Paeds SBA Lecture Henry Taylor Alex Nesbitt Question 1 A two year old boy is brought into A&E following 2 days of cough, coryza and a fever. Today the cough changed and he developed stridor. He is currently sitting on his mother’s lap and has no signs of respiratory distress. He is active and has a temperature of 37.8. Question 1 What is the single most appropriate treatment for James? A Oral dexamethasone B Nebulised adrenaline C Nebulised salbutamol D Humidified oxygen E Amoxicillin Croup Mild Active child, audible stridor but no signs of respiratory distress Moderate Stridor with retractions/recession and decreased air entry Severe Stridor at rest with expiratory component, increased resp effort, retractions/recession, tachypnoea and pallor Other respiratory conditions What key features of each of these might be mentioned in a SBA stem? Bronchiolitis Viral induced wheeze/asthma Pneumonia Pertussis Cystic fibrosis Epiglottitis Diphtheria Question 2 Horatio (aged 3) has been brought into the GP surgery by his mother. He began coughing lest week and was “a bit hot” and lethargic. Since then, his eyes have become red and sore. He has now developed a rash (next slide). You check his notes and find that he has not received any immunisations since he was 6 months old. Question 2 Question 2 What is the single most likely complication of this condition? A Acute otitis media B Cellulitis C Pneumonia D Meningitis E Status epilepticus Question 2 What is the single most likely complication of this condition? A Acute otitis media B Cellulitis C Pneumonia D Meningitis E Status epilepticus Koplik Spots Viral Exanthems Name Cause Pattern of illness Complications Measles Measles paramyxovirus Cough, coryza & conjunctivitis. (Koplik spots) Acute otitis media, Bronchopneumonia, Encephalitis, SSPE Scarlet fever S.Pyogenes (toxin) Fever, rash, strawberry tongue, (Forcheimer spots) Infective (URTI, Sepsis) & Immune (GN, Rh Fever) Rubella Rubella togavirus Rash: starts on face & spreads to body. Sub-occipital lymphadenopathy Caution: Pregnant mothers Mumps Mumps paramyxovirus Fever & headache -> Parotidomegaly +/- rash Orchitis/Oophoritis, Pancreatitis, Meningitis Fifth disease/Erythema infectiosum Parvovirus B19 Low grade fever & rash: slapped cheek/lacelike Aplastic crisis, Caution: Pregnant mothers Roseola infantum HHV6 & 7 Sudden high fever for 3 days then rash Febrile convulsions Kawasaki disease Vasculitis Persistent fever (7d), followed Coronary artery by rash, conjunctivitis, aneurysms lymphadenopathy, skin desquamation Question 3 A 6 week old boy has been jaundiced for the past 5 weeks. His serum bilirubin is 140µmol/L (unconjugated 80µmol/L; conjugated 60µmol/L). He is clinically well, exclusively breast fed, and has pale yellow stools. What is the most likely diagnosis? A. B. C. D. E. Physiological jaundice Hereditary Spherocytosis Breast milk jaundice Congenital toxoplasmosis Biliary atresia Neonatal Jaundice Total serum bilirubin (µmol/L) 200 Early Severe hyperbilirubinaemia Prolonged Jaundice presenting <24 hours • • Jaundice persisting after 2 weeks • • Due to increased RBC breakdown + immature liver function; occurs in 60% of neonates. Present from 24 hrs to 2 weeks, bilirubin levels <200µmol/L. • Cephalohaematoma, prematurity + early and late causes Physiological Haemolytic, Infections (TORCH), Congenital (CN, Gilbert) • • • • • 24 hrs Breast-milk jaundice Infections (UTI) Cholestasis (Biliary atresia, choledochal cyst) Metabolic (galactosemia, α1-AT) Endocrine (hypothyroid) 2 weeks time Unconjugated Conjugated Early Neonatal Jaundice Haemolytic disorders Present early (first 1-3 days of life) Cause an unconjugated hyperbilirubinaemia • Haemolytic disease of the newborn (HDN) – – – • Glucose 6-phosphate dehydrogenase deficiency (G6PD) – • Aetiology: X linked enzyme