Anti-Psychotics]: Improve negative symptoms. Block 5HT2 receptors. (not DA receptor) Agranulocytosis (clozapine вЂ“ thatвЂ™s why it is not used as first-line drug) TD has not reported with clozapine and olanzapine. вЂў Pimozide: DOC for TouretteвЂ™s Syndrome вЂў Single episode of major depression вЂ“ Anti-depressant should be continued for a period of 6 months following patientвЂ™s response to the drug. вЂў First step in management of patient with major depression? вЂ“ rule out Hypothyroidism (order blood test) S.S.Patel. All rights reserved. Registered with US copy right office 98 вЂў MAOI drug interaction вЂ“ hypertensive crisis (Tyramine) and serotonin syndrome (SSRI, Pseudoephedrine, Meperidine) вЂў For bipolar patient on lithium, maintenance therapy should be continued for at least 1 yr following acute episode. If there are no relapses & patient attained good symptomatic control then lithium can be gradually tapered off and discontinue. вЂў Alternative drug for bipolar вЂ“ Valproic acid & Carbamazapine вЂў вЂў Lithium has been known to precipitate / exacerbate Psoriasis Treatment of choice for acute management of mania, psychosis or extreme agitation вЂ“ Haloperidol (rapid onset of action) or Lorazepam. Lithium takes 4-10 days. вЂў Drugs should be avoided in post-traumatic stress disorder? вЂ“ Benzodiazepines. Treatment of post-traumatic stress disorder вЂ“ SSRIs, exposure or cognitive therapy. вЂў вЂў вЂў вЂў Personality disorders вЂ“ Individual Psychotherapy Anxiety disorders вЂ“ Behavioral Therapy (breathing exercise, exposure therapy) Schizophrenia вЂ“ Supportive Therapy (build up trust so they talk more with you) Drug addiction вЂ“ Group Therapy вЂў вЂў вЂў вЂў вЂў вЂў First-line Treatment of Schizophrenia вЂ“ Atypical Anti-psychotics (Risperidone) Problem with compliance вЂ“ Give Fluphenazine (every 2 weeks) or Haloperidol (every 4 weeks) (both comes in IM preparation) c/o Sedation вЂ“ give Risperidone [side effects вЂ“ movement problem likes EPS and Parkinsonism] Side effect of Olanzepine вЂ“ Obesity. Nothing works вЂ“ Give Clozapine Treatment of catatonic Schizophrenia вЂ“ Benzodiazepam (Lorazepam) Fluphenazine can cause hypothermia. вЂў вЂў Acute Treatment of Panic Attack вЂ“ Alprazolam Chronic Treatment of Panic Attack вЂ“ SSRIs вЂў S.S.Patel. All rights reserved. Registered with US copy right office 99 PULMONOLOGY Obstructive Pulmonary Disease Restrictive Pulmonary Disease вЂў в†‘ airway resistance вЂў в†“ expiratory flow rate вЂў в†‘ TLC , в†“ FEV1 / FVC вЂў в†‘ in lung recoil вЂў в†“ in all lung volume вЂў в†‘ or N FEV1/ FVC PFT suggest obstructive pattern в†’ Diffusing Capacity в†ђ PFT suggest restrictive pattern пЈ¦ в†“ в†“ в†“ в†“ в†“ в†“ N/ Near N в†“ Chronic Bronchitis Asthma - в†“в†“в†“в†“ в†“в†“в†“ N / Near N в†“ Emphysema в†“ Extrapulmonary Restriction ( eg. Kyphoscoliosis, morbid obesity) в†“ Interstitial lung disease A вЂ“ a gradient = 150 вЂ“ 1.25 X PCO2 вЂ“ PaO2 Normal в†’ 5 вЂ“15 mmHg Hypoxemia в†‘ A вЂ“ a gradient в†“ Pulmonary Origin N A-a gradient в†“ Extrapulmonary origin вЂў PAO2 = % O2 ( 713) вЂ“ arterial PCO2 / 0.8 вЂў вЂў вЂў PAO2 = 0.21 ( 713) вЂ“ 40 / 0.8 = 100 mmHg PaO2 = 95 mmHg A-a gradient = 5 mmHg вЂў Atelectasis вЂ“ most common cause of fever in 1st 24-hrs post operatively * CXR вЂ“ best initial diagnostic test for pulmonary diseases S.S.Patel. All rights reserved. Registered with US copy right office 100 CXR Calcified Pulmonary Nodule Low Risk Patient (< 35 yrs old, nonвЂ“smoker) High Risk Patient (>50 yrs old, smoker) C X R every 3 months x 2 yrs Open lung biopsy Stop follow up, if no growth CXR Pleural Effusions Thoracocentesis (Next Best Step) в†“ LDH & Protein level Transudate Exudate LDH effusion < 200 > 200 LDH E / S < 0.6 > 0.6 Protein E / S < 0.5 < 0.5 - All 3 value must meet to diagnose transudative effusion - If atleast one criterion is not met, then exudative effusion - low pleural fluid PH & glucose (<60 mg /dl) are an indication for chest tube placement in patient with parapneumonic effusion CXR в†“ Atelectasis в†“ Appears on CXR as volume loss / densely consolidated в†“ Bronchoscopy with subsequent removal of mucous plug (Best Treatment / next step) вЂў вЂў вЂў вЂў S.S.Patel. Bacterial Pneumonia в†’ Consolidation в†’ dull to percussion, в†‘ vocal framitus Pneumothorax в†’Hyperresonant to percussion, в†“ Breath sound Pleural Effusion в†’ dull to percussion, в†“ Breath sound, в†“ vocal framitus Atelectasis в†’ dull to percussion, Absent Breath sound, loss of framitus All rights reserved. Registered with US copy right office 101 Pneumothorax в†’ opposite side вЂў Deviation of Trachea вЂў Atelectasis (lower lobe) в†’ elevation of diaphragm (same side) вЂў Trachea в†’ Rt & Lt main bronchus в†’ terminal bronchioles в†’ respiratory bronchioles в†’ alveolar duct (AD) в†’ alveoli Foreign body inhalation in child, next step? пѓ Direct laryngoscopy and rigid bronchoscopy to remove foreign body вЂў Atelectasis (upper lobe) в†’ same side пЃ® Asthma: Reversible airway obstruction Л— Cough induced by forceful expiration (characteristic of airway hyperactivity) Л— Poor prognosis factors вЂ“ Pulsus paradoxus, absent breath sounds, decrease wheezing, cyanosis, bradycardia, normal PaCO2 in acute Asthmatic patient Л— Best initial therapy вЂ“ Beta-agonist(Albuterol) Л— Exercise induced asthma вЂ“ beta agonist / chromolyn (mast cell stabilizer) Л— Acute exacerbation of Asthma вЂ“ Oxygen (first-step) and Inhaled beta2-agonist [steroids can be used when beta2-agonist fail. IV steroid is preferred over oral] [oral steroid is only used if patient is not vomiting]; IV Steroids Л— PFT should be done for documentation of Asthma вЂў Asthmatic patient present with c/o worsening of Asthma at night, night cough & Wheezing, Diagnosis? GERD; next step? пѓ proton pump inhibitors (omeprazole, pentoprazole) вЂў Subcutaneous emphysema in asthamatic (crepitation over the face & neck), next step? вЂ“ CXR [it is benign in asthamatic patient. CXR should be order to rule out pneumothorax] вЂў вЂњSilent chestвЂќ (absent air entry bilaterally) in Acute asthmatic patient on IV steroid, next step? Intubation вЂў Allergic bronchopulmonary Aspergillosis (ABPA) вЂ“ skin prick test (first step) should be done in all asthmatic patient who are suspected of having ABPA; Tx: oral prednisone вЂў Chronic eosinophilic pneumonia вЂ“ peripheral infiltrates that are photographic negative of pulmonary edema on CXR is the pathognomonic feature of chronic eosinophilic pneumonia вЂ“ >40% of eosinophils on bronchoalveolar lavage пЃ® вЂў вЂў вЂў S.S.Patel. COPD(Emphysema & chronic Bronchitis): Non-reversible airway obstruction в†“ Recoil & в†‘ Airway resistance Emphysema: cigarette smoking & alpha1-anti-trypsin deficiency (AAT) в†‘compliance (more dilated alveoli) and в†“elasticity (failure to keep airway lumen open вЂ“ essential for expiration so air trapped during expiration) вЂ“ Centriacinar [distended respiratory bronchioles вЂ“ air trapped in AD & alveoli] Panacinar [distended whole respiratory unit (Resp bronchioles, AD & alveoli) вЂ“ air trapped in whole unit] All rights reserved. Registered with US copy right office 102 вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў Chronic Bronchitis: productive cough for at least 3 months for 2 consecutive years вЂ“ smoking cigarette & cystic fibrosis - в†‘mucus in bronchi obstruct terminal bronchioles & narrowing of lumen due to chronic inflammation and fibrosis Emphysema Hyperinflation of bilateral lung fields With flattening of diaphragm, small CXR вЂ“ Size heart Chronic Bronchitis Increased pulmonary markings PFT вЂ“ best diagnostic test FEV1 вЂ“ Single most important factor to determine prognosis of COPD Treatment вЂ“ Smoking cessation (Best treatment to slow progression) Acute Exacerbation в†’ Oxygen, Systemic steroid, Antibiotics (Should cover H.influenzae & Pneumococcus), Bronchodilators Out patient Tx option в†’ Ipratropium (1st line drug) & Home O2 Bronchodilators (2nd line drug) Vaccination [Influenza (yearly)] First step in management of massive hemoptysis пѓ Rigid bronchoscopy (not flexible) Copious in amount & foul smelling sputum, initial test? пѓ CRX; next step? пѓ CT chest [Causes: Bronchiectasis, Lung Abscess, Anaerobic Pneumonia] в– Lung Abscess: Alcoholic, Extremely bad odor ( like decomposing dead animal) пЃ® TB: Alveolar macrophage в†’ CD4+ T-cells в†’ Macrophage release IL-12 (stimulates TH1 cells) and IL-1 (fever; activate TH1 cells) в†’ TH1 release IL-2 (self stimulation of TH1) and бµ§ interferon (activate macrophage to kill tubercular bacilli) в†’ Inflammatory mediators release from macrophage are responsible for tissue damage (no endotoxin or exotoxin) в†’ Lipid from tubercular bacilli leads to caseous necrosis пЃ® - Bronchiactasis: Permanent dilation of bronchi & bronchioles. Chronic infection [gram(-) organisms] [destruction of cartilage & elastic tissues] Persistent cough with purulent copious sputum production, wheezes, crackles Chest CT вЂ“ Best noninvasive test Treatment в†’ Bronchodilators, chest physical therapy, postural drainage, and Antibiotics Vaccination (Pneumococcal вЂ“ every 5 yrs & Influenza вЂ“ yearly) пЃ® - Idiopathic pulmonary Fibrosis: Involve only lung except clubbing Unknown etiology, occur in 5th decade CXR вЂ“ Reticular / Reticulonodular disease S.S.Patel. All rights reserved. Registered with US copy right office 103 - Chest CT вЂ“ ground glass appearance PFT- restrictive pattern Treatment вЂ“ steroid with / without Azathioprine (help in only 20% of pt) пЃ® - - Sarcoidosis: 20 вЂ“ 40 yrs. Old women Presence of nonspecific non-caseating granuloma in the lung and other organs CXR вЂ“ bilateral hilar adenopathy (90% of cases) Hypercalcemia (в†‘ 1-О±-hydroxylase by macrophage leads to в†‘ VitвЂ“D) [Hypercalciuria is occur in around 50% of cases whereas Hypercalcemia is seen in around 10-20% of cases of Sarcoidosis] в†‘ ACE (60 % of patients) Decrease cellular immunity [low helper/suppressor T-cell ratio] and activation of humoral immunity [increase CD4/CD8 ratio] Ophthalmoscopic examination (uveitis & conjunctivitis - >25% of the cases) Treatment вЂ“ systemic steroids when it involve uveitis / CNS / Hypercalcemia Tx of Hypercalcemia in Sarcoidosis вЂ“ hydration + glucocorticoids - Pulmonary pathology in Sarcoidosis пѓ inflammatory granuloma Pulmonary pathology in Systemic Sclerosis (Scleroderma) пѓ interstitial fibrosis - пЃ® Pneumoconiosis: - CXR вЂ“ small irregular opacities, interstitial densities, ground glass appearance, honey combing - Asbestosis в†’ H/O exposure, usually involve lower lung fields - CXR вЂ“ diffuse /local pleural thickening, pleural plaques, calcification at the level of diaphragm - Lung biopsy вЂ“ barbell shaped asbestos fiber (Best diagnostic test) - в†‘в†‘в†‘ Risk of Bronchogenic CA - в†‘ Risk of pleural / peritoneal mesothelioma пЃ® - Coal minerвЂ™s / coal workerвЂ™s pneumoconiosis (CWP): Usually involve upper half of lung Increase Levels of IgA, IgG, C3 , anti-nuclear Ab, RF Caplan syndrome вЂ“ Rheumatoid nodule in the periphery of the lung in a patient with RA & CWP пЃ® Silicosis Hyaline nodule, usually involve upper lobe - Strong association with TB, Pt should go yearly PD & if PPD >10mm then INH for 9 months пЃ® S.S.Patel. Pulmonary Thromboembolism: Sudden onset of dyspnea along with tachycardia ECG Right Axis Deviation h/o long term immobility Ventilation вЂ“ perfusion (V /Q) scan (Best initial test) All rights reserved. Registered with US copy right office 104 - Pulmonary Angiogram (most accurate test) V/Q scan в†’ Doppler U/S of lower limb or CT angiogram of chest в†’ Pulmonary angiography Treatment: continuous Heparin therapy x 5 days + Warfarin x 6 months. If Hemodynamically unstable в†’ THROMBOLYTICS, EMBOLECTOMY (IF THROMBOLYTICS ARE CONTRAINDICATED ) Pregnant patients в†’ low molecular weight Heparin x 6 months - вЂў Pleuritic chest pain (pain increase on