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Vitamin K deficiency bleeding in cholestatic infants with Alpha-1-Antitrypsin deficiency
  1. Peter M van Hasselt (p.vanhasselt{at}umcutrecht.nl)
  1. Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Netherlands
    1. Karin Kok
    1. Division of Gastroenterology and Hepatology, Radboud University Medical Center Nijmegen, Netherlands
      1. Adriane D M Vorselaars
      1. Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Netherlands
        1. Lotte van Vlerken
        1. Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Netherlands
          1. Ed Nieuwenhuys
          1. Department of Immunochemistry, Sanquin Diagnostics, Amsterdam, Netherlands
            1. Tom J de Koning
            1. Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Netherlands
              1. Richard A de Vries
              1. Department of Gastroenterology and Hepatology, Rijnstate Hospital, Arnhem, Netherlands
                1. Roderick H J Houwen, MD PhD
                1. Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Netherlands

                  Abstract

                  Objective: Exclusively breast-fed infants with unrecognized 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 (A1ATD) induces a variable degree of cholestasis, we assessed the risk of VKD bleeding in infants with cholestatic jaundice due to A1ATD.

                  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 5 Dutch diagnostic centres for alpha-1-antitrypsin phenotyping and/or genotyping. We determined the risk of VKD bleeding upon diagnosis of A1ATD 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 A1ATD were studied. VKD bleeding was noted in 15/20 (75%) of breast-fed infants, compared with 0/20 of formula-fed infants with A1ATD. The relative risk for VKD bleeding in breast-fed versus formula-fed infants was at least 15.8 (95% confidence interval 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 breast-fed infants with A1ATD was high and did not correlate with serum level of conjugated bilirubin at diagnosis. A similar absolute risk was previously reported in breast-fed 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 breast-fed infants with cholestatic jaundice, irrespective of underlying etiology.

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