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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Biliary atresia and prolonged neonatal jaundice

Paediatrics and child health · UK scope · Sources checked 27 Aug 2026 · Clinical review pending

RapidFull
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Cholestasis with acute deterioration

Bleeding, sepsis, hypoglycaemia, encephalopathy, tense ascites or cardiovascular instability in a jaundiced infant indicates urgent hepatic or systemic decompensation.

Action: Use ABCDE, check glucose and coagulation, give parenteral vitamin K when deficiency bleeding is possible under paediatric guidance and involve a specialist paediatric liver centre urgently.

Synopsis

Distinguish unconjugated transitional jaundice from cholestasis, identify pale stool and dark urine early and accelerate specialist investigation of biliary atresia while protecting nutrition and fat-soluble vitamins.

  • Prolonged jaundice means more than 14 days at 37 weeks gestation or above, and more than 21 days below 37 weeks.
  • Ask parents to describe or show stool and urine directly; pale chalky stool and dark staining urine are the highest-yield cholestasis clues.
  • First-line tests under NICE are conjugated bilirubin, FBC, maternal and infant blood groups and DAT, with urine culture when UTI is suspected and confirmation of newborn metabolic screening.
  • Conjugated bilirubin above 25 micromol/L needs expert advice because it may indicate serious liver disease.
  • Do not stop breastfeeding merely to test breast-milk jaundice and do not use visual bilirubin assessment alone.
  • Suspected biliary atresia needs same-day discussion with paediatric gastroenterology or a specialist liver centre; early investigation preserves the chance of effective Kasai surgery.
  • Specialist evaluation uses liver tests, coagulation, glucose, infection and metabolic studies, expert ultrasound and sometimes biopsy or operative cholangiography.
  • Kasai portoenterostomy removes fibrotic extrahepatic remnants and drains bile through a jejunal loop; earlier surgery generally offers better native-liver drainage.
  • Cholestatic infants need high-energy feeding, specialist fat formulation and measured supplementation of vitamins A, D, E and K, with ongoing monitoring.

Key red flags

Jaundice lasting beyond 14 days in a term baby or 21 days in a preterm baby requires prolonged-jaundice assessment.

Pale chalky or white stool and dark urine that stains the nappy indicates conjugated cholestasis until proved otherwise.

Any conjugated bilirubin above 25 micromol/L warrants expert advice under NICE, regardless of total bilirubin appearance.

Poor feeding, faltering growth, hepatosplenomegaly, bleeding or bruising strengthens concern for significant liver or metabolic disease.

Fever, lethargy, irritability or reduced urine can indicate UTI or sepsis causing or accompanying jaundice.

Do not reassure from breastfeeding or a well appearance when stool is pale; biliary atresia can initially coexist with apparently good health.

Investigation priorities

01
First-line fractionated serum bilirubinFirst stepFirst line

Distinguish conjugated cholestasis from predominantly unconjugated prolonged jaundice.

Management branches

ScreenFractionate prolonged jaundice

Jaundice persists beyond the gestation-specific NICE threshold.

  1. Inspect stool and urine and examine growth, liver, spleen and systemic state.
  2. Measure conjugated bilirubin, FBC, groups and DAT, assess UTI and confirm metabolic screening.
  3. Seek expert advice for direct bilirubin above twenty-five or immediately for pale stool or clinical deterioration.
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Sources and review status4 sources · checked 27 Aug 2026 · clinical review pending+
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Apply principles in context and verify current guidance when a decision affects care. Source check completed 27 Aug 2026; clinical approval remains outstanding.

  • NICE CG98 Neonatal Jaundice RecommendationsProlonged-jaundice thresholds, direct bilirubin and initial investigations.
  • NHS Newborn Jaundice OverviewRecognition, urgent symptoms and parent advice.
  • CLDF Biliary Atresia InformationPale stool, specialist diagnosis, Kasai surgery and transplantation.
  • Children Liver Disease Foundation Yellow AlertUK early recognition resources for prolonged jaundice and stool colour.

Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom