Pediatrics Review

1. Pediatrics Review Gina Neto, MD FRCPC Pediatric Emergency Medicine

2. Objectives Pediatric Emergency in 45 min! Recognize and manage acute pediatric presentations from newborn to older children Describe evidence based management of selected pediatric conditions

3. Case • 4 day old girl presents to the ED with jaundice. • Today is sleepy and has to be woken for feeds. • Born at term, SVD. No complications. BW 3.5 kg. • T 37.2, HR 140, RR 36, BP 90/50. Wt3.1 kg • No distress. Sleeping but arouses easily. • Skin jaundiced, sclera icteric.

4. Case • Further history • Baby is breastfeeding every 3 hrs but falling asleep, stays on breast for 30 min. • No urine output since last evening. • GBS neg, SROM 1 hr before delivery. • Asian ethnicity. Mom is O pos.

5. Case • Assessment? • Initial management? • Jaundice is a medical emergency! • Start phototherapy immediately • > 10% wt loss– dehydration vs poor weight gain • IV fluids • Sleepy vs Lethargic • ? Needs septic work up

6. Neonatal Jaundice • Most common medical presentation in 1st week of life • Unconjugated vs Conjugated • Physiologic jaundice • 3rd day of life •  RBC mass, Immature liver conjugation, Increased enterohepatic circulation • Poor feeding and dehydration • “Not Enough Breastfeeding Jaundice” • Increased enterohepatic circulation, Decreased bilirubin clearance

7. Neonatal Jaundice • Breast milk jaundice • Starts Day 5-7, Well baby • May last for several weeks • ? Component in milk that inhibits conjugation • Blood destruction • Immune (Hemolysis) • ABO incompatibility, Rh Disease • Non-Immune • Blood Disorders (G6PD, Spherocytosis) • Hematoma, Polycythemia • Sepsis • Other (Gilbert, Crigler-Najjar, Hypothyroidism)

8. Neonatal Jaundice • Conjugated hyperbilirubinemia is always pathologic • Liver Disease • Biliary obstruction (atresia, choledochal cyst) • Hepatitis • Sepsis • STORCH infection • Syphilis, Toxoplasmosis, Rubella, CMV, HSV, Hep B • Metabolic disorders • Galactosemia, Tyrosinemia

9. Neonatal Jaundice • Bilirubin Induced Encephalopathy • Basal ganglia involvement • Early • High-pitched cry, lethargy, hypotonia • Late • Hypertonia, extensor rigidity, seizures, coma, death • Long term • Athetoid cerebral palsy, deafness

10. Neonatal Jaundice • Labs • Bilirubin – total and conjugated  STAT ! • CBC, Blood group, Direct antibody test (Coombs)     • Consider Septic Workup, Lytes, BUN, Cr, Glu, VBG • Start phototherapy immediately • Converts unconjugated bilirubin into water soluble isomers • If treatment needed: • Consider IV hydration, Keep baby warm • Catheter Urine (~8% will have a UTI)

11. Neonatal Jaundice Remember to also plot on Exchange Transfusion Graph if in treatment range

12. Case • 5 day old boy presents with vomiting and lethargy • Over the past 24 hrs the baby has become increasingly sleepy and difficult to feed. • Vomited several times. • No wet diapers since last night. • Born at term. SVD. No complications. BW 3.5 kg

13. Case • T 35.2oC, HR 200, RR 60, BP 90/50. • O2 sat 96% • Lethargic, mottled infant • Chest - clear, mild intercostal indrawing • CV - normal HS, cap refill 5 sec • Abd - slightly distended

14. Case • What is the physiologic status of the infant? • Shock • Compensated or decompensated? • Initial management? • ABC’s • O2 • IV fluids • Check glucose • Warm the baby!!

15. Septic Appearing Newborn “THE MISFITS” (Causes of Shock in the Newborn) • Trauma • Non-accidental • Heart • Duct dependent lesions • Coarctation of aorta, Hypoplastic left heart, Aortic stenosis • Arrythmias – SVT • Endocrine • Congenital Adrenal Hyperplasia • Thyrotoxicosis

16. Septic Appearing Newborn “THE MISFITS” (Causes of Shock in the Newborn) • Metabolic - Hypoglycemia, Hyponatremia • Inborn errors of metabolism • Sepsis • Formulaerrors • Intestinal catastrophes - Volvulus, Necrotizing Enterocolitis • Toxins • Seizures

17. Sepsis • Risk factors • Prematurity • PROM >18 hrs • Fever in mother or infant at delivery • Multiple births • Previous sibling with GBS infection • Pathogens • Group B Strep 30%, E. coli 30-40%, Other Gram neg 15-20% • Gram pos (including Listeria monocytogenes) 10% • Viral (HSV, HZV, RSV, Coxsackie), Chlamydia