deficiency -> no glutathione production in RBCs -> vulnerable to oxidative stress; also impaired bilirubin conjugation in hepatocytes. Hereditary Spherocytosis – – • Aetiology: rhesus incompatibility or ABO incompatibility ABO incompatibility is more common, but produces a much milder, self-limiting disease Ix: Coombs test (DAT) Aetiology: AD>AR; RBC cytoskeletal genes Ix: osmotic fragility test, blood film, PCR ℞ - phototherapy -> exchange transfusion Prolonged Neonatal Jaundice Unconjugated hyperbilirubinaemia • Breast milk jaundice – Presents >24 hours, usually resolves by 5-6 weeks – Aetiology: incompletely understood; breast feeding: • Inhibits conjugation in liver • Increases enterohepatic circulation – Ix: monitor bilirubin levels – transcutaneous, capillary, or venous sample. – ℞: usually none needed, phototherapy Prolonged Neonatal Jaundice Conjugated hyperbilirubinaemia • Biliary atresia – Presents >24 hours, persists >2 weeks. – Aetiology: inflammatory obliteration of biliary tree causes cholestasis – Moderately elevated total serum bilirubin Conjugated bilirubin >20µmol/L or >20% total bilirubin. – Pale stools, dark urine – Ix: Abdo US, HIDA scan, biopsy, intraoperative cholangiography – ℞: Kasai procedure (hepatoportoenterostomy) -> liver transplant Question 3 A 6 week old boy has been jaundiced for the past 5 weeks. His serum bilirubin is 140µmol/L (unconjugated 80µmol/L; conjugated 60µmol/L). He is clinically well, exclusively breast fed, and has pale yellow stools. What is the most likely diagnosis? A. B. C. D. E. Physiological jaundice Hereditary Spherocytosis Breast milk jaundice Congenital toxoplasmosis Biliary atresia Question 3 A 6 week old boy has been jaundiced for the past 5 weeks. His serum bilirubin is 140µmol/L (unconjugated 80µmol/L; conjugated 60µmol/L). He is clinically well, exclusively breast fed, and has pale yellow stools. What is the most likely diagnosis? A. B. C. D. E. Physiological jaundice Hereditary Spherocytosis Breast milk jaundice Congenital toxoplasmosis Biliary atresia Question 4 A 4 week old girl with Down’s syndrome has nonbilious, non-bloody vomiting occurring shortly after most feeds. She is formula fed, gaining weight, and passing normal stool. What is the most likely diagnosis? A. B. C. D. E. Duodenal atresia Gastro-oesophageal reflux Pyloric stenosis Cow’s milk protein allergy Intestinal volvulus Infantile Vomiting Disorder Typical Age Features Incidence Ix ℞ Gastrooesophageal reflux <6 months 40% Postprandial, often small volume, effortless. non-bilious ± fussiness, feed refusal If feeding + growing none indicated. Thicken feeds, position head up after feeds. ± H2 receptor blocker/PPI Pyloric stenosis 2-7 weeks (4M:F), 0.1% Projectile non-bilious vomiting. + hungry, wt loss. + RUQ mass. hypochloraemic alkalosis, low K+ Test feed, US Correct fluid balance, Pyloromyotomy Volvulus 0-3 weeks, Malrotation in 0.5% Recurrent bilious vomiting/acute obstruction Upper GI series, Resect necrotic Surgical bowel, Ladd’s exploration procedure Duodenal atresia 1st week 1 in 5000 (25% Down’s) Scaphoid abdomen, bilious vomiting from birth, obstruction AXR – ‘double bubble’ Surgical Cow’s milk protein allergy <12 months Vomiting, diarrhoea, blood in stool, anaemia ± FTT, ± atopy Trial of dietary elimination Maternal diet / hydrolysed formulas Question 4 A 4 week old girl with Down’s syndrome has nonbilious, non-bloody vomiting occurring shortly after most feeds. She is formula fed, gaining weight, and passing normal stool. What is the most likely diagnosis? A. B. C. D. E. Duodenal atresia Gastro-oesophageal reflux Pyloric stenosis Cow’s milk protein allergy Intestinal volvulus Question 4 A 4 week old girl with Down’s syndrome has nonbilious, non-bloody vomiting occurring shortly after most feeds. She is formula fed, gaining weight, and