inspiration), tachycardia & dyspnea in patient on contraceptive pills, diagnosis? пѓ Pulmonary embolism / infarction пЃ® - Adult Respiratory Distress Syndrome (ARDS): в†‘ Permeability of the alveolar вЂ“ capillary membrane & Pul.edema Alveolar macrophage в†’ cytokines в†’ Neutrophil в†’ damage capillary membrane CXR вЂ“ diffuse interstitial infiltrates; whiteout of both lung fields Swan-Ganz Catheter вЂ“ normal cardiac output & capillary wedge pressure в†‘ Pulmonary artery pressure Л— Mx of ARDS: PEEP (around 8-9 cmH2O), High oxygen and low tidal volume; Oxygen flow should be decrease after patient improve to prevent oxygen toxicity пЃ® - Sleep Apnea: Daytime Somnolence Obstructive sleep Apnea в†’ floppy airway, obese patient Central sleep Apnea в†’ inadequate ventilatory drive Treatment в†’ Acetazolamide, progesterone and supplemental O2 пЃ® Bronchogenic Carcinoma: - Squamous cell CA в†’ Centrally located в†’ Hypercalcemia вЂ“ PTH-like substance - Small cell CA в†’ Centrally located в†’ SIADH, Eaton-Lambert, Venocaval obstruction Syndrome, HornorвЂ™s syndrome - Large Cell CA в†’ Peripherally located - Adenocarcinoma в†’ peripherally located в†’ Pleural effusion with high hyaluronidase level in effusion fluid. Bronchoalveolar CA is subtype - Popcorn calcification, concentric, central, or diffuse homogenous calcification on CXR is suggestive of a benign pathology of pulmonary nodules вЂў Diagnosis : Sputum Cytology (SCC - >80%), Bronchoscopy (>90% for centrally located) - Needle Aspiration Biopsy в†’ peripheral nodule with effusion - Treatment : Resection (when possible), etoposide & platinum for small CA, - Non-small cell CA вЂ“ Cyclophosphamide, Adriamycin and platinum - Effusions can be sclerosed with Tetracycline вЂў Pancoast tumor вЂ“ HornorвЂ™s syndrome, Phrenic N involvement [Chest movement asymmetry] [a dangerous sign in patient with Pancoast tumor]; Radiation therapy is the treatment of choice for Pancoast tumor with distant metastasis вЂў HornorвЂ™s syndrome in smoker, next step? вЂ“ CXR (to rule out lung CA) S.S.Patel. All rights reserved. Registered with US copy right office 105 REPRODUCTIVE SYSTEM пЃ® Cryptorchidism: undescended testis вЂ“ usually resolve by 1 year of age вЂ“ increase risk for seminoma, infertility пЃ® Vericocele: вЂњbag of wormsвЂќ appearance вЂ“ Lt sided most common вЂ“ infertility пЃ® Hypospadias в†’ urethral opening on ventral side of the penis в†’ never do circumcision в†’ Tx : surgical repair пЃ® Epispadias в†’ urethral opening on dorsal side of the penis пЃ® Posterior Urethral valves в†’ newborn boy, not urinate during the first day of life or distended bladder and weak urinary stream вЂ“ thickened urinary bladder wall on U/S в†’ Dx: voiding cystogram в†’ Tx: Endoscopic fulguration . пЃ® Hydrocele вЂ“ fluid collection b/w layer of tunica vaginalis, transillumination positive в†’ usually resolve by 1 year of age. If it is not resolved by 1 year of age then surgical intervention пЃ® Л— Л— Л— Л— Л— Л— Testicular Tumors: unilateral painless testicular mass in young person Germ cell (95%) вЂ“ malignant Sex-cord (5%) вЂ“ benign Germ cell вЂ“ Seminoma (40%) and Non-seminoma (60%) Seminoma вЂ“ metastasis [para-aortic в†’ hematogenous (lung)] Seminoma (в†‘hCG) & Non-Seminoma (в†‘ AFP & hCG) Tx: inguinal exploration & high ligation of the spermatic cord with removal of the testicles and spermatic cord (radical orchiectomy) (best initial treatment) вЂў Large mediastinal mass with elevated hCG & AFP, diagnosis? non-seminomatous germ cell tumor вЂў Nodule on prostate [not enlarge prostate (BPH)] and/or PSA >4 ng/ml, next step? вЂ“ biopsy of prostate пЃ® BPH (Benign Prostatic Hypertrophy): DHT (dihydrotestosterone) causes hyperplasia of glandular and stromal cells вЂ“ develop in central zone so do not palpable by digital rectal examination (DRE) вЂ“ trouble initiating & stopping urinary stream, dribbling, nocturia, dysuria вЂ“ no relation with prostate CA вЂ“ Complication: UTI (most common), Sepsis (E.coli), Post-obstruction renal failure вЂ“ Tx: Finasteride (5-О±-reductase inhibitor) (act on epithelial component of prostate gland); TURP (Transurethral resection of prostate) [Most common complication of TURP is Hyponatremia, cause? fluid containing glycine, mannitol or sorbitol is used to flush during TURP] [Most common long-term complication of TURP вЂ“ retrograde ejaculation] S.S.Patel. All rights reserved. Registered with US copy right office 106 вЂў Patient [pt.] with BPH present with irritative symptoms, next step? Urine analysis & serum creatinine пЃ® Prostate CA: DHT dependent вЂ“ develop in peripheral zone so palpable by DRE вЂ“ usually asymptomatic until advanced вЂ“ PSA >10 ng/ml highly predictive вЂ“ bone metastasis (common) [it is the only tumor which has osteoblastic activity where as other tumor has osteoclastic (ostolytic) activity when metastasize to bone] вЂў First line Tx of Metastatic prostate CA вЂ“ Leuprolide [LHRH agonist], [must be given with Androgen blockers (Flutamide) initially to prevent flare up reaction] + Radiation пЃ® Prostatitis: urine analysis and urine culture вЂ“ non-bacterial Prostatitis (Prostatodynia) is more common than acute and chronic bacterial Prostatitis вЂ“ clinical picture is similar in both bacterial and non-bacterial Prostatitis and WBCs are >20/hpf in prostatic secretion in both but in non-bacterial, culture of prostatic secretion is negative and no h/o past UTI. Tx of non-bacterial Prostatitis вЂ“ sitz baths and anti-inflammatory drugs вЂў Afebrile patients with prostatic tenderness and irritative voiding symptoms, no H/o past UTI, diagnosis? пѓ Prostatodynia пЃ® Acute bacterial prostatitis пѓ gram stain & culture of a mid stream urine sample (prostatic massage is contraindicated due to the possibilities of disseminating the infection) вЂ“ Tx: Ciprofloxacin / TMP-SMX пЃ® Testicular Torsion: severe testicular pain of sudden onset without fever, pyuria (or) h/o recent mumps в†’ swollen, extremely tender high riding horizontally lie testis в†’ Emergency surgery пЃ® Acute epididymitis: severe testicular pain of sudden onset with fever, pyuria в†’ swollen, extremely tender testis in normal lie в†’ Tx: Antibiotics [Ceftriaxone + Doxycycline] (USG в†’ to rule out torsion) пЃ® Recurrent, Painful oral & genital ulcers + erythema nodosum, diagnosis? пѓ BehcetвЂ™s Syndrome (Autoimmune mechanism) пЃ® Penis fracture, next step? пѓ Retrograde urethrogram fallowed by Surgical repair пЃ® Penile CA in situ вЂ“ BowenвЂ™s disease вЂ“ one lesion (thicken whitish plaque with a slight ulcerated surface) вЂ“ risk for SCC (squamous cell CA) - Bowenoid Paulosis вЂ“ multiple reddish brown popular lesion вЂ“ No risk for SCC - Erythroplasia of Queyart вЂ“ multiple shiny red plaques вЂ“ risk for SCC п‚§ S.S.Patel. Pearly penile papules are considered a normal variant and are not transmitted through sexual contact and has no malignant potential All rights reserved. Registered with US copy right office 107 в– * п‚§ п‚§ Embryology: Post-conception week 1: Implantation of blastocyst Intra-tubal phase: first half of the week 1 Intra-uterine phase: entery of the morula into the uterus Morula differentiate into a hollow ball of cells Outer layer вЂ“ trophoblast (Placenta) Inner layer вЂ“ embryo * Post-conception week 2: Development of the bilaminar germ disk with epiblast and hypoblast layer Invasion of maternal sinusoids by syncytiotrophoblast (ОІ-HCG test becomes positive) Post-conception week 3: Migration of cells through the primitive streak between the epiblast and hypoblast to form the trilaminar germ disk with ectoderm, mesoderm and endoderm layers п‚§ * * Post-conception week 4-9: Organs & organ systems development п‚§ п‚§ Chorionic villous sampling (CVS): fetal Karyotyping (9-12 weeks) Amniocentesis: genetic purpose (15-20 weeks), Rh-isoimmunization (after 24 weeks), fetal maturity (after 34 weeks) Percutaneous Umbilical blood sampling: fetal Karyotyping, IgM antibody detection, blood typing, intra-uterine blood transfusion (after 20 weeks) п‚§ п‚§ п‚§ п‚§ п‚§ Placental Hormones: ОІ-HCG, Human Placental Lactogen (HPL) Progesterone sources: corpus luteum of pregnancy (6-7 weeks), corpus luteum & placenta (7-9 weeks), Placenta (after 9 weeks) Estrogen sources: Estradiol (non-pregnant reproductive period): follicular granulosa cells; Estriol (during pregnancy): DHEA-S from fetal adrenal gland convert into Estriol by placental sulfatase; Estrone (menopause): peripheral adipose tissues Steroid binding globulins increase during pregnancy, so total hormone level increase but free hormone level remains unchanged (same thing happen in person on anabolic steroids, OCP) therefore hypothyroid patient should increase dose of thyroxine. п‚§ Braxton-Hicks Contraction: painless, low intensity, long duration contraction (during 2nd trimester) п‚§ Maternal Serum AFP (MS-AFP): [0.85 вЂ“ 2.5 MoM] Elevated MS-AFP вЂ“ obstetric US to confirm gestational date (1st step) Low MS-AFP вЂ“ obstetric US to confirm gestational date (1st step) п‚§ Triple marker screen: MS-AFP, hCG & Estriol (15-20 weeks) в†“в†“ MS-AFP & Estriol, в†‘ hCG вЂ“ trisomy-21 вЂ“ next step? вЂ“ Amniocentesis for Karyotyping в†“в†“в†“ All three вЂ“ trisomy-18 вЂ“ next step? вЂ“ Amniocentesis for Karyotyping S.S.Patel. All rights reserved. Registered with US copy right office 108 п‚§ Gestational Diabetes Screen вЂ“ b/w 24-28 wks вЂ“ 1-hr 50g oral GTT (screening test) вЂ“ If level >140 mg/dl, do definitive test (3-hrs 100g oral GTT done after overnight fasting) вЂ“ FBS (<126 gm/dl), After 100g oral GTT вЂ“ at 1-hr (<180 gm/dl), at 2-hrs (<155 mg/dl), at 3-hrs (<140 mg/dl) п‚§ п‚§ c/o в†“ fetal movement, no fetal heart tone on Doppler, next step? ultrasonography c/o в†“ fetal movement, fetal heart tone heard on Doppler, next step? Non-stress test Ultrasonography (Biophysical profile) вЂ“ FHR (without any stressor like oxytocin), fetal body movement, tone, breathing, amniotic fluid index п‚§ п‚§ c/o decrease fetal movement, next step? вЂ“ NST (non-stress test) Reactive Reassuring > 10 beats / min (< 32 weeks) > 15 beats / min (> 32 weeks) Two acceleration in 20 mins Non-reactive Next step? Vibroacoustic Stimulation Still non-reactive, next step? Biophysical Profile (US) 4-6 Perform CST 8-10 Highly reassuring Negative Highly reassuring 0-2 Prompt delivery Positive (late deceleration) Worrisome (deliver if >36 weeks) в– Perinatal Infections: п‚§ S.S.Patel. Group B beta-hemolytic streptococci (GBBS): candidates for intra-partum Penicillin G prophylaxis вЂ“ positive GBBS urine culture, previous baby with GBBS sepsis, positive 3rd semester vaginal culture, preterm gestation, maternal fever, membrane rupture > 18 hrs All rights reserved. Registered with US copy right office 109 п‚§ п‚§ Toxoplasmosis: 3rd trimester primary infection with T.gondi вЂ“ high risk of vertical transmission Cesarean section: genital HSV lesions & HIV positive patients [Condylomata acuminata is a manifestation of HPV infection and it is not a C/I for vaginal delivery] в– Important Pregnancy changes: Increase вЂ“ plasma volume, RBC mass (less compare to plasma), factors 7,8,9,10, cardiac output, tidal volume, renal blood flow, GFR, pituitary & thyroid gland size п‚§ Respiratory alkalosis occur during pregnancy п‚§ в†“ Renal glucose threshold п‚§ в†“ gastric & bowel motility * Herpes Gestationis: autoimmune (not due to virus) вЂ“ rash and vesicles around umbilicus in second / third trimester, recur during subsequent pregnancy вЂ“ Tx: topical triamcinolone * Pruritic urticarial papules and plaques of pregnancy (PUPPP): it typically develops on the abdomen, especially with periumbilical striae distensae, while the umbilicus is usually spared (contrast to Herpes Gestationis) S.S.Patel. All rights reserved. Registered with US copy right office 110 Pregnancy Bleeding Who is compromising? Mother (low BP, shock) Fetus (bradycardia) Next step? Vasa