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The challenge of managing hemophilia A and STEC-induced hemolytic uremic syndrome

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Abstract

Background

The hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy leading to acute kidney injury in children. In most cases it is triggered by an infection caused by Shiga-like toxin-producing Escherichia coli (STEC). Endothelial damage plays a central role in the pathogenesis of disease. Hemophilia A is a genetic disorder leading to factor VIII (FVIII) deficiency, an important factor in the coagulation system.

Case

Here we describe a hemophilia A patient who developed HUS due to a STEC O26 infection. The patient developed not only acute kidney injury, but also severe gastro-intestinal and neurological complications. Increased amounts of recombinant FVIII (rFVIII) had to be administered during the acute phase of the disease to reach acceptable blood levels of FVIII, in order to control the hemorrhagic colitis and to prevent severe neurological complications.

Conclusion

The patient’s treatment schedule of rFVIII during the HUS period was a serious challenge, and we cannot exclude that it contributed to the severity of the HUS by enhancing the thrombotic microangiopathic process. The role of factor VIII administration in the severe outcome of this disease is discussed.

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Acknowledgments

This work was partially supported by the Dutch Kidney Foundation (C09.2313 to DW).

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Authors and Affiliations

  1. Department of Pediatric Nephrology, Radboud University Nijmegen Medical Centre, P.O. Box 9101, 6500 HB, Nijmegen, the Netherlands

    Dineke Westra, Lambert P. W. J. van den Heuvel & Nicole C. A. J. van de Kar

  2. Department of Pediatric Hematology-Oncology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands

    Paul P. T. Brons

  3. Department of Pediatric Nephrology, Erasmus MC–Sophia Children’s Hospital, Rotterdam, The Netherlands

    Eiske M. Dorresteijn

  4. Department of Pediatric Oncology-Hematology, Erasmus MC–Sophia Children’s Hospital, Rotterdam, The Netherlands

    Auke Beishuizen

Authors
  1. Dineke Westra
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  2. Eiske M. Dorresteijn
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  3. Auke Beishuizen
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  4. Lambert P. W. J. van den Heuvel
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  5. Paul P. T. Brons
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  6. Nicole C. A. J. van de Kar
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Corresponding author

Correspondence to Nicole C. A. J. van de Kar.

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Supplementary Table 1

Laboratory evaluation at different time points. (DOC 42 kb)

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Westra, D., Dorresteijn, E.M., Beishuizen, A. et al. The challenge of managing hemophilia A and STEC-induced hemolytic uremic syndrome. Pediatr Nephrol 28, 349–352 (2013). https://doi.org/10.1007/s00467-012-2312-8

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  • DOI: https://doi.org/10.1007/s00467-012-2312-8

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