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Vitamin K deficiency bleeding in cholestatic infants with alpha-1-antitrypsin deficiency

P M van Hasselt1, K Kok, A D M Vorselaars

  • 1Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Lundlaan 6, 3584EA Utrecht, The Netherlands. p.vanhasselt@umcutrecht.nl

Archives of Disease in Childhood. Fetal and Neonatal Edition
|May 6, 2009
PubMed

Insights

Exclusively breastfed infants with alpha-1-antitrypsin deficiency (A1AD) and cholestatic jaundice face a high risk of vitamin K deficiency (VKD) bleeding. This risk is uniform and does not depend on the degree of cholestasis.

Area of Science:

  • Neonatology
  • Pediatric Gastroenterology
  • Hepatology

Background:

  • Exclusively breastfed infants with undiagnosed cholestatic jaundice are susceptible to vitamin K deficiency (VKD) bleeding.
  • The influence of cholestasis severity on VKD bleeding risk in these infants remains unclear.
  • Alpha-1-antitrypsin deficiency (A1AD) causes variable cholestasis, making it a suitable model to study this risk.

Purpose of the Study:

  • To assess the risk of VKD bleeding in infants diagnosed with cholestatic jaundice due to A1AD.
  • To determine if the degree of cholestasis, indicated by conjugated bilirubin levels, correlates with VKD bleeding risk.

Main Methods:

  • Study included infants with A1AD (ZZ or SZ phenotype) born in the Netherlands (1991-2006).
  • Data on VKD bleeding were collected for breastfed and formula-fed infants.
  • Serum conjugated bilirubin levels were analyzed for correlation with bleeding risk.

Main Results:

  • Fifteen of 20 (75%) breastfed infants with A1AD experienced VKD bleeding, versus 0 of 20 formula-fed infants.
  • The relative risk of VKD bleeding in breastfed versus formula-fed infants was at least 15.8.
  • Conjugated bilirubin levels at diagnosis did not correlate with the risk of VKD bleeding.

Conclusions:

  • Breastfed infants with A1AD face a high risk of VKD bleeding, irrespective of cholestasis severity.
  • This risk is comparable to that observed in breastfed infants with biliary atresia.
  • Adequate vitamin K prophylaxis is crucial for exclusively breastfed infants with cholestatic jaundice, regardless of the underlying cause.
Abstract

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