passing normal stool. What is the most likely diagnosis? A. B. C. D. E. Duodenal atresia Gastro-oesophageal reflux Pyloric stenosis Cow’s milk protein allergy Intestinal volvulus Question 5 An 11 year old boy has had intermittent, severe, periumbilical abdominal pain for four months. It has caused him to miss 10 days of school. He weighs 32kg and his height is on the 50th centile. He reports occasional vomiting, but no weight loss or diarrhoea. Question 5 • Is 32kg an appropriate weight for an 11 year old boy? For a pre-pubertal child Approximate weight = (age + 4) x 2 • An 11 year old should weight (11+4) x 2 = 30kg • A 6 year old should weigh 20kg etc. Question 5 An 11 year old boy has had intermittent, severe, periumbilical abdominal pain for four months. It has caused him to miss 10 days of school. He weighs 32kg and his height is on the 50th centile. He reports occasional vomiting, but no weight loss or diarrhoea. What is the most likely diagnosis? A. B. C. D. E. Functional abdominal pain Coeliac disease Crohn’s disease Intussusception Lactose intolerance Abdominal Pain Disorder Typical Age Features Incidence • Table School of comparison age Peri-umbilical, intermittent for Functional /Recurrent Ix ℞ Education and reassurance, attend school 30% >3/12; + vomiting in 30% ºdiarrhoea, fever, FTT Bloods, Endoscopy if ?coeliac Coeliac disease Esp. 1-3 yrs 1% in UK FTT, lethargy, diarrhoea (large pasty stools). ± vomiting, distension, TTG, total IgA; HLA typing Endoscopy Gluten free diet Crohn’s disease Esp. Adolescence 0.1% FTT, weight loss, anaemia, lethargy, peripheral stigmata Endoscopy + colonoscopy steroids, elemental diet, biologics. ASA, azathioprine. Surgical Lactose intolerance any age 5% in N Europeans Bloating, flatus, and diarrhoea occur 1-3 hrs after dairy consumption Diet trial, H breath test, reducing substances in stool Lactose free diet Intussusception 3/12 – 2 yrs 2M:F. 0.4% Intermittent colicky abdo pain, obstruction, redcurrant jelly stool AXR US Air insufflation Surgical Question 5 An 11 year old boy has had intermittent, severe, periumbilical abdominal pain for four months. It has caused him to miss 10 days of school. He weighs 32kg and his height is on the 50th centile. He reports occasional vomiting, but no weight loss or diarrhoea. What is the most likely diagnosis? A. B. C. D. E. Functional abdominal pain Coeliac disease Crohn’s disease Intussusception Lactose intolerance FTT “insufficient usable nutrition resulting in failure to gain weight appropriately • No universally accepted diagnostic criteria, examples: – Child < 3rd-5th percentile for weight – Fall of 2 percentile lines on growth chart • Consider Height (HT), Weight (WT), Head Circumference (HC) – Type I – WT low, HT + HC relatively preserved • Malnutrition – neglect, poverty, feeding difficulties, anorexia • Malabsorption – Coeliac, Crohn’s, etc. • Increased caloric demand – Cystic fibrosis, CHD, metabolic – Type II – WT + HT low, HC preserved • Dwarfism, endocrine growth abnormalities • Severe malnutrition – Type III – WT, HT + HC low • Prenatal infection, congenital abnormalities • Severe malnutrition • Constitutionally small child • Note these patterns may suggest an aetiology, but are not diagnostic. Any child with FTT requires a full work up FTT • Red flags for organic cause – Abnormal cardio examination – Developmental delay – Dysmorphic features – Recurrent infections – Organomegaly/lymphadenopathy – Vomiting, diarrhoea, dehydration – Adequate caloric intake 4 common causes of organic FTT = the 4 C’s Coeliac Cardiac Cystic fibrosis Cerebral palsy if present require extensive Ix if not evaluate + manage caloric intake Question 6 Emma is a 4 year old who has come in to A&E with abdominal pains and vomiting. She recently had a coryzal illness. Her blood gas shows a metabolic