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Haemophilia A in two premature infants

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Abstract

The incidence of prematurity in Germany is about 10% and premature infants with haemophilia A (OMIM 306700) are in fact very rare. We report two new cases, one born in the 28th gestational week, weighing 1200 g with a factor VIII (FVIII) level of 0.03 IU/ml treated with bolus injections of plasma derived FVIII concentrate (pdFVIII), and one born at week 30, weighing only 710 g with a factor level of <0.01 IU/ml and treated with recombinant FVIII concentrate (rFVIII). Recovery of FVIII was 96% in case 1 and 120% in case 2, FVIII half-life was 6 h and 8 h, respectively. During FVIII substitution, neither bleeding, thrombosis nor inhibitor development were noted in both infants. Conclusion:Immediate and frequent factor VIII substitution appears to be safe and effective for prophylaxis and treatment in premature haemophilic neonates.

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Abbreviations

FVIII :

factor VIII

IVH :

intraventricular haemorrhage

pdFVIII :

plasma derived factor VIII concentrate

rFVIII :

recombinant factor VIII concentrate

vWF :

von Willebrand factor

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Acknowledgements

The authors wish to thank all involved clinicians, especially Dr. Garhammer and her team of the Neonatal Intensive Care Unit, Children’s Hospital Dritter Orden, Munich, Germany, and the neonatologists of the Allgemeines Krankenhaus Celle, Germany and the Children’s Hospital, Medical School Hannover, Germany.

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Authors and Affiliations

  1. Department of Paediatric Haemostaseology, Dr. von Hauner’s Children’s Hospital, University of Munich, Lindwurmstrasse 4, 80337 , Munich, Germany

    Christoph Bidlingmaier & Karin Kurnik

  2. Labor Wagner Stibbe, Hannover, Germany

    Frauke Bergmann

Authors
  1. Christoph Bidlingmaier
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  2. Frauke Bergmann
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  3. Karin Kurnik
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Correspondence to Christoph Bidlingmaier.

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Bidlingmaier, C., Bergmann, F. & Kurnik, K. Haemophilia A in two premature infants. Eur J Pediatr 164, 70–72 (2005). https://doi.org/10.1007/s00431-004-1542-6

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  • DOI: https://doi.org/10.1007/s00431-004-1542-6

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