18. Sepsis • ABC’s, Fluid Resuscitation, Glucose • “Septic work up” • CBC, Blood C&S • Cath urine R&M, C&S • LP - if stable • CXR - if resp sx's • Viral serology • Treatment • Ampicillin & • Gentamycin or Cefotaxime • If suspect herpes – Add Acyclovir

19. Congenital Adrenal Hyperplasia • Most common is 21 hydroxylase deficiency (95%) • Aldosterone and cortisol deficiency • Excess production of testosterone • Girls - Ambiguous genitalia • Boys - Adrenal crisis/shock at ~1 week • Management: • Correct fluid and electrolyte imbalances • Low Glu, Low Na, High K • Hydrocortisone • Fludrocortisone

20. Cardiogenic Shock • Acyanotic duct dependent lesions • Critical left heart obstruction • Poor systemic blood flow • Acidosis and shock • Hypoplastic left heart syndrome • Coarctation of the aorta • Aortic stenosis • Total anomalous pulmonary venous return

21. PDA Closure • Increased O2 sat with first breath  contraction of ductus arteriosus • physiologic closure at ~12 hrs • anatomic closure at 2-3 wks • Duct dependent lesions • No pulmonary blood flow  cyanosis • No systemic blood flow  shock

22. Cardiogenic Shock • Acyanotic duct dependent lesions • ABC’s • Fluid resuscitation • Consider Intubation • Maintain relative hypoxia and hypercarbia • Start Prostaglandin E1 • Infusion at 0.05-0.1 g/kg/min • Complications: • Apnea in ~15%, Hypotension, Fever, Seizures • Improvement within 15-30 min

23. Case • 3 day old girl brought to the ED with a history of progressive cyanosis and irritablity • T 36.3oC, HR 160, RR 48, BP 94/40. O2 sat 74% • Irritable, cyanotic infant • Chest clear. S1 S2, soft systolic murmur, normal pulses, cap refill 3 sec • What is your approach to this infant? • Differential diagnosis?

24. Cyanotic Newborn Cyanotic heart lesions • Decreased pulmonary blood flow • Tetralogy of Fallot (TOF) • Pulmonary atresia or stenosis • Desaturated blood shunted to systemic circulation • Transposition of great vessels (TGA) • Truncus arteriosus • Tricuspid atresia • Total anomalous pulmonary venous return (TAPVR)

25. Cyanotic Newborn • Non Cardiac Causes • Upper airway obstruction • Choanal atresia • Pulmonary disease • Pneumonia • Diaphragmatic hernia • Persistent pulmonary hypertension • Neurologic • Hemorrhage, hydrocephalus, infection • Neuromuscular disorders • Polycythemia • Methemoglobinemia

26. Cyanotic Newborn • Management • ABC’s, Glucose • 100% oxygen test • Poor response to oxygen suggests cyanotic cardiac lesion • Use only diagnostically  O2 promotes closure of PDA • Maintain relative hypercarbia and hypoxia • pCO2 45-50, O2 sat <90% • Start Prostaglandin E1 • Infusion at 0.05-0.1 g/kg/min

27. Case 1 month old boy with 2 day history of irritability and poor feeding. Difficulty breathing today. No fever or URTI sx’s. Born at term, healthy. T 37.5oC, HR 160, RR 80, BP 85/50. O2 sat 92% Mild respiratory distress, indrawing, bilateral crackles S1 S2, III/VI systolic murmur, normal pulses Liver at 5 cm below CM

28. Case • What is the most likely diagnosis?

29. Congestive Heart Failure • Left to Right shunts • Presentation at 1 month • Decreasing pulmonary vascular resistance 1st month of life • Increased blood flow into lungs • Symptoms • Irritability, Diaphoresis • Poor feeding (early fatigue), Failure to thrive • Signs • Tachypnea, Tachycardia, Respiratory distress • Enlarged liver

30. Congestive Heart Failure • VSD most common • Other: ASD, PDA • Diagnosis • Pansystolic Murmur, Hyperactive precordium • ECG – LVH • CXR – cardiomegaly, vascular redistribution • ED Management • ABC’s, Glucose • Furosemide • CPAP

31. Congenital Heart Disease - Age of presentation

32. Case • 3 week old boy vomiting every feed for 24 hours. • Vomit is yellow. No diarrhea. Dry diaper since this morning. • HR 180, RR 40, T 37.2 R. Irritable and restless. • Eyes sunken. Mouth dry. Cap refill 5 sec. • Abd distended and diffusely tender. • What is your approach to this infant? • Differential diagnosis?