acidosis with partial respiratory compensation. She has glycosuria and ketonuria. You diagnose and treat her for diabetic ketoacidosis (DKA) according to your trust guidelines. Question 6 You have calculated that Emma is 10% dehydrated (she normally weighs 15kg). You treat her with an appropriate insulin infusion and now calculate her fluid replacement. What is the correct fluid replacement regimen for her? A 2500ml over 48 hours B 4000ml over 48 hours C 2750ml over 24 hours D 1250ml over 24 hours E 5250ml over 72 hours Question 6 You have calculated that Emma is 10% dehydrated (she normally weighs 15kg). You treat her with an appropriate insulin infusion and now calculate her fluid replacement. What is the correct fluid replacement regimen for her? A 2500ml over 48 hours B 4000ml over 48 hours C 2750ml over 24 hours D 1250ml over 24 hours E 5250ml over 72 hours Fluid replacement in DKA Replacement should be more cautious than in adults and takes place over 48h. Why? Daily fluid requirement in children: 100mls each kg for first 10kg 50mls each kg for next 10kg 20mls each kg for each additional kg Question 6 You have calculated that Emma is 10% dehydrated (she normally weighs 15kg). You treat her with an appropriate insulin infusion and now calculate her fluid replacement. Daily requirement = 10 x 100 + 5 x 50 = 1250ml Therefore, requirement for 48h = 2500ml Deficit = 10% of 15kg = 1.5kg = 1500ml Total Replacement = 2500ml + 1500ml = 4000ml Managing DKA Protocols vary slightly between centres, find one at your hospital or from the CATS website to learn. Management strategy: 1. Stop ketone production 2. Rehydrate/resuscitate & normalise electrolytes 3. Identify and treat underlying cause Question 7 Amy is a 9 month old who has being crying more than usual over the last 24 hours and has a fever of 38.5. A urine dip is positive for nitrites and leucocytes and she is successfully treated with a short course of trimethoprim. Question 7 What is the most appropriate follow up for this episode? A Reassure Amy’s parents that no further follow-up is necessary B An ultrasound in the next 6 weeks and DMSA scan C An ultrasound in the next 6 weeks and a MCUG (micturating cysturethrogram) D An ultrasound in the next 24 hours E An ultrasound in the next 6 weeks Question 7 What is the most appropriate follow up for this episode? A Reassure Amy’s parents that no further followup is necessary B An ultrasound in the next 6 weeks and DMSA scan C An ultrasound in the next 6 weeks and a MCUG (micturating cysturethrogram) D An ultrasound in the next 24 hours E An ultrasound in the next 6 weeks NICE Guidelines Acute USS USS within 6 weeks DMSA 4-6 months later MCUG If atypical / recurrent All others If atypical / recurrent If atypical/ recurrent 6 months – 3 yrs If atypical If recurrent If atypical / Recurrent X 3 yrs If recurrent If recurrent X 0-6 months If atypical Question 8 A father brings his 3 year old son to the GP’s with worsening fever, diarrhoea, and vomiting. The GP feels a large mass in the left flank which crosses the midline. Urine analysis reveals elevated HVA levels. What is the mass most likely to be? A. B. C. D. E. Wilms’ tumour Spleen Neuroblastoma Intussusception Carcinoid tumour Wilms’ Tumour aniridia Wilms’ tumour, firm smooth grey/tan mass Triphasic histology – blastema, mesenchyme, epithelium Neuroblastoma Large adrenal mass Small round blue cell tumour Distribution of neuroblastoma Abdominal Mass Disorder Typical Age Incidence Cause History Exam and Ix Wilms’ tumour <5 (peak 3-4) 0.1/1000 Malignancy of embryonic renal tissue. ± genetic predisposition (e.g. WAGR) Abdominal mass. Often otherwise asymptomatic Large smooth firm renal mass, doesn’t usually cross midline. Biopsy = triphasic Neuroblastoma <5 (peak 0-2) 0.1-0.2 /1000 Malignancy of Fever, fatigue, wt neural