RT Journal Article SR Electronic T1 Vitamin K deficiency bleeding in cholestatic infants with alpha-1-antitrypsin deficiency JF Archives of Disease in Childhood - Fetal and Neonatal Edition JO Arch Dis Child Fetal Neonatal Ed FD BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health SP F456 OP F460 DO 10.1136/adc.2008.148239 VO 94 IS 6 A1 P M van Hasselt A1 K Kok A1 A D M Vorselaars A1 L van Vlerken A1 E Nieuwenhuys A1 T J de Koning A1 R A de Vries A1 R H J Houwen YR 2009 UL http://fn.bmj.com/content/94/6/F456.abstract AB Objective: Exclusively breastfed infants with unrecognised cholestatic jaundice are at high risk of a vitamin K deficiency (VKD) bleeding. It is presently unknown whether (the size of) this risk depends on the degree of cholestasis. Since alpha-1-antitrypsin deficiency (A1AD) induces a variable degree of cholestasis, we assessed the risk of VKD bleeding in infants with cholestatic jaundice due to A1AD.Patients and methods: Infants with a ZZ or SZ phenotype born in The Netherlands between January 1991 and December 2006 were identified from the databases of the five Dutch diagnostic centres for alpha-1-antitrypsin phenotyping and/or genotyping. We determined the risk of VKD bleeding upon diagnosis of A1AD in breastfed and formula fed infants and searched for correlations between serum levels of conjugated bilirubin and the risk of bleeding.Results: A total of 40 infants with A1AD were studied. VKD bleeding was noted in 15/20 (75%) of breastfed infants, compared with 0/20 of formula fed infants with A1AD. The relative risk for VKD bleeding in breastfed versus formula fed infants was at least 15.8 (95% CI 2.3 to 108). Conjugated bilirubin levels at diagnosis did not correlate with the risk of VKD bleeding.Conclusions: The risk of VKD bleeding in breastfed infants with A1AD was high and did not correlate with serum level of conjugated bilirubin at diagnosis. A similar absolute risk was previously reported in breastfed infants with biliary atresia under the same prophylactic regimen. This confirms that—without adequate prophylaxis—the risk of VKD bleeding is uniformly high in exclusively breastfed infants with cholestatic jaundice, irrespective of underlying aetiology.