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Prise en charge néphrologique des patients hémophiles A : difficultés diagnostiques et thérapeutiques illustrées par le cas de 2 patients

Authors
  • Fedi, Mathilde
  • Falaise, Céline
  • Lanot, Antoine
  • von Kotze, Clarissa
  • Robert, Thomas
  • Piétri, Léa
  • Henri, Patrick
  • Delmotte, Nicolas
  • Botta, Danielle
  • Verhelst, David
  • Chambost, Hervé
  • Brunet, Philippe
  • Jourde-Chiche, Noemie
Publication Date
Jan 01, 2019
Identifiers
DOI: 10.1016/j.nephro.2018.10.002
PMID: 30660587
OAI: oai:HAL:hal-02623209v1
Source
HAL-Descartes
Keywords
Language
English
License
Unknown
External links

Abstract

Hemophilia A is an X-linked genetic hemorrhagic disorder characterized by a factor VIII deficiency. The availability of secured substitution products has led to a dramatic improvement of life expectancy in hemophiliac patients. Nowadays, adult hemophiliac patients may develop Chronic Kidney Disease (CKD) resulting from age-related comorbidities (hypertension, obesity, diabetes). In addition, the high prevalence of viral infections in this population exposes patients to an increased risk of CKD. The risk of hemorrhage in hemophiliac patients is a challenge for their clinical management, both for diagnostic procedures (kidney biopsy in particular) and for renal replacement therapy (dialysis or renal transplantation) when it is needed. This work provides an update of the literature data concerning the management of hemophiliac patients in nephrology, illustrated by the cases of two patients.

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