Previa Stabilize mother (IV line with large bore needle) Important causes Abruptio Placenta Painful vaginal bleeding h/o cocaine abuse, h/o trauma increase fundal heights, abnormal shape (in conceal bleeding) Management depends upon Maternal or fetal jeopardy, fetal maturity Complications: DIC, shock with ATN (acute tubular necrosis) D/D: Abruptio placenta & Uterine rupture can be difficult to distinguish when present with h/o trauma, but culdocentesis suggestive of hemoperitoneum is seen in uterine rupture and helpful in diagnosis S.S.Patel. All rights reserved. Next step? Emergency C-section Placenta Previa Painless vaginal bleeding Management depends upon Maternal or fetal jeopardy, fetal maturity Complications: Shock with ATN, SheehanвЂ™s Syndrome Never perform a digital or speculum examination until US rules out placenta previa Placenta accreta Placenta invades the myometrium, but does not penetrate the entire thickness of the muscle. Placenta increta Occurs when the placenta further extends into the myometrium Placenta percreta The worst form of the condition, The placenta penetrates the entire myometrium to the uterine serosa (invades through entire uterine wall). Registered with US copy right office 111 Abortion No cervical dilatation Missed Non-viable pregnancy Suction D&C or Conservative until spontaneously aborted п‚§ Cervical dilatation present Inevitable Product of conception not passed Emergency suction D&C Threatened Viable pregnancy No intervention is generally indicated. Mx: Regular out patient follow-up Incomplete Some Product of conception has passed Emergency suction D&C Complete Abortion: All products of conception passed, US show no intrauterine contents. Fetal Demise Uterine fundus less than dates (< 20 weeks) Absence of fetal movement (> 20 weeks) Absent heart activity on US Management DIC present Emergency delivery DIC absent Suction D&C (< 20 weeks) Induction of labor (> 20 weeks) в– Rh-Isoimmunization: Mother Rh-negative, fetus Rh-positive п‚§ Indirect CoombвЂ™s test (screening test) вЂ“ at 28 weeks of gestation п‚§ Atypical antibody titer вЂ“ >1:8 вЂ“ risk for fetus <1:8 вЂ“ no fetal risk п‚§ Tx: Intrauterine transfusion if gestational age <34 weeks п‚§ Delivery if gestational age >34 weeks S.S.Patel. All rights reserved. Registered with US copy right office 112 п‚§ п‚§ в– п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Prevention: RhoGAM is given to Rh-negative mother at 28-weeks of gestation, within 72-hrs of amniocentesis, D&C, chorionic villous sampling and delivery of Rh-positive baby Kleihauer-Betke test: measure the volume of fetal RBCs in maternal circulation Premature Rupture of Membrane (PROM): Pooling positive, Nitrazine positive (paper turns blue), fern positive If uterine contraction present, Tocolysis is contraindicated If infection present вЂ“ cervical culture, antibiotics, prompt delivery If infection absent вЂ“ management depends upon fetal maturity <36 wks вЂ“ Hospitalize, cervical culture, steroids, antibiotics >36 wks вЂ“ prompt delivery Chorioamnionitis signs & symptoms: maternal fever, uterine tenderness, PROM, absence of UTI or URI в– Preterm labor: gestational age (20-37 wks), atleast three contractions in 30 mins, cervical dilation (>2 cm) / effacement п‚§ Bed rest & IV hydration (first step), IV MgSO4, steroids, cervical cultures, antibiotics п‚§ Ritodrine (only FDA approved tocolytics) вЂ“ beta-agonist [side effects вЂ“ hypotension, tachycardia, hyperglycemia, hypokalamia, pulmonary edema] в– п‚§ п‚§ п‚§ Post term labor: >42 wks of gestation Dates sure, favorable cervix вЂ“ aggressive management Dates sure, unfavorable cervix вЂ“ cervical PGE2 followed by IV oxytocin Dates unsure вЂ“ conservative management & await spontaneous labor HTN in Pregnancy Gestational HTN BP - 140/90 mm of Hg after 20 wks without proteinuria Conservative management S.S.Patel. Mild Preeclampsia BP - 140/90 mm of Hg 1-2 + proteinuria Hemoconcentration Delivery is the only definitive cure < 36 wks вЂ“ conservative in the hospital > 36 wks вЂ“ delivery IV oxytocin + IV MgSO4 All rights reserved. Severe Preeclampsia BP - 160/110 mm of Hg 3-4 + proteinuria Hemoconcentration Thrombocytopenia Elevated liver enzymes Aggressive prompt delivery at any gestational age IV MgSO4 to prevent Eclampsia Registered with US copy right office 113 в– Eclampsia: Preeclampsia + seizure п‚§ Aggressive prompt delivery at any gestational age after stabilization of mother п‚§ Intracerebral hemorrhage вЂ“ most common cause of death in eclampsia в– Chronic HTN: BP 140/90 mmHg before 20 wks of gestation; conservative management for mild-moderate HTN / Methyldopa; DBP should be maintain b/w 90-100 mmHg вЂ“ Drug of choice for chronic HTN & diabetic nephropathy in pregnancy: Labetalol (ACE inhibitors are contraindicated) п‚§ DOC for hypertensive emergency in pregnant women вЂ“ Labetalol / Hydralazine в– HELLP syndrome: Hemolysis, Elevated Liver enzymes and Low Platelets count; Tx: aggressive prompt delivery at any gestational age + IV MgSO4 to prevent eclampsia в– Symmetric IUGR вЂ“ both head & body вЂ“ usually before 28 wks. (chromosomal abnormality, infection) в– Asymmetric IUGR вЂ“ Head is spare & body affected вЂ“ usually after 28 wks. (MotherвЂ™s factors пѓ hypertension, preeclampsia, chronic renal disease) в– Overview of Labor: п‚§ More than 3 contractions in 10 mins, each lasing >30 sec п‚§ Engagement в†’ Decent в†’ Flexion в†’ Internal Rotation в†’ Extension в†’ External Rotation в†’ Expulsion (First 3 steps occur simultaneously) п‚§ п‚§ п‚§ Stage-1: Onset of contraction to complete cervical dilation (10 cm) Latent Phase: ends with the acceleration with cervical dilation (3-4 cm); No descent of fetus occur; 20-hrs in Primipara & 14-hrs in Multipara Active Phase: ends with complete cervical dilation (10 cm); descent of fetus occur; 1.2 cm/h in Primipara & 1.5 cm/h in Multipara п‚§ п‚§ Stage-2: ends with delivery of fetus Up to 2-hrs in Primipara & 1-hr in Multipara п‚§ Stage-3: ends with expulsion of placenta (gush of blood vaginally, decrease fundal heights, вЂњlengtheningвЂќ of umbilical cord); up to 30-mins in all women п‚§ Stage-4: close observation of parturient for 1-2 hrs after delivery п‚§ Oxytocin (similar to ADH) can cause water intoxication by retention of water [which dilute Na and produce hyponatremia] п‚§ Prolong active phase of stage-1: Oxytocin if hypotonic contraction; Emergency C-section if contractions are adequate S.S.Patel. All rights reserved. Registered with US copy right office 114 п‚§ п‚§ п‚§ п‚§ Prolong stage-2: IV Oxytocin if hypotonic contraction If adequate contraction, check fetal head is engage or not If head engage, consider trial of forceps or vacuum If head is not engaged, emergency C-section п‚§ Prolong stage-3: IV Oxytocin / IM Methylergomatrin / manual removal of placenta / hysterectomy (rarely) п‚§ Prolapsed umbilical cord: Emergency C-section п‚§ Preferred contraception in early post-partum period вЂ“ sterilization, condom & progesterone only pills (mini pills) п‚§ Non-reassuring FHR (fetal heart rate) tracing: Baseline rate show tachy- / bradycardia without explanation, absent acceleration, repetitive variable deceleration, repetitive late deceleration, absent variability Л— Variable deceleration (without contraction) вЂ“ fetal cord compression Л— Early deceleration (with contraction) вЂ“ fetal head compression Л— Late deceleration (after contraction) вЂ“ uteroplacental insufficiency S.S.Patel. All rights reserved. Registered with US copy right office 115 Cervical Cancer How will it present to us? Abnormal routine Pap smear (Atypical Sqmaous Cells) c/o Post-coital bleeding Next step? Next step? HPV DNA testing & Colposcopy Cervical Biopsy If biopsy confirms invasive CA, Next step? If abnormal findings (mosaicism, punctuation, white lesions, abnormal blood vessels), Next step? Ectocervical biopsy of above colposcopic findings Endocervical curettage (ECC) (All non-pregnant women) Metastatic work-up Managements If Pap smear finding is worse than histology or If abnormal ECC, Next step? Cone Biopsy п‚§ п‚§ HPV subtypes associated with cervical CA вЂ“ 16, 18, 31, 33, 35 Atypical squamous cells (ASC) on pap smear вЂ“ ASC-US or ASC-H (canвЂ™t exclude HSIL) п‚§ Classifications: Pre-cancerous stages Dysplasia Mild Moderate Severe CIS CIN I II III Bethesda LSIL HSIL CIN = cervical intraepithelial neoplasia CIS = carcinoma in situ LSIL = low grade squamous intraepithelial lesions HSIL = high grade squamous intraepithelial lesions That means: Mild dysplasia = CIN-I = LSIL Moderate dysplasia = CIN-II = HSIL Severe dysplasia / CIS = CIN-III = HSIL S.S.Patel. All rights reserved. Registered with US copy right office 116 в– Management of Pre-cancerous lesions: п‚§ п‚§ п‚§ ASC-US, CIN-I: repeat Pap smear in 4-6 months (If patient is unreliable for follow-up, do Colposcopy instead of waiting for patient to come back for Pap smear) CIN-I, CIN-II, CIN-III: Cryotherapy, laser, LEEP, cold-knife conization Biopsy confirmed recurrent CIN-II,III: Hysterectomy в– Management of Invasive Cervical CA: Depend upon stage вЂў Stage 0 - full-thickness involvement of the epithelium without invasion into the stroma (carcinoma in situ) вЂў Stage I - limited to the cervix o IA - diagnosed only by microscopy; no visible lesions п‚§ IA1 - stromal invasion less than 3 mm in depth and 7 mm or less in horizontal spread п‚§ IA2 - stromal invasion between 3 and 5 mm with horizontal spread of 7 mm or less o IB - visible lesion or a microscopic lesion with more than 5 mm of depth or horizontal spread of more than 7 mm п‚§ IB1 - visible lesion 4 cm or less in greatest dimension п‚§ IB2 - visible lesion more than 4 cm вЂў Stage II - invades beyond cervix o IIA - without parametrial invasion, but involve upper 2/3 of vagina o IIB - with parametrial invasion вЂў Stage III - extends to pelvic wall or lower third of the vagina o IIIA - involves lower third of vagina o IIIB - extends to pelvic wall and/or causes hydronephrosis or non-functioning kidney вЂў Stage IVA - invades mucosa of bladder or rectum and/or extends beyond true pelvis вЂў Stage IVB - distant metastasis п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ S.S.Patel. Stage-Ia1: simple hysterectomy Stage-Ia2: hysterectomy + lymphnodes removal Stage Ib1 and IIa less than 4 cm: radical hysterectomy with removal of the lymph nodes or radiation therapy. Stage Ib2 and IIa more than 4 cm: radiation therapy and cisplatin-based chemotherapy, hysterectomy or cisplatin chemotherapy followed by hysterectomy Stage-IIb, III, IV: Radiation and Chemotherapy (cis-platinum) Pregnancy & Cervical CA: Diagnosis & management is same as in non-pregnant women except ECC is not done in pregnant women All rights reserved. Registered with US copy right office 117 в– п‚§ п‚§ п‚§ п‚§ п‚§ Endometrial CA: Prolong Estrogen (E) exposure without Progesterone (P) Post-menopausal bleeding (most common presentation) Next step? вЂ“ Endometrial sampling If negative histology вЂ“ Hormone replacement therapy If positive histology вЂ“ Surgery, Radiotherapy, Chemotherapy в– п‚§ п‚§ п‚§ Leiomyomas: most common benign uterine tumor Intramural вЂ“ within the wall of the uterus Submucous вЂ“ located beneath the Endometrium; causes Menorrhagia (heavy bleeding) and Metrorrhagia (irregular bleeding in between menses) Subserosal вЂ“ located beneath uterine serosa Asymmetric, Non-tender, enlarged uterus in the absence of the pregnancy Tx: GnRH analog (3-6 months), myomectomy, embolization, Hysterectomy п‚§ п‚§ п‚§ в– Adenomyosis: п‚§ endometrial glands and stoma located within the myometrium of the uterine wall (uterus) п‚§ Pain immediately before and during menses п‚§ Symmetric, tender, enlarged uterus in the absence of the pregnancy п‚§ Tx: levonorgestrel intrauterine system в– Endometriosis: п‚§ endometrial glands and stoma located outside the uterus п‚§ Chocolate