crest cells in loss, diarrhoea, adrenal vomiting medulla/sympatheti c chain. Abdominal mass, can cross midline. +urine HVA/VMA. ±calcified. Biopsy: small blue cell Splenomegaly any Infections, RBC and Hb disorders, lymphoma, leukaemia, SLE LUQ/left sided mass, notch, can’t get above Hepatomegaly any Biliary atresia, viral hepatitis, heart failure RUQ mass, ±pulsatile, ±tender Hepatosplenom egaly any Leukaemia, thalassemia, MPS, liver Question 8 A father brings his 3 year old son to the GP’s with worsening fever, diarrhoea, and vomiting. The GP feels a large mass in the left flank which crosses the midline. Urine analysis reveals elevated HVA levels. What is the mass most likely to be? A. B. C. D. E. Wilms’ tumour Spleen Neuroblastoma Intussusception Carcinoid tumour Question 8 A father brings his 3 year old son to the GP’s with worsening fever, diarrhoea, and vomiting. The GP feels a large mass in the left flank which crosses the midline. Urine analysis reveals elevated HVA levels. What is the mass most likely to be? A. B. C. D. E. Wilms’ tumour Spleen Neuroblastoma Intussusception Carcinoid tumour Question 9 A 3 day old baby girl collapses on the way to A&E, she had become progressively blue and breathless over the last few hours. High flow oxygen has been administered for the past 20 minutes but has had little effect on the PaO2. What is the most likely diagnosis? A. B. C. D. E. Transposition of the great arteries IRDS Ventricular septal defect Patent Ductus Arteriosus Eisenmenger’s syndrome Congenital Cyanotic Heart diseases • Cyanosis is due to a Right-to-Left shunt • 3 causes: 1. 2. 3. Congenital Cyanotic Heart diseases • Cyanosis is due to a Right-to-Left shunt • 3 causes: ToF Pathophysiology 1. Overriding Aorta 2. RV outflow obstruction 3. Large VSD 4. RV hypertrophy Together = venous mixing, reduced pulmonary circulation. DA provides L->R shunt. Presentation Variable RV outflow obstruction = range of severity. Exertional cyanotic spells (tet spells), breathlessness, FTT. CXR – boot shaped heart TGA Eisenmenger’s Transposition of aorta and pulmonary artery. Chronic L->R shunt results in increased pulmonary vascular resistance and pulmonary HTN. Increased right sided pressure reverses shunt, becomes R->L shunt & get cyanosis. Co-exists with VSD, ASD, or PDA. Allows partial pulmonary circulation Severe cyanotic episode often at day 3 of life (when DA closure occurs). L->R shunt causes breathlessness, FTT, chest pain, haemoptysis. Cyanosis onset around 2 years. Question 9 A 3 day old baby girl collapses on the way to A&E, she had become progressively blue and breathless over the last few hours. High flow oxygen has been administered for the past 20 minutes but has had no effect on the PaO2. What is the diagnosis? A. B. C. D. E. Transposition of the great arteries IRDS Ventricular septal defect Patent Ductus Arteriosus Eisenmenger’s syndrome Question 9 5 A 3 day old baby girl collapses on the way to A&E, she had become progressively blue and breathless over the last few hours. High flow oxygen has been administered for the past 20 minutes but has had no effect on the PaO2. What is the diagnosis? A. B. C. D. E. Transposition of the great arteries IRDS Ventricular septal defect Patent Ductus Arteriosus Eisenmenger’s syndrome Congenital Non-Cyanotic Heart diseases • Non-cyanotic heart disease is due to Left-to-Right shunt – ASD • Presentation – asymptomatic, recurrent chest infections, arrhythmia (as adult) • O/E: ESM at L sternal edge – wide S2 • ℞: monitor, if large enough to cause RV dilatation -> surgical correction – VSD • Most common congenital heart disease • Small = <3mm, asymptomatic, most resolve spontaneously. Loud pansystolic murmur at left sternal edge – ℞ await closure – usually at 1-2 years • Large = >3mm, breathlessness, heart failure, FTT, recurrent