33. Volvulus • 40% present in first week, 80% present by 1 month • Malrotation • Short small bowel mesentery, ligament of Treitz poorly fixed • Twisting of the bowel around the superior mesenteric artery • Sudden onset of bilious vomiting • Acute abdomen with shock • Bowel ischemia and necrosis, GI bleeding • ABC’s, Fluid resuscitation, Glucose • Upper GI series • Emergent surgery

34. Pyloric Stenosis • 4-6 weeks of age • Male to female 4:1, first born males • 5% of siblings and 25% if mother was affected • Symptoms of gastric outlet obstruction • Non-bilious vomiting • Emesis increases in frequency and eventually becomes projectile • Peristaltic wave, palpable mass in epigastrium “olive” • Labs – low K, low Cl, metabolic alkalosis • Ultrasound

35. Case 1 yr boy with vomiting and diarrhea since last night. This morning he had three loose stools with blood. He cries intermittently in cycles of 10 to 20 minutes. T36.5, HR 118, RR 40, BP 100/50. Pale and lethargic. Abd soft, mild tenderness. Mass palpable in RLQ. Investigations? Diff Dx?

36. Intussusception • Usually invagination of ileum into cecum (75%) • 6 months to 3 yrs • Males to female 3:2 • 90% are idiopathic • Post viral illness – hypertrophy of Peyer patches • Pathologic causes - Meckel diverticulum, polyps, hematoma (Henoch-Schonlein Purpura), lymphoma/leukemia, cystic fibrosis

37. Intussusception • Classic triad present in 10-30% • Intermittent, crampy abdominalpain • Vomiting • “Currant jelly" stools • Late sign, indicates intestinal edema and mucosal bleeding • Lethargy in 25% • Ultrasound (Sens 97-100%, Spec 88-100%) • AXR (Sens 45%, Spec 21%) • Lack of air in RLQ, obstruction • Target sign, Crescent sign

38. Intussusception • Target sign

39. Intussusception • Crescent Sign

40. Intussusception • Air Contrast Enema • Success rate 95% • Bowel perforation in 1-3% • Recurrence rate 10-15% • 50% within first 24 hrs • Other 50% within 10 mos

41. Case • 4 yr old with bruising to both legs today • Pain with walking, swollen ankles. • Abdominal pain with blood in stool. • Diagnosis? • Complications?

42. Henoch-Schonlein Purpura • IGA mediated vasculitis • 2-11 yrs • Rash 100% • Palpable petechiae/purpura, can be urticarial • Arthritis 70% • Ankles > knees >wrists > elbows • Abdominal pain 50% • Intussusception 2% • Nephritis 40% (ESRD in ~1%)

43. Henoch-Schonlein Purpura • Investigations • CBC, PT PTT, Lytes, BUN, CR; Urinalysis • Prot, Alb, Immunoglobulins • Strep testing – Throat swab, ASOT • Weekly U/A and BP until sxs resolve then monthly for 6 mos • Treatment • NSAID’s for pain relief • Consider steroids for abdominal, testicular, CNS involvement • Controversial for renal complications • Nephrology consult if hypertension, nephrotic sx’s

44. Case • 2 yr old boy with fever for 6 days. • Red eyes but no discharge. • Generalized rash. • Erythema of the palms of hands and soles of feet. • Red, swollen lips. • Enlarged cervical lymph nodes. • Diagnosis? • Complications?

45. Kawasaki Disease • Usually < 4 yrs old • peak 1-2 yrs • Fever for > 5 days and 4 of: • Bilateral non-purulent conjunctivitis • Rash • Changes of peripheral extremities • Initial stage: reddened palms and soles • Convalescent stage: desquamation of fingertips and toes • Changes of lips and oral cavity • Cervical lymphadenopathy ( >1.5 cm)

46. Kawasaki Disease • Subacute phase - Days 11-21 • Desquamation of extremities • Arthritis • Convalescent phase - > Day 21 • If untreated ~ 25% coronary artery aneurysms • Other manifestations: • Uveitis, Pericarditis, Myocarditis • Hepatitis, Gallbladder hydrops • Aseptic meningitis

47. Kawasaki Disease Incomplete (Atypical) • Fever >5 d with 2-3 criteria AAP Kawasaki statement Newburger et al. Pediatrics, 2004

48. Kawasaki Disease Supplemental Lab Criteria ESR >40 CRP >3 WBC > 15 000/mm Anemia Platelets after 7 days > 450 Elevation of ALT Albumin < 3 Urine >10 WBC/hpf

49. Kawasaki Disease Treatment • IV Immunoglobulin (2 g/kg) • Reduces coronary aneurysms to 3% if given within 10 days of onset of illness • Defervescence with 48 hrs • ASA • During acute phase high dose (80-100 mg/kg/day) then low dose (3-5 mg/kg/day) for 6-8 weeks • Stop if normal ECHO

50. Case 4 month old with difficulty breathing x 2 days. Cough and congestion. Poor Feeding. Moderate distress. T 37. RR 80. HR 160. Sat 94% Crackles and wheezes bilaterally. Indrawing. Management?