cyst of the ovary, utero-sacral ligament nodularity, pain during intercourse (dyspareunia), infertility п‚§ Dx: Laparoscopy п‚§ Tx: OCPs (oral contraceptive pills) в– п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Vulvar neoplasm: c/o intense vulvar pruritus Melanoma вЂ“ black lesions вЂ“ biopsy & excision PagetвЂ™s disease вЂ“ red lesions вЂ“ biopsy & excision Squamous cell CA вЂ“ HPV association вЂ“ whitish lesions вЂ“ biopsy & excision Hypertrophic dystrophy of the Vulva вЂ“ corticosteroid ointment Atrophy of Vagina вЂ“ Estrogen cream п‚§ п‚§ PagetвЂ™s disease (of breast, cervix) вЂ“ Adenocarcinoma PagetвЂ™s disease of bone вЂ“ isolated elevation of Alkaline phosphate in elderly Patient with normal Ca+2, Po4 & amino transferase level - Worrisome changes in PagetвЂ™s disease of bone вЂ“ sarcomatous (1-3%) вЂ“ Tx: asymptomatic PagetвЂ™s disease of bone вЂ“ nothing; symptomatic вЂ“ bisphosphonates S.S.Patel. All rights reserved. Registered with US copy right office 118 Adnexal Mass Pre-Pubertal Germ cell tumors Next step? Obtain tumor markers LDH вЂ“ Dysgerminoma ОІ-HCG вЂ“ Choriocarcinoma AFP вЂ“ Endodermal sinus tumors (Yolksac tumors) п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Reproductive Age Simple cyst Complex mass [Teratoma (Dermoid cyst)] Ectopic Pregnancy Next step? ОІ-HCG US (ultrasonography) Post-Menopausal Ovarian Cancer Next step? Find GI tract and Urinary tracts lesions Simple Cyst on US: <7 cm, next step? вЂ“ observe for 6-8 wks, resolved spontaneously, start OCPs to prevent further functional cysts fro forming; >7 cm or already on OCP (for atleast 2-months), next step? вЂ“ Laparoscopy Complex mass on US: Cystectomy (to preserve ovarian function); Oophorectomy if conservative failed Any cyst in pregnancy which is greater than 5 cm should be removed during second trimester of pregnancy Most common benign ovarian tumor вЂ“ serous cystadenoma Most common malignant ovarian tumor вЂ“ serous cystadenocarcinoma Granulosa-theca cell tumors вЂ“ secrete estrogen Sertoli-Leyding cell tumors вЂ“ secrete testosterone KrukenbergвЂ™s tumor вЂ“ contains signet ring cells from Metastatic stomach cancer Dysgerminoma вЂ“ resembles Seminoma of the testis MeigвЂ™s Syndrome вЂ“ Ovarian fibroma, Ascites & right sided pleural effusion Abdominal pain + Adnexal mass, diagnosis? вЂ“ Torsion of the ovary, next step? вЂ“ surgical intervention (exploratory laparotomy) пЃ® Immature teratoma вЂ“ primitive epithelial cells & developing skeletal muscles, potentially malignant [teratoma вЂ“ derivatives of all 3 germ layers [ectoderm, mesoderm and endoderm вЂ“ hair, tooth, thyroid gland, etc] S.S.Patel. All rights reserved. Registered with US copy right office 119 пЃ® Dermoid cyst: A dermoid cyst is a mature cystic teratoma containing hair and other structures characteristic of normal skin and other tissues derived from the ectoderm. The term is most often applied to teratoma on the skull sutures and in the ovaries of females. пЃ® Struma ovarii: mature teratoma that contains mostly thyroid tissue. пЃ® Gestational Trophoblastic Neoplasia (GTN): п‚§ HTN in the first trimester, fundus larger than dates, вЂњsnowstormвЂќ appearance on US, grape-like vesicles, high beta-hCG, hyperthyroidism п‚§ Complete Mole: empty egg, 46XX (parental), No fetus, progression to malignancy (20%) п‚§ Incomplete Mole: 69(XXX), fetal parts present, progression to malignancy (10%) п‚§ Lung most common site of metastasis, Tx: Suction D&C п‚§ After D&C for molar pregnancy, patient should be put on OCP so they donвЂ™t get pregnant and beta-hCG level monitor effectively п‚§ Follow-up with beta-hCG [upto 1-yr вЂ“ if benign; upto 5-yrs вЂ“ if malignant] в– Uterine Incontinence: п‚§ п‚§ п‚§ п‚§ О± & ОІ receptors вЂ“ prevent micturation cholinergic receptors вЂ“ enhance micturation Urethra вЂ“ О± receptors (estrogen) Bladder вЂ“ ОІ receptors (progesterone) & cholinergic receptors п‚§ п‚§ п‚§ Genuine stress incontinence: Cystocele, positive Q-tip test [Q-tip rotate >300 when intra-abdominal pressure increase, eg. Cough] Mx: Kegel exercise, estrogen replacement, urethropaxy п‚§ п‚§ п‚§ Irritative incontinence: Infection, stone, tumor, foreign body Mx: treat the cause п‚§ п‚§ п‚§ п‚§ Hypertonic incontinence: Idiopathic detrusor contraction Urgency (most common symptoms) Mx: Anticholinergic medication п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Hypotonic incontinence: Denervated bladder (DM, Multiple Sclerosis) c/o pelvic fullness, в†“ pudendal nerve sensation Mx: Intermittent self catheterization, cholinergic medications Fistula, Bypass incontinence: h/o radical pelvic surgery or h/o pelvic radiation therapy, c/o continuous leaking Mx: Surgical repair S.S.Patel. All rights reserved. Registered with US copy right office 120 в– п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Ectopic Pregnancy: Amenorrhea, vaginal bleeding and unilateral pelvic-abdominal pain Positive beta-hCG, no intrauterine pregnancy (IUP) on US IUP detected with vaginal sonogram at 1500 mIU beta-hCG titer IUP detected by abdominal sonogram at 6500 mIU beta-hCG titer Beta-hCG level in normal IUP doubles every 58-hrs Age is an important risk factor for an ectopic pregnancy besides PID (35-44 age group, 3 fold increase in risk for ectopic pregnancy) Mx: Confirm diagnosis (first step) (beta-hCG titer >1500 mIU, no IUP) Ruptured ectopic: immediate surgical intervention (salpingectomy) Unruptured ectopic: pregnancy mass <3.5 cm, beta-hCG level <6000 mIU, absent fetal heart motion, no folic acid supplementation вЂ“ Methotraxate If above criteria not met вЂ“ Laparoscopy (salpingostomy) Rh-negative mother should receive RhoGAM, follow-up with beta-hCG titer Beta-hCG routinely drawn on day 4 and day 7 of treatment of ectopic pregnancy with Methotraxate. If it drops 15% from day 4 to day 7, treatment of ectopic pregnancy is thought to be successful and patient returns for beta-hCG blood draw until it is negative п‚§ Bacterial Vaginosis: thin, grayish-white discharge, fishy odor, vaginal pH above 5, epithelial cells with smudged borders (вЂњclueвЂќ cells) due to bacteria adherent to cell membranes вЂ“ Tx: Metronidazole (contraindicated in pregnancy, use Clindamycin during pregnancy) п‚§ Trichomonas Vaginitis: frothy, green discharge, itchy, burning pain with intercourse, vaginal pH above 5, sexually transmitted disease (STD), вЂњtrichomonadsвЂќ on microscopic examination вЂ“ Tx: Metronidazole (treat both partners) - Tx of trichomoniasis in lactating women вЂ“ 2 g of Metronidazole single dose and ask her to discontinue breast feeding for 12-24 hrs п‚§ Yeast Vaginitis: curdy, white discharge, pH <4.5, not a STD, Pseudohyphae on microscopic examination вЂ“ Tx: single dose Fluconazole в– Menstrual Cycle Hormones: п‚§ п‚§ п‚§ п‚§ S.S.Patel. FSH: stimulate granulosa cells to secrete E & Inhibin (inhibits FSH release) Estrogen: Negative feedbacks to FSH Low E вЂ“ Negative feedbacks to LH High E вЂ“ Positive feedbacks to LH LH: stimulate production of androgens from theca cells, LH surge stimulates synthesis of prostaglandins to enhance follicular rupture & ovulation Progesterone: secreted by corpus luteum, prepare Endometrium for blastocyst implantation All rights reserved. Registered with US copy right office 121 Abnormal Vaginal Bleeding Premenarchal Vaginal foreign body Sexual abuse Precocious puberty Sarcoma botryoides (grape like protrusion through vagina) в– п‚§ п‚§ п‚§ п‚§ Reproductive Age Pregnancy Anatomic causes DUB (Dysfunctional Uterine Bleeding) Post-Menopausal Atrophic changes Endometrial carcinoma Puberty changes: Thelarchy (breast development) вЂ“ 9-10 yrs (E from the ovary) Adrenarchy (pubic & Axillary hair) вЂ“ 10-11 yrs (Adrenal hormones) Maximal growth rate вЂ“ 11-12 yrs Menarchy (onset of menses) вЂ“ 12-13 yrs в– Precocious Puberty: development of puberty before age 8 in girls and before age 9 in boys п‚§ GnRH stimulation test вЂ“ helpful to differentiate central Vs peripheral cause of precocious puberty - в†‘в†‘ LH after GnRH injection indicates central cause п‚§ Central: hypothalamic-pituitary-ovarian axis disturbance п‚§ Peripheral: ovarian tumor, adrenal tumor, exogenous estrogen, McCune-Albright п‚§ Management of patient with precocious puberty пѓ GnRH agonist to inhibit secretion of estrogen to prevent premature fusion of the epiphyseal plates S.S.Patel. All rights reserved. Registered with US copy right office 122 Precocious Puberty Gonadotropin Dependent CNS Pathology Sarcoidosis Meningitis Other causes Tx: treat pathology Idiopathic 80% of cases Tx: Leuprolide Gonadotropin Independent McCuneвЂ“Albright Syndrome Autonomous stimulation of aromatase enzyme which в†‘в†‘ estrogen production from ovary CafГ©-au-lait skin spots Multiple cystic bone lesions (Polyostic fibrous dysplasia) Tx: Aromatase enzyme inhibitors McCune Albright syndrome has association with CushingвЂ™s syndrome, not CushingвЂ™s disease (which is due to Pituitary tumor) Granulosa cell tumor Secrete estrogen Tx: Surgical removal of tumor в– Dysfunctional Uterine Bleeding: Anovulation (So no progesterone effect which left estrogen unopposed), no secretory phase, no mid-cycle rise in temperature вЂ“ Biopsy: Proliferative Endometrium with stromal breakdown. No secretory endometrium вЂ“ Tx: OCPs в– First step in management of heavy unremitting endometrial hemorrhage through menarche and perimenopause period пѓ high dose unconjugated estrogen (to suppress bleeding); Once stabilize D&C should be performed & pt should be put on OCP в– If perimenopausal women c/o an episode of heavy dysfunctional bleeding or irregular menses, then do endometrial surveillance in the form of vaginal ultrasound (to ensure endometrial thickness <4mm) or endometrial biopsy S.S.Patel. All rights reserved. Registered with US copy right office 123 Primary Amenorrhea Breast Absent Uterus Present Breast Present (secondary sexual characteristics present) Uterus Absent Next step? вЂ“ Karyotyping and Testosterone level Mullerian Agenesis (Rokitansky вЂ“ Kuster вЂ“ Hauser Syndrome) Normal female (XX) Normal Testosterone Tx: Surgical elongation of vagina Androgen Insensitivity (Testicular feminization) Genetically male (XY) Secondary female sexual characteristics Tx: Remove testis and give estrogen supplement Next step? вЂ“ Karyotyping and FSH level TurnerвЂ™s syndrome 45 (XO) в†‘в†‘ FSH Streak ovary, cystic hygroma (web neck) Tx: OCP (oral contraceptive pills) Hypothalamic Pituitary failure в†“в†“ FSH Absent secondary sexual characteristic в– Kallman Syndrome: anosmia + absent GnRH вЂ“ Tx: OCPs в– Secondary Amenorrhea: absence of menses for 3 months if previously regular menses or for 6 months if previously irregular menses п‚§ Hypogonadotropic (Hypothalamic or Pituitary dysfunction) п‚§ Hypergonadotropic (ovarian follicular failure) п‚§ Eugonadotropic (pregnancy, Anovulation, out-flow obstruction) п‚§ First step in management вЂ“ beta-hCG titer to rule out pregnancy п‚§ Next step? вЂ“ TSH level (hypothyroidism) Prolactin level (Hyperprolactinemia) п‚§ Next step? вЂ“ Progesterone challenge test (PCT) п‚§ If PCT positive вЂ“ diagnosis of Anovulation [Estrogen production is adequate] п‚§ If PCT negative вЂ“ inadequate Estrogen / Out-flow obstruction п‚§ Estrogen вЂ“ Progesterone challenge test (EPCT) п‚§ If EPCT positive вЂ“ inadequate Estrogen [Y-chromosome mosaicism, savage syndrome (ovarian resistant syndrome)] п‚§ If EPCT negative вЂ“ out-flow obstruction S.S.Patel. All rights reserved. Registered with US copy right office 124 п‚§ Cause of amenorrhea in patient who lost more weight вЂ“ estrogen deficiency (due to low LH and GnRH). They are also at risk of developing Osteoporosis п‚§ Premenstrual Syndrome (PMS): wide range of physical & emotional symptoms occur, typically two weeks before menstruation and disappear with menses for atleast 3 consecutive cycles Tx: SSRIs (fluoxetine); If it fails, give