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Neonatal Jaundice

Key considerations: Kernicterus (bilirubin encephalopathy), Biliary atresia, Hemolytic disease (Rh/ABO incompatibility, G6PD)

Cannot miss

  • Kernicterus (bilirubin encephalopathy)
  • Biliary atresia
  • Hemolytic disease (Rh/ABO incompatibility, G6PD)
  • Sepsis
  • Hypothyroidism
  • Galactosemia

Likely diagnoses

  • Physiologic jaundice
  • Breastfeeding jaundice (inadequate intake)
  • Breast milk jaundice (late onset)
  • Cephalohematoma reabsorption

Red flags

  • Jaundice in first 24 hours of life (ALWAYS pathologic)
  • Total bilirubin rising >5mg/dL per day
  • Direct (conjugated) bilirubin >1mg/dL or >20% of total
  • Lethargy, poor feeding, high-pitched cry (encephalopathy)
  • Total bilirubin approaching exchange transfusion threshold
  • Jaundice persisting >2 weeks (direct bilirubin evaluation needed)

Workup

  • history: Age in hours (critical for plotting on nomogram); Feeding type and adequacy: breastfeeding frequency, wet/stool diapers; Weight loss from birth weight (>7% concerning); Blood type of mother (Rh, ABO incompatibility); Family history: G6PD, hereditary spherocytosis, prior siblings with jaundice; Birth history: bruising, cephalohematoma, vacuum/forceps; exam: Cephalocaudal progression of jaundice (face → trunk → extremities = increasing levels); Hepatomegaly (biliary atresia, metabolic); Splenomegaly (hemolysis); Cephalohematoma or bruising; Neurological: tone, suck, Moro reflex, alertness; Acholic (pale/clay) stools — biliary atresia; labs: Total and DIRECT bilirubin; Blood type and Coombs (direct antiglobulin test); CBC with reticulocyte count and peripheral smear; G6PD level; If direct bilirubin elevated: liver function tests, TSH, urine reducing substances; imaging: Hepatobiliary ultrasound if direct hyperbilirubinemia (biliary atresia); bedside: Transcutaneous bilirubinometer (screening — confirm elevated values with serum)

Management

  • immediate: Plot total bilirubin on Bhutani nomogram using AGE IN HOURS; Start phototherapy based on AAP hour-specific thresholds; If bilirubin at exchange transfusion threshold: start intensive phototherapy AND prepare for exchange; general: Treat underlying cause (hydration for breastfeeding jaundice, etc.); Frequent feeding (q2-3h) to promote enterohepatic circulation; Do NOT interrupt breastfeeding — supplement if needed, but continue nursing; specific: diagnosis: Phototherapy-level jaundice; steps: Intensive phototherapy (blue LED lights); Check bilirubin q4-6h to assess response; Continue feedings during phototherapy; Adequate hydration — may need IV supplementation; Goal: bilirubin decrease of 1-2mg/dL in first 4-6 hours; diagnosis: Exchange transfusion level; steps: This is a MEDICAL EMERGENCY; Intensive phototherapy immediately while preparing for exchange; Double-volume exchange transfusion removes ~85% of circulating bilirubin; NICU admission; Monitor for complications: electrolyte derangements, thrombocytopenia, infection; diagnosis: Direct hyperbilirubinemia; steps: This is NEVER physiologic — always investigate; Biliary atresia: Kasai portoenterostomy must be performed by 60 days of life for best outcomes; Urgent hepatobiliary ultrasound and pediatric surgery/GI consult; Metabolic workup: galactosemia screen, thyroid function

Disposition

  • admit: Bilirubin at or above phototherapy threshold; Rapidly rising bilirubin (>5mg/dL/day); Signs of bilirubin encephalopathy; Exchange transfusion level; Direct hyperbilirubinemia requiring workup; discharge: Bilirubin below phototherapy threshold with close follow-up; Adequate feeding and weight gain documented; Follow-up bilirubin check within 24-48 hours; Clear return precautions given to family; consults: Neonatology for exchange transfusion; Hematology for hemolytic disease; Pediatric GI/surgery if biliary atresia suspected

Clinical pearls

  • ALWAYS plot bilirubin by AGE IN HOURS on the Bhutani nomogram — a "normal" total bili may actually be dangerous depending on the age in hours
  • Jaundice in the first 24 hours is NEVER physiologic — always pathologic. Think hemolysis (ABO/Rh incompatibility, G6PD)
  • Direct (conjugated) hyperbilirubinemia is NEVER normal — always investigate. Biliary atresia needs surgery by 60 days for best outcomes
  • Kernicterus is preventable — the key is identifying at-risk neonates before discharge and ensuring early follow-up

Source and review

  • AAP Clinical Practice Guideline for Hyperbilirubinemia 2022 (revised), Bhutani Nomogram. Last reviewed: 2024-11-01