chest infections. Quiet murmur. – ℞ – surgery at 3-6/12 – PDA • • • • Failure of DA closure 2/12 after expected date of delivery Presentation: usually asymptomatic. O/E: continuous murmur under L clavicle ℞: indomethacin, coil/occlusion device. NB: PGE infusion keeps open, e.g. in TGA. Innocent Murmurs • Present in 30% of children • Resolve spontaneously • Features – Systolic – Soft blowing murmur – Asymptomatic – Left sternal edge No radiation, added sounds, fever, or anaemia • Ddx: ESM (location), PDA (continuous), VSD (harsh, symptoms) Question 10 Mary is a four year old who has been brought into A&E by ambulance. She has been having continuous tonic-clonic seizures for the last 8 minutes. She has been given a dose of buccal midazolam by LAS and her BM is 5.4. Question 10 What is the next most appropriate intervention? A Phenytoin infusion B Buccal midazolam C IV lorazepam D PR paraldehyde E Rapid sequence induction with thiopental Question 10 What is the next most appropriate intervention? A Phenytoin infusion B Buccal midazolam C IV lorazepam D PR paraldehyde E Rapid sequence induction with thiopental Question 10 (Part two) You successfully terminate Mary’s seizure and continue your ABCDE assessment. On exposure, you find the following: Question 10 (Part two) What is the single most likely underlying cause of Mary’s seizure? A Neurofibromatosis type I B Neurofibromatosis type II C Pneumococcal meningitis D Tuberous sclerosis E West syndrome Question 10 (Part two) What is the single most likely underlying cause of Mary’s seizure? A Neurofibromatosis type I B Neurofibromatosis type II C Pneumococcal meningitis D Tuberous sclerosis E West syndrome Tuberous sclerosis Multi system disorder: CNS – Cortical tubers, giant cell astrocytoma & subependymal nodules. Learning disabilities and autistic spectrum disorders. Skin - Multiple manifestations Kidneys – Angiomyolipomas Eyes – Coloboma, papilloedema Heart – Rhabdomyomas Lungs – Cystic replacement of parenchyma Tuberous sclerosis Multi system disorder: Neurofibromatosis type I Sturge-Weber Syndrome Question 11 Sarah is an 11 year old who has developed left sided hip pain and a limp over the last few days. Her BMI is 34, she is afebrile and her observations are stable. What is the single most likely cause of her presentation? A Avascular necrosis B Perthe’s disease C Septic arthritis D Slipped upper femoral epiphysis E Transient synovitis Question 11 8 Sarah is an 11 year old who has developed left sided hip pain and a limp over the last few days. Her BMI is 34, she is afebrile and her observations are stable. What is the single most likely cause of her presentation? A Avascular necrosis B Perthe’s disease C Septic arthritis D Slipped upper femoral epiphysis E Transient synovitis The Limping Child Condition Presentation Minor injury Post-traumatic Septic arthritis/osteomyelitis Fever, excruciating inability to weight bear/to mobilise a limb. Red hot joint Neoplasia Insidious onset, increasing bone pain +/- fractures & weight loss. CNS Present from birth/acute. Other neurological signs (eg: high arched feet) Juvenile Idiopathic Arthritis Oligo/polyarthritis lasting longer than 6 weeks Transient synovitis Aged 3-8, preceding URTI, dx of exclusion DDH Aged 0-4, dx on delivery/baby check. Risk factors: First born, FH, female, breech delivery, macrosomia Perthes Aged 5-9, idiopathic avascular necrosis fem head, intermittent limp & groin/hip/knee pain Rx: Analgesia & bed rest SUFE Aged 10+, Risk factors: Male, Obese, Pubertal, Afro-Caribbean Summary • • • • • • • • • • • Respiratory infections Viral exanthems Neonatal Jaundice Infantile Vomiting Abdominal Pain and FTT DKA Abdominal Mass UTI Congenital heart disease Neurocutaneous syndromes Childhood orthopaedic conditions
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