Alprazolam; Diuretics, Vit-B6 п‚§ c/o в†‘в†‘ hair growth in female Virilization (Hirsutism + Musculinizing signs like clitorimegaly, baldness, etc.) Hirsutism Congenital Adrenal Hyperplasia Gradual onset в†‘в†‘ 17-OH progesterone Tx: Corticosteroid replacement PCO (Polycystic Ovarian Syndrome) (SteinLeventhal Syndrome) в†‘в†‘ LH:FSH, Obese, в†‘ Testosterone Cystic appearance of ovary (neckless) on US Tx: OCP, Metformin Adrenal Tumor Rapid onset в†‘в†‘ DHEAS level Tx: Surgical removal Ovarian Tumor Rapid onset в†‘в†‘ Testosterone level Tx: Surgical removal Idiopathic Positive family history Normal level of all hormones Tx: Spironolactone п‚§ п‚§ S.S.Patel. First step in management of infertility in patient with PCOD вЂ“ weight loss; If it fails, try Clomiphen citrate or Metformin Metformin has shown to induce ovulation in patient with PCOD. It should be discontinued during pregnancy once pregnancy is documented All rights reserved. Registered with US copy right office 125 в– Infertility: п‚§ First step in management вЂ“ semen analysis п‚§ Screening test for ovulation вЂ“ BBT (basal body temp) & mid-cycle progesterone level п‚§ Luteal phase Defect пѓ Abnormal basal body temperature curve & low mid-luteal progesterone level. Confirmation of diagnosis? пѓ Endometrial biopsy. Initial Treatment? пѓ Progesterone supplement; if it fails, then? пѓ Clomiphene citrate / hMG (human menopausal gonadotropin) п‚§ Best measure for pt with premature ovarian failure who still want to conceive пѓ egg donation п‚§ Once diagnosis of premature ovarian failure is made (< 35 yrs of age, low estrogen, high FSH) next step? пѓ Chromosome analysis to rule out presence of Y chromosome в– п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Hormone replacement therapy in Menopause: Women with uterus вЂ“ Estrogen + Progesterone Women without uterus вЂ“ Estrogen Indication: vasomotor symptoms only (optimally do not exceeded 4 yrs) Estrogen alternatives: Tamoxifen, Raloxifen Raloxifen can be given in patient with positive family history for Breast CA to prevent osteoporosis First-line drug to prevent osteoporosis вЂ“ Bisphosphonates Conclusion from WHI trial for HRT (Hormone Replacement Therapy) No cardiovascular benefit Increased risk for stroke with long вЂ“ term use Increased risk for Breast CA [but not with unopposed estrogen] пЃ® Contraception: Estrogen causes Thromboembolism, not progestin п‚§ Contraception of choice in patient with bleeding disorders вЂ“ Depot Medroxyprogesterone acetate. (it is ideal for h/o PID, heavy menstrual bleeding, heavy smoking, fibroids, sickle cell disease) [Menorrhagia is the most common complication in women using вЂњNorplantвЂќ (six capsules of levonorgestrel)] п‚§ Emergency contraception вЂ“ levonorgestrel within 12-hrs (good efficacy within 48-hrs; appears to work up to 120-hrs after intercourse) п‚§ OCP decrease chances of ovarian CA п‚§ Pregnancy decrease chances of ovarian and breast CA п‚§ OCP decrease the risk of Gonococcal PID [Basis вЂ“ it increases thickness of mucous & prevent gonococci to enter in fallopian tube] п‚§ Advise regarding contraception in adolescents вЂ“ use barrier methods in addition to OCP (barrier method is useful to prevent STDs) п‚§ п‚§ S.S.Patel. Identical twins (monozygotic twins вЂ“ embryo split during blastomere & blastocyst stage) вЂ“ monochorionic, monoamniotic & monochorionic, diamniotic Lactation suppression пѓ tight lifting bra & ice packs All rights reserved. Registered with US copy right office 126 BREAST DISEASES в– Fibroadenoma в†’ young woman ( child baring age ) в†’ firm, rubbery mass, moves easily on palpation (Breast mouse ) в†’ sonogram of breast (best diagnostic Test ) в†’ Tx: Surgical Excision (optional) в– Cystosarcoma Phyllodes в†’ young woman в†’ very large mass в†’ benign but has potential to become malignant в†’ core / Incisional biopsy в†’ Tx: Surgical removal (mandatory) в– Fibrocystic Disease в†’ multiple bilateral breast cysts вЂњcome & goвЂќ at different time in menstrual cycle в†’ mammogram (first step) в†’ if persistent / dominant mass в†’ Aspiration (not FNA) в†’ still persist в†’ biopsy; if bloody fluid on Aspiration в†’ cytology в– Intraductal Papilloma в†’ young woman with bloody nipple discharge в†’ mammogram в†’ galactogram guided surgical resection в– Breast Abscess в†’ lactating mother в†’ fever, leucocytosis, fluctuating red, hot, tender mass in breast в†’ Tx: Incision & Drainage with biopsy of the abscess wall в– Breast cancer в†’ mammogram (irregular area of increased density with fine microcalcification) with core biopsy в†’ Lumpectomy with Axillary node sampling + Post-op-radiation ( small tumor , away from nipple and areola ) / Modified radical mastectomy (best choice) п‚§ If unresectable в†’ chemotherapy п‚§ If Axillary node positive в†’ chemotherapy п‚§ If hormone receptor sensitive в†’ Add Tamoxifen (Pre-menopausal) / Anastrazole (post-menopausal) п‚§ Breast cancer signs & Symptoms в†’ palpable breast mass, retraction of overlying skin, retraction of nipple, (вЂњorange peelвЂќ skin), palpable Axillary nodes. в– Ductal carcinoma in situ в†’ cannot metastasize ( No Axillary sampling ) . Л— Tx: Total simple mastectomy / Lumpectomy + Post-op- radiation п‚§ Tubular Breast CA в†’ Best prognosis п‚§ Inflammatory Breast CA в†’worst prognosis п‚§ Infiltrating Ductal CA в†’ most common п‚§ Lobular CA в†’ high incidence of bilateral involvement п‚§ Other types в†’ Medullary, Mucinous пЃ® Breast CA during pregnancy: same procedure for diagnosis as without pregnancy в—Џ Tx : same as without pregnancy except No chemotherapy in first trimester , No Radiotherapy during entire pregnancy . в—Џ There is no need for termination of pregnancy S.S.Patel. All rights reserved. Registered with US copy right office 127 TOXICOLOGY п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Ipecac вЂ“ to induce vomiting-within 1-2 hrs-conscious patient Gastric lavage вЂ“ patient with altered mental status/unconscious-within 1st hour except TCA ingestion in which anticholinergic action delay gastric emptying Both ipecac & lavage are contraindicated in caustic substance ingestion Charcoal вЂ“ administer every 2-4 hrs вЂ“ not effective in metal poisoning and different alcohol Poisoning Whole bowel irrigation вЂ“ for large volume pill ingestions in which the pills can be seen on an x-ray Dialysis вЂ“ When serious symptoms are present (coma, hypotension, etc.) Forced Alkaline Diuresis вЂ“ Salicylates (Aspirin), Barbiturate Sodium Bicarbonate- TCA (Tricyclic Antidepressants) Naloxone + Dextrose + Thiamine вЂ“ any patient who present with altered mental status (or) coma with unknown drug ingestion пЃ® Acetaminophen: (140 mg / kg toxic dose) Л— N-acetyl Cystine в†’ as early as possible Л— When more than 24 hrs have elapsed since ingestion, there is no specific therapy that can prevent OR reverse the toxicity. пЃ® Alcohols (methanol, Ethylene glycol, Isopropyl alcohol): Л— Methanol вЂ“ paint thinner, solvents, windshield washer- visual disturbances, blindness - в†‘anion gap metabolic acidosis Л— Ethylene glycol вЂ“ automotive antifreeze вЂ“ Renal failure & Oxalate crystals в†‘anion gap metabolic acidosis Л— Isopropyl alcohol вЂ“ Ketosis without elevated anion gap acidosis Л— Tx: ethanol infusion followed by hemodialysis, Fomepizole (alcohol dehydrogenase inhibitor) can be used instead of ethanol infusion. Л— Fomepizole infusion is now proffered for Tx of ethanol, methanol and ethylene glycol poisoning. Fomepizole should not be used simultaneously with ethanol during treatment of methanol and ethylene glycol poisoning пЃ® Carbon monoxide (CO) poisoning: burn around face, entrapment in fire, winter season; c/o headache, absent fever and negative meningeal signs, involvement of other family members at home Л— carboxyhemoglobin level (Best initial test / next step) Л— Tx: 100% O2 administration / hyperbaric O2 in severe cases. пЃ® Acids and Alkali ingestion: Upper Endoscopy (most important / next step in management) вЂ“ never try to neutralize (Acid by Alkali (or) Alkali by acid ) пЃ® Digoxin Toxicity: Л— GI symptoms, Neurologic & Visual Symptoms, Cardiac disturbance (Arrhythmia) S.S.Patel. All rights reserved. Registered with US copy right office 128 Л— Hypokalemiaв†’в†‘ Toxicity (K+ & digoxin, both compete for Na+-K+ ATPase pump) Л— EKGв†’ atrial tachycardia with AV block (specific for digoxin toxicity) Л— Tx: Repeated doses of charcoal, Digoxin-specific antibodies, correction of electrolyte abnormalities, phenytoin / lidocaine for ventricular arrhythmic пЃ® Lead poisoning: paint, soil, drinking water contaminated with lead вЂ“ Sideroblastic anemia, Lead lines on x вЂ“rays, Neuropathies (wrist drop) Tx: environmental / behavior intervention; If blood level >44 вЂ“ EDTA / succimer + environment intervention; If blood level >70 вЂ“ Hospitalization + IV BAL (Dimercaprol) [BAL is fast acting than EDTA] [Penicillamine in adults] [oral succimer in children]; for Dx use venous blood sample пЃ® Mercury Poisoning: thermometer, Sphygmomanometer Tx: Dimercaprol (BAL) / Penicillamine / Succimer пЃ® Arsenic Poisoning: garlic smell , watery diarrhea ( like in Cholera ) Tx: Symptomatic treatment пЃ® Cyanide Poisoning: Bitter Almond smell Tx: Amyl nitrate пЃ® Salicylates: Tinnitus (more specific complain) пЃ® TCA: H/O depression, Anticholinergic symptoms, Torsade de pointes [wide QRS complex (Indicators of severity of TA overdose), QT prolongation]. Tx: Immediate sodium bicarbonate (first step / next step in management) [M/A: alleviate cardio depressant action on sodium channel] пЃ® Opiates: pin point pupils, respiratory depression. Л— Tx: Naloxone пЃ® Cocaine: inhibit re-uptake of NE & DA. Л— Hypertension, Arrhythmia, Seizure, Abortion Л— Tx: Diazepam, Labetalol (anti-hypertensive) . пЃ® Benzodiazepines: somnolence, dysarthria, ataxia, stupor Л— Tx: Flumazenil (prepare to treat seizure from acute withdrawal from benzodiazepines antagonism) вЂў Slurred speech, unsteady gait and drowsiness in young patient пѓ Benzodiazapine Overedose [look for distinguishing features of other drugs present same way пѓ Opioid (miosis), Alcohol & phenytoin (nystagmus, Hand tremor)] пЃ® Barbiturates: Respiratory depression, absent EEG activity Л— Tx: Forced alkaline diuresis, supportive S.S.Patel. All rights reserved. Registered with US copy right office 129 пЃ® Hallucinogens: Marijuana, LSD, PCP (angel dust) Л— Tx: Benzodiazepines (eg. Diazepam) пЃ® Bradycardia, AV block, Hypotension & diffuse wheezing in Hypertensive Patient attempt suicide, diagnosis? пѓ Beta-blockers toxicity; Tx? пѓ Glucagon S.S.Patel. All rights reserved. Registered with US copy right office 130 TRAUMA в– First thing to do is maintain Airway, Breathing & Circulation. п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ Airway : must be secured first if there is expanding hematoma (or) emphysema in the neck Cricothyroidotomy (field) / Orotracheal intubation (ER, most preferred route) Cervical spine injury в†’ orotracheal (if head is secured) / nasotracheal Maxillofacial injury (in adult) в†’ Cricothyroidotomy / percutaneous transtracheal ventilation Maxillofacial injury (in child) в†’ Percutaneous needle Cricothyroidotomy (not the classic with tube which causes subglottic stenosis in child) followed by tracheostomy Cricothyroidotomy tube can be kept only for 48-hrs in adult. If ventilatory support required beyond 48-hrs, Tracheostomy should be done. Breathing: Breath sound on both sides of chest / pulse oxymatory Circulation: SBP should be > 90 mmHg, Palpable pulse Shock in Trauma в†“ Bleeding в†“ Low CVP в†“ Treatment Trauma center Near by Transportation в†“ Cardiac Temponade в†“ High CVP в†“ - No respiratory distress - Tx: Pericardiocentesis OR Pericardial window в†“ Tension Pneumothorax в†“ High CVP в†“ - Respiratory distress - No breath sound & Hyperresonant to percussion - Tracheal deviation - Tx: big needle inserted in upper pleural space followed by chest tube connected to underwater seal Trauma center far away IV fluids (Ringer lactate/Normal Saline) оЂЉ Any penetrating injury require surgical intervention and repair of the damage п‚§ п‚§ п‚§ Unconscious patient with Head trauma в†’ CT scan (1st step in management) Linear skull fracture(closed) в†’ left alone / observe Linear skull fracture (open) в†’ wound closure only (ER) S.S.Patel. All rights reserved. Registered with US copy right office 131 п‚§ Comminuted/ depressed skull fracture в†’ repair in OR (Operating Room) в– Acute Epidural Hematoma в†’ Middle meningeal artery в†’ hit on side of the headв†’ unconsciousness в†’ lucid interval в†’ unconsciousness; CT scan в†’ biconvex, Lens-shaped Hematoma Tx: Craniotomy в– Acute Subdural Hematoma в†’ Tear of bridging veins в†’ bigger trauma and much sicker & severe neurological damage в†’ CT scan в†’ semilunar, crescentshaped hematoma Tx: craniotomy п‚§ Chronic Subdural Hematoma: elderly / severe alcoholics в†’ Tearing bridging vein в†’ mental function deteriorates over several days to Weeks в†’ Tx в†’ Surgical evacuation. п‚§ Diffuse Axonal Injury в†’ CT scan shows diffuse blurring of the grayвЂ“white matter interface and multiple small punctate hemorrhages в†’ Tx : Prevent rise in ICP Gunshots in Neck в†“ Middle Zone в†“ Surgical Exploration ( even though patient is asymptomatic ) Upper / lower Zone в†“ Diagnostic procedures then Surgery в†“ в†“ Upper Zone в†“ Arteriography for Diagnosis & embolization . в†“ Lower Zone в†“ Arteriography Esophagogram Bronchoscopy в†“ Surgery п‚§ Stab wound to Neck в†’ Observe (if asymptomatic) п‚§ Stab wound to Neck, Crepitation at the base of neck в†’ Bronchoscopy (1st step) followed by bronchoscopy guided intubation (best choice) / orotracheal intubation п‚§ Blunt Trauma to the Neck в†’ X-ray / CT scan (tenderness over cervical spine, neurological deficits) в†’ Intervention п‚§ Signs & Symptoms of Spinal cord injuries в†’ High-dose prednisone в†’ MRI S.S.Patel. All rights reserved. Registered with US copy right office 132 п‚§ Rib fractures в†’ pain relief by nerve block (no strapping / binding) п‚§ Plain Pneumothorax (not tension) в†’ CXR в†’ Tx п‚§ Sucking Chest wound ( flap ) в†’ occlusive dressing (taped on 3 sides) п‚§ Hemothorax в†’ CXR в†’ Chest tube / Thoracotomy (> 1500 ml blood / > 600 ml in 6 hrs) п‚§ Flail chest в†’ segment of the chest wall cave inside during inspiration and bulge out during expiration in multiple ribs fractures вЂ“ Tx: fluid restriction, Diuretics, use of colloids, Respirators with bilateral chest tubes. (to prevent tension pneumothorax) п‚§ Pulmonary contusion в†’ вЂњwhite outвЂќ of the lungs on CXR в†’ Tx: same as above п‚§ Traumatic rupture of Diaphragm в†’ bowel in the left chest on X-ray в†’ Tx : Surgical repair from the abdomen в– Subcutaneous Emphysema в†’ CXR в†’ Bronchoscopy в†’ Surgical repair в– Traumatic rupture of Aorta в†’ deceleration injury / 1st rib /sternum fracture CXR (wide mediastinum) в†’ transesophageal echo / spiral CT в†’ Aortogram (if 2nd fail) в†’ surgical repair в– Air embolism: subclavian vein is opened to the air which sucks air during inspiration (hissing sound) в†’ sudden death (Supraclavicular node biopsy , CV line placement , CV line disconnected and leave it open to the air) в†’ Tx: Immediate head down and raised right shoulder в– Fat embolism в†’ long bones fracture (femur) в†’ CXR : Bilateral patchy infiltrates в†’ fever , tachycardia в†’ Tx : Respiratory Support в– Gunshot wound to the Abdomen (any entrance / exit wound below the level of the nipple) в†’ exploratory laparotomy в– Stab wound to the Abdomen (Stable patient with no protruding viscera) в†’ observe & Standard wound care в– Stab wound to the Abdomen (unstable patient / protruding viscera) в†’ exploratory laparotomy S.S.Patel. All rights reserved. Registered with US copy right office 133 в– Blunt abdominal Trauma в†’ Signs & Symptoms of peritoneal irritation в†’ Exploratory laparotomy в– Blunt Abdominal Trauma в†’ Signs & Symptoms of internal bleeding (shock) в†’ Stable patient в†’ CT scan в†’ Intervention в– Above scenario в†’ unstable patient в†’ Diagnostic peritoneal lavage / sonogram в†’ intervention в– Fractures of lower ribs on Left side в†’ rupture spleen в– Coagulopathy during operation в†’ FFP + platelate transfusion в– Prolonged laparotomy в†’ more IV fluids given в†’ Abdominal compartment Syndrome ( Abdomen canвЂ™t close without tension ) в†’ Tx : temporary cover with absorbable mesh / non вЂ“ absorbable plastic в– Pelvic Fracture в†’ External fixation / arteriographic embolization. в– Hemodynamically unstable with pelvic fracture due to blunt trauma вЂ“ first step? USG abd or Diagnostic peritoneal lavage to rule out intraperitoneal hemorrhage (hвЂ™ge) вЂ“ If negative, next step? Pelvic angiogram to rule out retroperitoneal hвЂ™ge вЂ“ if hвЂ™ge present, prepare for appropriate embolization в– H/O Trauma в†’ Blood in the Urine (urological injuries) Gross Hematuria в†“ sonogram ( Best ) / IVP Microscopic Hematuria в†“ в†“ в†“ Children Men ( Adults ) в†“ в†“ Asymptomatic / Symptomatic Asymptomatic в†“ (do nothing) Sonogram / IVP в†“ (When ask best diagnostic test for suspecting renal damage, Symptomatic Answer CT scans of abdomen) в†“ Sonogram / IVP - Penetrating urologic injuries (gunshot in lower abdomen) вЂ“ surgical exploration & repair - Pelvic Fracture + Hematuria (Blood at urethral meatus) в†’ retrograde urethrogram в†’ surgical repair S.S.Patel. All rights reserved. Registered with US copy right office 134 . - Pelvic fracture + gross blood on catheterization в†’ retrograde cystogram then surgical repair - Rib fractures, Abdominal contusion + Gross hematuria and normal retrograde cystogram в†’ CT scan Abdomen (Renal damage) - H/O Renal damage + few weeks after develop CHF & Flank bruit в†’ Traumatic arteriovenous Fistula & subsequent CHF - Blunt Renal trauma doesnвЂ™t require operation unless avulsion of renal pedicles which can be complicated by arteriovenous fistula later в– Sensation that patient wants to urinate but he canвЂ™t в†’ Posterior urethral injury в– Hematoma of penile shaft (corpora cavernosa fracture) в†’ urological emergency в†’ immediate surgical repair пЃ® Gunshot wound to Extremities в†“ No or vascular injury Unlikely в†“ Standard wound care в†“ possibility of vascular injury в†“ Arteriogram / Doppler в†“ Surgical repair if Injury present в†“ definite vascular injury в†“ surgical exploration And repair п‚§ Complex extremity Injury в†’ 1st fracture stabilization в†’ 2nd vascular repair в†’ lastly nerve repair п‚§ Crushing Injury в†’ Tx: plenty of fluids, diuretic, Alkalization of urine п‚§ Compartment Syndrome в†’ Fasciotomy S.S.Patel. All rights reserved. Registered with US copy right office 135 MISCELLANEOUS п‚§ Edema: Transudate (в†‘ Hydrostatic pressure & в†“ Oncotic pressure) Exudates (в†‘ vascular permeability) п‚§ п‚§ п‚§ п‚§ п‚§ Shock: Low perfusion pressure to tissues вЂ“ Hypovolemic (low circulatory volume); Cardiogenic (heart is not pumping well) and Septic (vasodilation leads to blood flow too quickly and give less time for tissue to extract oxygen) Cardiogenic shock вЂ“ low CO & High PCWP ARDS вЂ“ Normal PCWP [PCWP = pulmonary capillary wedge pressure] Septic shock вЂ“ High CO, Low PCWP & Normal mixed venous O2 Hypovolemic shock вЂ“ low CO, low PCWP, low mixed venous O2 п‚§ п‚§ п‚§ High PCWP & low CVP вЂ“ LV dysfunction [CVP = central vein pressure] High PCWP & high CVP вЂ“ Cardiac temponade Respiratory distress & high CVP вЂ“ Tension Pneumothorax вЂў Abdominal Aortic Aneurysm вЂ“ Atherosclerosis вЂ“ (rupture - left flank pain , hypotension, pulsatile mass) вЂ“ below renal artery origin is most common site вЂў Syphilitic Aneurysm вЂ“ Aortic arch aneurism, tertiary syphilis, vasculitis of vasavasorum, Aortic valve regurgitation, brassy cough due to stretching of Lt. recurrent laryngeal N by aneurysm вЂў Ehler-Danlos Syndrome: defect in type-I and type-III collagen вЂ“ poor wound healing, aortic dissection (MCC of death), hyper mobile joints вЂў Chrug Strauss Syndrome вЂ“ Vasculitis + eosinophilia вЂ“ Asthma (nocturnal chest tightness, short of breath, etc) вЂ“ Nephritic syndrome, positive p-ANCA вЂ“ Leukotrines inhibitors can cause Chrug Strauss Syndrome вЂў Capillary Hemangiomas in newborn regress with age вЂў Sturge-Weber syndrome вЂ“ Nevus flammeus (birth mark) on face in distribution of Ophthalmic branch of CN 5 вЂ“ Ipsilateral malformation of pia matter vessel overlying occipital & parietal lobes вЂў Takayasu arteritis (Pulseless disease) вЂ“ young asian girl вЂ“ granulomatous vasculitis of aortic arch вЂў Kaposi sarcoma вЂ“ HHV-8 вЂ“ malignant tumor of endothelial cells вЂ“ raised, redpurple flat lesion to plaque & nodules вЂў Bacillary angiomatosis вЂ“ benign capillary proliferation involving skin & viscera in AIDS вЂ“ simulate Kaposi sarcoma вЂ“ Bartonella Henselae (causative agent) S.S.Patel. All rights reserved. Registered with US copy right office 136 п‚§ Von Hippel Lindau вЂ“ Autosomal Dominant вЂ“ cerebellar hemangioblastomas, Pheochromocytoma, renal adenocarcinoma (high incidence) вЂў Kartagenar Syndrome: Л— Immotile cilia syndrome Л— Recurrent sinusitis, infertility & situs inverses вЂў - Rhabdomyosarcoma: Tumor of striated muscles. Head & Neck and genitourinary tract. Grape-like mass protruding through vagina (Sarcoma botryoides) вЂў - Osteogenesis Imperfecta: Blue sclera, brittle bones Defective synthesis of type-I collagen в– вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў HLA association with Diseases: HLA-A3 вЂ“ Hemochromatosis HLA-B27 вЂ“ Ankylosing Spondylitis HLA-DR2 вЂ“ Multiple Sclerosis, Goodpasture, Narcolepsy, Hay fever HLA-DR2,DR3 вЂ“ SLE HLA-DR3 вЂ“ Celiac Sprue, Dermatitis Herpatiformis HLA-DR3,DR4 вЂ“ Type-1 DM HLA-DR4 вЂ“ Rheumatoid Arthritis(RA), Pemphigus Vulgaris HLA-DR5 вЂ“ Pernicious Anemia, Juvenile RA HLA-DR7 вЂ“ Steroid-responsive Nephrotic Syndrome вЂў вЂў Hereditary Angioedema вЂ“ C1 esterase deficiency Paroxysmal Nocturnal Hemoglobinuria вЂ“ Defect in molecule anchoring decay accelerating factor(DAF) which normally degrade C3&C5 convertase on hematopoetic cell membranes therefore in the absence of DAF complement mediated Intravascular lysis of RBC occur(Hemoglobinuria) BrutonвЂ™s Agammaglobulinemia & Wiskott-Aldrich Syndrome are the only Xlinked recessive immune deficiency syndromes. BrutonвЂ™s Agammaglobulinemia вЂ“ tyrosine kinase deficiency. Wiskott-Aldrich Syndrome вЂ“ Eczema, Thrombocytopenia, deletion of T & B cells, Low IgM, Non-Hodgkin Lymphoma Phagocyte Dysfunction [CGD (Chronic Granulomatous Disease), ChediakHigashi Syndrome] вЂ“ Extracellular Bacteria (Staph. Aureus) + Fungi (Aspergillosis) T-Cells Deficiency (DiGeorge Syndrome) вЂ“ Intracellular Organisms (Virus, Candida, TB) but NOT Staph. Aureus, Hypocalcaemia due to absent parathyroid glands; 3rd&4th pouch defect, absent thymus too. B-cells Deficiency(BrutonвЂ™s Agammaglobulinemia) вЂ“ Extracellular Pyogenic Bacteria but NOT intracellular вЂў вЂў вЂў вЂў вЂў вЂў S.S.Patel. All rights reserved. Registered with US copy right office 137 вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў SCID вЂ“ Bacteria, Virus, Fungus; Adenosine Deaminase deficiency; Neutrophils - в†‘ or normal, B&T cells - в†“в†“в†“ C3 deficiency вЂ“ Pyogenic Bacteria C1,C4 or C2 deficiency вЂ“ Opsonization not efficient C6-8 deficiency вЂ“ Neisseria Infections в†‘IgM but deficient IgG & IgA вЂ“ CD40 ligand deficiency on activated T-cells At blood transfusion, blood types are appropriate to ABO & Rh typing, but still person develop Anaphylaxis в†’ Selective IgA deficiency. Patient may have h/o recurrent sinopulmonary infections. Leukocyte Adhesion Deficiency вЂ“ deficiency of selectins or ОІ2 integrins (CD11a:CD18) вЂ“ delayed separation of umbilical cord in newborn NADPH oxidase produce free radicals of oxygen