ORIGINAL ARTICLES

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

P M van Hasselt¹, K Kok², A D M Vorselaars¹, L van Vlerken¹, E Nieuwenhuys⁴, T J de Koning¹, R A de Vries³, R H J Houwen¹

¹ Department of Pediatrics, Wilhelmina Children’s Hospital, University Medical Center Utrecht, The Netherlands
² Department of Medicine, Division of Gastroenterology and Hepatology, Radboud University Medical Center Nijmegen, The Netherlands
³ Department of Internal Medicine, University Medical Center Groningen, Groningen, The Netherlands
⁴ Department of Immunochemistry, Sanquin Diagnostics, Amsterdam, The Netherlands

^{Correspondence to} Dr P M van Hasselt, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Lundlaan 6, 3584EA, Utrecht, The Netherlands; p.vanhasselt{at}umcutrecht.nl

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.

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