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Paeds Vivasfetal-neonatal-and-perinatal

Paeds Vivas · fetal-neonatal-and-perinatal

Conjugated jaundice and neonatal cholestasis: Viva

Branching clinical structured oral on conjugated jaundice and neonatal cholestasis: when to measure a split bilirubin, the biliary atresia exclusion pathway, the Kasai operation, and the role of transplantation.

branching clinical structured oral2 min readVerification in progress

Target exams

RACP DWERACP DCEMRCPCH Clinical
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Target exams

RACP DWERACP DCEMRCPCH Clinical
Prompt
A registrar phones you about a four-week-old breastfed term infant who is still jaundiced. The registrar plans to reassure the parents that this is breast-milk jaundice and review in two weeks. The examiner asks how you respond.

Branch 1: The decision to investigate

The candidate should immediately identify that reassurance without a conjugated bilirubin is unsafe. Jaundice persisting beyond two weeks in a term infant mandates a split bilirubin measurement. Breast-milk jaundice is a diagnosis of unconjugated hyperbilirubinaemia only and cannot be invoked until a conjugated fraction has been measured and found normal. The candidate must ask about stool and urine colour directly, because acholic stools or dark urine would make cholestasis and biliary atresia the leading concern. [1]

The conjugated fraction threshold is a conjugated bilirubin above 17 micromol per litre or more than 20 per cent of the total bilirubin. A result in this range confirms cholestasis, which is always pathological and triggers the full diagnostic panel. The candidate should explain that the single most avoidable error in neonatal cholestasis is reassurance without a split bilirubin, because it may delay a Kasai operation into the range where it cannot succeed. [1]

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References3ShowHide
  1. [1]Fawaz R, Baumann U, Ekong U, Fischler B, Hadzic N, Mack CL, McLin VA, Molleston JP, Neimark E, Ng VL, Karpen SJ Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr, 2017.PMID 27429428
  2. [2]Serinet MO, Wildhaber BE, Broué P, et al. Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics, 2009.PMID 19403492
  3. [3]Bezerra JA, Spino C, Magee JC, Shneider BL, Rosenthal P, Wang KS, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA, 2014.PMID 24794368
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