вЂ“ Superoxide dismutase converts it in to H2O2 (called respiratory burst) вЂ“ Myeloperoxidase combine it with Cl and form hypochlorus free radicals which kills organisms Chronic Granulomatous Disease вЂ“ absent NADPH oxidase вЂ“ absent respiratory burst [negative NBT (Nitro-blue tetrazolium)] Myeloperoxidase deficiency вЂ“ respiratory burst occurs вЂ“ so able to kill streptococcus species (catalase negative) but not staphylococci (catalase positive) JobвЂ™s syndrome вЂ“ defective Chemotaxis (staph infection) and в†‘ IgE (eczema) вЂў Decompression sickness (CaissonвЂ™s Disease) вЂ“ rapid ascent of deep sea drivers leads to formation of nitrogen gas bubble which occludes vessels lumen and causes thrombo-embolic events вЂ“ Tx: recompression by forcing nitrogen to solution again by increasing pressure and slow decompression вЂў Prader-Willi syndrome вЂ“ Microdeletion syndrome with hypogonadism, mental retardation, short stature, and obesity (chromosome 15 deletion is of Paternal origin) Angelman syndrome вЂ“ chromosome 15 deletion is of maternal origin (child continuously laughing) Cancers caused by radiation вЂ“ Acute Leukemia, Papillary CA of Thyroid CA due to smoking: mouth, larynx, esophagus (SCC), pancreas, Urinary bladder CA due to alcohol: oropharyngeal, upper to mid esophageal, HCC вЂў вЂў вЂў вЂў вЂў п‚§ п‚§ п‚§ п‚§ п‚§ п‚§ S.S.Patel. Ectopic Hormones & Tumor relationship: ACTH: most common ectopic secretion вЂ“ secreted in Small cell CA of Lung and Medullary CA of thyroid вЂ“ produce Cushing Syndrome ADH: secreted in Small cell CA of lung вЂ“ produce SIADH ОІвЂ“hcg: secreted in Trophoblastic tumors and germ cell tumors вЂ“ produce Gynacomastia and hyperthyroidism (similar to TSH) Erythropoietin: secreted in Renal cell CA and HCC (hepatocellular CA) вЂ“ produce secondary polycythemia Insulin-like peptide: secreted in HCC вЂ“ produce hypoglycemia Calcitonin: secreted in Medullary CA of thyroid вЂ“ produce hypocalcemia All rights reserved. Registered with US copy right office 138 п‚§ п‚§ PTH-like peptide: secreted in Small cell CA of Lung, Renal cell CA, Breast CA, Ovarian CA вЂ“ produce Hypercalcemia (low PTH) Serotonin: secreted in Carcinoid syndrome, SCC, Medullary CA of thyroid вЂ“ produce diarrhea, flushing, Valvular insuffiency (tricuspid insufficiency & Pulmonic stenosis) п‚§ Prostate cancer вЂ“ osteoblastic metastasis (в†‘ Alkaline phosphate) All other cancers which metastasize to bone has osteoclastic effect. п‚§ Down Syndrome (trisomy - 21) вЂ“ endocardial cushion defect (Atrial & ventricular septal defect) - в†‘ risk of Hurschprung disease & duodenal atresia - в†‘ risk for leukemia (Acutemegakaryocytic - вЂ№ 3yrs, ALL - вЂє 3yrs) - AlzheimerвЂ™s disease by age of 35yrs. EdwardвЂ™s Syndrome (trisomy вЂ“ 18) вЂ“ VSD, clenched hands with overlapping fingers, вЂњRocker bottom feetвЂќ PatauвЂ™s Syndrome (trisomy вЂ“ 13) вЂ“ VSD, cleft lip & cleft palate D E P вЂ“ 21, 18, 13 п‚§ п‚§ п‚§ п‚§ TurnerвЂ™s Syndrome вЂ“ Pre-ductal coarctation & bicuspid aortic valve, primary amenorrhea, cystic hygroma п‚§ Most of the spontaneous abortions are due to trisomy 16 вЂў - Marfan Syndrome: Defect in synthesizing fibrillin Mitral valve Prolapse, Aortic dissection. Subluxated lens, arachodactyly. Most common finding in patient with Marfan syndrome вЂ“ dural ectasia (90% of cases) [require MRI of spine for diagnosis] [lens dislocation and aortic dilatation вЂ“ 80% of cases] вЂў - Klinefelter Syndrome:( 47,XXY): Hypogonadism, Infertility, Gynacomastia в†‘в†‘ FSH & LH, в†“ в†“ Testosterone вЂў Л— вЂў Л— Л— Kartagenar Syndrome: Immotile cilia syndrome Recurrent sinusitis, infertility & situs inverses Fragile X-Syndrome: CGG repeat sequence Mental retardation, enlarge testis, prominent jaw, large ears п‚§ п‚§ п‚§ Low protein diet should be given in patient with renal failure and cirrhosis Kwashiorkor вЂ“ inadequate protein intake вЂ“ edema Marasmus вЂ“ inadequate calorie intake вЂ“ extreme muscle wasting S.S.Patel. All rights reserved. Registered with US copy right office 139 п‚§ п‚§ п‚§ Anorexia nervosa вЂ“ distorted body image Bulimia nervosa вЂ“ binging & purging (self induce vomiting) Vit-E вЂ“ decrease synthesis of Vit-K dependent coagulation factor в– - Lesch вЂ“ Nyhan Syndrome: Defective purine metabolism Deficient HPRT (HGPRT) Child tendency to compulsively bite his finger (self mutilation) Mental retardation HPRT Hyperuricemia is due to в†“ IMP (Hypoxanthine > IMP) в– Л— Л— Л— Tay Sachs Disease: Hexosaminidase A deficient Ganglioside accumulate in cells Charry red macula, No Hepatomegaly and cervical lymphadenopathy в– - Niemann вЂ“ Pick: Sphingomyelinase deficiency Sphingomyelin accumulate in cells Characteristic foamy macrophage, charry red macula but Hepatomegaly, & cervical Lymphadenopathy present в– Л— Л— Л— GaucherвЂ™s Disease: Gluocerebrosidase deficiency glucocerebroside accumulate in cells characteristic Macrophage (crumpled paper inclusion) в– - Klein- Waaredenberg Syndrome: mutation in PAX gene Dystonia Canthorum ( Lat. displacement of inner corner of eye ) Pigmentary abnormality Congenital Deafness, Limb abnormalities в– Л— Л— Л— Л— Phenylketonuria: Phenylalanine Hydroxylase Deficiency (Phenylalanine в†’ tyrosine) Musty odor from child, mental retardation Aspartame (artificial sweeteners) must be strictly avoided by phenyketonurics в†‘ Phenylalanine level in pregnant woman в†’ mental retardation in Infants в– Homogentisate Oxidase Deficiency: - accumulation of homogentisic acid in blood & excretion in urine - Ochronosis (accumulation of pigments in cartilages) в– Maple Syrup Urine Disease: Л— Branched chain ketoacid dehydrogenase deficiency S.S.Patel. All rights reserved. Registered with US copy right office 140 Л— impaired metabolism of Valine, Leucine, Isoleucine Л— Maple syrup odor in urine, Ketosis, coma, & death if not treated в– Л— Л— Л— Л— Л— Acute Intermittent Porphyria: Uroporphyrinogen вЂ“ 1 synthase deficient Episodic variable expression Acute abdomen (вЂњbelly full of scarsвЂќ) , brief psychosis No photosensitivity (в†‘Оґ ALA , PBG) Never give Barbiturates, Pyrazinamide, Gresiofulvin в– - Porphyria Cutanea Tarda: Uroporphyrinogen decarboxylase deficient Photosensitivity (в†‘ uroporphyrin 1) [urinary uroporphyrin - diagnostic test] Painless blistering on sun exposed area вЂ“ Tx: stop alcohol & estrogen use в– Homocystinuria: MarfanвЂ™s features, Arthrosclerosis in childhood, recurrent DVT Cystathione Synthase (Homocystine Cystathione) Vit-B6 п‚§ п‚§ Causes: Cystathione synthase deficiency, Vit- B6 deficiency, Homocystine methyltransferase deficiency, Folic acid & Vit- B12 deficiency Methionine is degraded via the HomocystineвЂ“Cystathione Pathway; so methionine is elevated in patient with cystathione synthase deficiency via activation of Homocystine methyltransferase by excess substrate homocystine. Galactosemia/Galactosuria п‚§ galactokinase deficiency Fructosemia/Fructosuria п‚§ Fructokinase deficiency - в†‘galactose in blood - cataract (Aldose reductase) - galactokinase trap galactose in cell by phosphorylation as galactose-1-phosphate п‚§ Gal-1-Uridyltransferase Deficiency - в†‘ fructose in blood - No cataract - fructokinase trap fructose in cell by phosphorylation as fructose-1-phosphate п‚§ Aldolase B Deficiency - convert galatose-1-phosphate into glucose-1-phosphate - If it deficient, galactose-1-phosphate accumulate in cells and produce symptoms - Liver, Brain & other tissue. - Symptoms evident while on Breast milk, so early onset of symptoms after birth - convert fructose-1-phosphate into DHAP & Glyceraldehydes -If it deficient, fructose-1-phosphate accumulate in cells and produce symptoms. - Liver , kidney - Symptoms are not evident while on Breast milk, so late onset of symptoms after birth - No cataract - cataract S.S.Patel. All rights reserved. Registered with US copy right office 141 - Hypoglycemia, Lactic acidosis, Jaundice, Mental retardation - Avoid milk & milk product - Hypoglycemia, Lactic acidosis, Jaundice, Proximal Renal tubular disorder resembling FanconiвЂ™s syndrome - Avoid Honey, table sugar which contain sucrose вЂў Cretinism вЂ“ hypothyroidism in child вЂ“ protruding tongue, prominent fontanels, chubby, umbilical hernia, short stature вЂў Short Stature: Half of adult height should be achieved by age of 2 yrs. вЂ“ short stature could be due to constitutional delay or due to genetic cause вЂ“ X-ray of hand & wrist (best test) вЂ“ Constitutional delay [Bone age вЂ“ 5 yrs old in 8 yrs old child] Genetic cause [Bone age 8 yrs in 8 yrs old child] [Low dose of testosterone can be used for Tx of constitutional delay, but only used for short time] вЂў Failure to thrive вЂ“ dietary modification is the best initial Tx. If history and physical finding suggestive of any organic cause, order lab test. If patient doesnвЂ™t improve within 4-6 wks of oral feeding, admit to the hospital and start nasogastric feeding. If child neglect / abuse are suspected, admit to the hospital. Daily calories up to 1 years of age is 100 kcal/kg вЂў Bilious vomiting in an infant means there is a malrotation with Volvulus until proven otherwise вЂ“ Tx: laparotomy вЂў Mongolian spots: flat blue (or) gray lesions with well-defined margins; disappear in first few years of life. Most common over the presacral area D/D: bruises of child abuse вЂ“ fade into surrounding skin and have different colors вЂў EpsteinвЂ™s pearls (Milia) : pearly white small inclusion cysts. вЂў - Cutis Marmorata : vasomotor response to cold stress. Skin assumes a lacy pattern similar to cobblestones. Persistent form is seen in trisomy 21, trisomy18. оЂЉ Erythema toxicum: Eosinphilis filled small papule(or)pustules on an erythematous base. D/D: Staphylococcal scalded skin syndrome : very ill looking infants, culture positive for Staph.Aureus, neutrophils in the lesions. вЂў - S.S.Patel. Salmon Patch: flat vascular lesion that disappear with time. glabella, eyelids, nuchal area D/D: Sturge-Weber Syndrome (Port wine stains): (вЂњbirth markвЂќ) on face in the distribution of the ophthalmic branch of CN-5. Remains permanently All rights reserved. Registered with US copy right office 142 вЂў Capillary Hemangiomas: bright red, reminiscent of a strawberry which regresses spontaneously. вЂў Nevus Sebaceous (of Jadassohn): yellow-orange hairless plaques resembling flat warts located on the scalp Removed in adolescence because of potential to become malignant вЂў вЂў Cephalhematoma вЂ“ subperiosteal bleed, does not cross suture lines, resolve spontaneously. Caput Succedaneum вЂ“ swelling of scalp, cross suture lines, resolve spontaneously. вЂў Subcutaneous fat necrosis в†’ rubbery, firm nodules on cheeks (or) buttocks occur during difficult labor, particularly forceps (or) vacuum extraction, resolve spontaneously. вЂў - Neonatal drug withdrawal: Infants born to actively addicted mothers Most common drugs вЂ“ Heroin (48 hrs) & Methadone (2-6 weeks) hyperactivity, irritability, fever, tremors/jitters, high-pitched crying, vomiting, poor feeding, seizures Tx: narcotics, sedatives and hypnotics as well as swaddling and reducing noxious stimulation вЂў Adrenal hemorrhage is seen almost exclusively in newborn and is an indicator of birth trauma, stress, anoxia or dehydration. Follow up with US in 1-2 wks вЂў Newborn can lose up to 10% of wt during first week вЂў Facial Palsy in Infant : affected part of face doesnвЂ™t move when baby cry вЂў GERD in Infants : h/o spitting on burping, h/o aspiration pneumonia (Rt. lung pneumonia), wheezing вЂў - Breast Milk: immunologic factors: IgA, lactoglobulins and maternal macrophage Low Vit-k в†’ All newborn infants should receive Vit-k at birth to prevent hemorrhagic newborn disease. Vit-D supplementation в†’ if motherвЂ™s intake is inadequate / if there is a limited sun exposure to baby. Iron supplementation for baby on cow milk (whole milk). Folate & vit-B6 supplementation for baby on goat milk Mother with HBsAg positive can breast feed her child immediately after birth Contraindication to breast feeding вЂ“ HIV positive mother, Lithium, mother taking cocaine, heroin, PCP, marijuana, nicotine Breast feeding should continue until age of 1 yr, start iron supplementation at age of 6 months Mother should breastfeed infant atleast every 4-hrs S.S.Patel. All rights reserved. Registered with US copy right office 143 п‚§ - Attention Deficit Hyperactive Disorder (ADHD) Inability to attend to the task at hand, increased motor activity and impulsivity. Tx: methylphenidate / Dextroamphetamine & psychological therapy. п‚§ - Enuresis: Day & night bladder control is usually attained by age 5 yrs. first step in management of pt with enuresis пѓ urine analysis Causes вЂ“ UTI, urinary tract abnormality, Psychogenic, developmental delay of the bladder, Diabetes, Stress incontinence, waiting too long to void Tx: Non pharmacological в†’ в†“ water intake at night, alarm system, reward system. Pharmacological в†’ Imipramine, Desmopressin - п‚§ п‚§ - Asperger Syndrome: more communicative, appear more socially aware; do not have language impairments, repetitive behavior. Rett syndrome: neurodegenerative disorder, only affect females. Stereotypic hand movement (hand wringing) & Acquired microcephaly. в– ENT: Tx of benign positional vertigo вЂ“ canalith repositioning procedures вЂў Non-sedating nasal decongestant (Pseudoephedrine) before diving has been shown to reduce the ear and sinus barotraumas by 75% вЂў Adolescent with nasal mass & epistaxis вЂ“ angiofibroma (MCC of nasal mass) вЂў Ingestion of button battery in child should be removed immediately with endoscopy because it contains heavy metals which can produce tissue damages. For other foreign body ingestion, wait and watch (comes out in stool) вЂў Tx of external otitis вЂ“ cleaning of ear canal with cerumen wire loop or cotton swab (first step) [cleaning with hydrogen peroxide is an alternative method if tympanic membrane is visualize and it is intact] вЂў Nasal cytology is the best step in management of patient with rhinitis вЂў Patient c/o aural fullness, audible popping sound while swallowing, intermittent ear pain and hearing loss, diagnosis? вЂ“ Eustachian tube dysfunction вЂў Hereditary hearing loss вЂ“ sensorineural вЂў Osteosclerosis вЂ“ Autosomal dominant, conductive hearing loss, absent stapedial reflex в– Ophthalmology: Orbital cellulitis вЂ“ admit the patient in the hospital вЂ“ start IV broad spectrum antibiotics (for at least 3 days) then continue on oral regimen for few days as an out-patient follow up вЂў Orbital cellulitis вЂ“ unilateral proptosis, normal fundoscopy вЂў Cavernous sinus thrombosis вЂ“ bilateral proptosis, papilledema вЂў Tx of Diabetic retinopathy вЂ“ Proliferative (disc neovascularization) вЂ“ immediate laser photocoagulation; Non-proliferative вЂ“ Retinoscopy (to check any macular edema) followed by elective laser photocoagulation вЂў Ocular Melanoma вЂ“ ocular ultrasound (for diagnosis) and MRI (to check extrascleral extension) вЂ“ Tx: Enucleation S.S.Patel. All rights reserved. Registered with US copy right office 144 вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў Blepharospasm вЂ“ periodic involuntary closure of eye (form of focal Dystonia) вЂ“ Tx: Botulinum toxin injection Anterior Uveitis (iritis) вЂ“ pain, redness, photophobia, irregular pupils and leucocytes in anterior chamber вЂ“ around 25% of patient with Sarcoidosis have uveitis Endophthalmitis вЂ“ ophthalmic emergency вЂ“ intraocular surgery, h/o eye injury; c/o light flashing, severe pain, on examination hypopyon and failure to visualize retinal vessels (80% of cases) вЂ“ urgent ophthalmic referral is necessary вЂ“ Tx: Vitreous or Aqueous culture and Intavitreal antibiotics; Vitrectomy; Enucleation Tx of Candida Endophthalmitis вЂ“ vitrectomy + IV Amphotericin B Amblyopia в†’ vision impairment resulting from interference with the processing of images by the brain during the first 6-7 yrs of life в†’ Strabismus / Congenital cataract / Retinoblastoma в†’ Initial treatment of Amblyopia: occlusion therapy (continuous covering of normal eye) в†’ correction of problem needed as early as possible Intermittent painless loss of Vision in elder (Amaurosis fugax), next step? Duplex study of neck. Cause? вЂ“ emboli Dacryocystitis вЂ“ infection of lacrimal sac Hordeolum (Stye) вЂ“ Abscess located over the upper or lower eyelid Herpes simplex Keratitis пѓ corneal vesicle & dendritic ulcers Herpes zoster ophthalmicus пѓ elderly patient вЂ“ burning & itching sensation in periorbital area, vesicles in the distribution of the cutaneous branch of the first branch of trigeminal nerve (Ophthalmic N) HSV keratitis in HIV пѓ painful lesion, fundoscopic findings of peripheral Pale lesions & central retinal necrosis CMV retinitis in HIV пѓ painless, fundoscopic findings of hemorrhage and fluffy or granular lesions around the retinal vessels Grade of Hypertensive Ratinopathy (I пѓ IV): Av nicking пѓ copper wiring пѓ silver wiring, flame shaped hemorrhage (hвЂ™ge), Exudates пѓ papilledema Central Retinal Vein Occlusion: painless вЂ“ disc swelling, vein dilated & tortuous, retinal hemorrhage & cotton wool spots Central Retinal Artery Occlusion: painless вЂ“ pallor of optic disc, cherry red fovea and boxcar segmentation of blood in the retinal vein Follicular conjunctivitis & neovascularization of cornea (pannus), diagnosis? вЂ“ Trachoma Sympathetic Ophthalmia: вЂњspared eye injuryвЂќ вЂ“ immune mediated inflammation of one eye (the sympathetic eye) after a penetrating injury to the other eye вЂ“ mechanism? вЂњuncovering of hidden antigenвЂќ Open angle glaucoma вЂ“ central vision spared Macular degeneration вЂ“ peripheral vision spared (Tx: laser photocoagulation) в– Preventive Medicine: Mx of exposed health care worker to TB вЂ“ place PPD now and repeat it in 3-wks вЂў Nicotine replacement is preferred over buproprion for the management of nicotine withdrawal during early stage of smoking cessation вЂ“ give behavioral counseling S.S.Patel. All rights reserved. Registered with US copy right office 145 вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў S.S.Patel. and nicotine patch [First counseling then treatment; If you have never counseled patient regarding smoking cessation, do not pick prescribe nicotine patch or any other treatment for smoking cessation (most probably wrong answer)] CXR is not done routinely for screening purpose for lung cancer There are no screening test indicated routinely for many cancers like bladder CA, Ovarian CA, pancreatic CA, lung CA, Hepatocellular CA, etc so do not pick answer telling you order this screening test to rule out this CA Prophylaxis of meningococcal meningitis вЂ“ Rifampin Prophylaxis of meningococcal meningitis in women taking OCP вЂ“ ciprofloxacin HRT can cause significant elevation in triglyceride. If hypertriglyceridemia doesnвЂ™t solve after discontinuation of HRT and dietary modification , start treatment Smoking cessation has shown benefit in patient with osteoporosis Cigarette smokers have been shown to have more wrinkles than do nonsmokers Dehydration aggravate skin damage and therefore patient going on vacation in a area with lot of sun exposure should be educated to drink more water and wear protective clothes Prevention of malignant melanoma вЂ“ Protective clothing Most important risk factor in stroke вЂ“ HTN A clear association has been found b/w excessive alcohol intake & development of hypertension Several studies has suggested strong correlation b/w alcohol intake and colon CA Colonoscopy should be started at age of 50 yrs in a general population with average risk and at age of 40 yrs or 10 yrs earlier than detection of colon CA in family member, whichever come first Patient with ulcerative colitis and pancolitis should begin surveillance colonoscopy after 8 yrs of having the disease Do nothing after complete resection of <2cm pedunculated adenomatous polyp; surveillance вЂ“ colonoscopy after 3 yrs; If negative at 3 yrs follow up, surveillance colonoscopy period can be extended to 5 yrs. Mammography screening вЂ“ started b/w 40-49 yrs of age вЂ“ every 2 yrs; After 50 yrs of age вЂ“ annually [The American Medical Association, American College of Radiology, American Cancer Society, American College of Obstetricians and Gynecologists, and the USPSTF all support mammography screening beginning at age 40; In patient with h/o breast CA in first degree relative, begin at 10 yrs earlier than detection of breast CA in family member] Pap smear вЂ“ within 3 yrs after onset of sexual activity or age of 21 yrs, whichever occur first [some says age of 18 yrs] [If 3 annual pap smears are negative, then do pap smear every 2 yrs until age of 70 yrs] [Annual pap smear screening is recommended for all women regardless of their sexual orientation (hetero/homosexual women)] Prostate CA screening вЂ“ African-American and individual with family h/o prostate CA should begin annual screening (PSA level and digital rectal exam) at age of 40 yrs; at age of 50 yrs for everyone else Screening of DM is indicated in everyone aged 45 yrs or older. [FBS] All rights reserved. Registered with US copy right office 146 вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў вЂў Gestational Diabetes Screen вЂ“ b/w 24-28 wks вЂ“ 1-hr 50g oral GTT (screening test) вЂ“ If level >140 mg/dl, do definitive test (3-hrs 100g oral GTT done after overnight fasting) вЂ“ FBS (<126 gm/dl), After 100g oral GTT вЂ“ at 1-hr (<180 gm/dl), at 2-hrs (<155 mg/dl), at 3-hrs (<140 mg/dl) Triple marker screen вЂ“ b/w 15-20 wks вЂ“ MS-AFP, hCG, Estriol - в†“в†“ (MS-AFP & Estriol), в†‘ hCG в†’ Trisomy 21 (Down syndrome), next step? вЂ“ Amniocentesis; в†“в†“в†“ (All three) в†’ Trisomy 18, next step? вЂ“ Amniocentesis for karyotyping Rh-isoimmunization вЂ“ Blood typing at first prenatal visit вЂ“ If Rh-negative mother, Indirect CoombвЂ™s test at 28 wks of gestation [<1:8 вЂ“ no fetal risk]; give RhoGAM to all Rh-negative mothers at 28-wks of gestation RhoGAM is given to Rh-negative mothers at 28-wks of gestation, after delivery of Rh-positive infant, within 72-hrs of amniocentesis, D&C, chorionic villous sampling Chlamydia Trachomatis Screening вЂ“ All sexually active women age 24-yrs and younger and in other women who are at increased risk of STDs CDC recommended vaccination id Adult вЂ“ Td (every 10 yrs after age of 18 / last booster at age of 50), acellular Pertusis (booster b/w age of 19-64), Influenza (All adults >50 yrs of age вЂ“ IM route; Healthy 5-49 yrs of age, non-pregnant women вЂ“ Intranasal), Pneumococcal (All adults >65 yrs of age) Vaccination in HIV positive patient вЂ“ Influenza (IM), MMR, S. pneumoniae, Hepatitis First trimester screening tests вЂ“ CBC, Rubella IgG antibody, Hep B, Blood type, Rh & Antibody screen, cervical culture, urine culture, PPD, VDRL/RPR, Pap smear and HIV ELISA [require specific consent] Screening tests for All newborn вЂ“ Hypothyroidism, Galactosemia, Phenylketonuria Voiding after intercourse has been shown to decrease the risk of UTI in sexually вЂ“ active females Breast cancer prevention trial suggest that Tamoxifen reduce the risk of breast cancer in patients who have an increased risk of developing breast cancer [Ideal body weight for female вЂ“ 5 feet height = 100 lbs then add 5 lbs for each inch increase in height; Ideal body weight for male вЂ“ 5 feet height = 106 lbs then add 6 lbs for each inch increase in height] Drugs ACE inhibitors Beta blockers Aspirin Home Oxygen Spironolactone Decrease long term mortality in different condition CHF, Prevent diabetic nephropathy, reduce insulin resistance Acute MI, Perioperative period Acute MI COPD, Right sided heart failure due to pulmonary hypertension With ACE inhibitors in CHF Good Luck S.S.Patel. All rights reserved. Registered with US copy right office 147