Plasma therapy in von Willebrand factor protease deficiency View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2002-08-16

AUTHORS

Georges Deschênes, Agnès Veyradier, Sylvie Cloarec, Stéphane Benoit, Isabelle Desbois, Yves Gruel, Hubert Nivet

ABSTRACT

. We report a patient with relapsing hereditary hemolytic uremic syndrome (HUS) that began in the neonatal period with life-threatening jaundice and hemolytic anemia. He progressed to end-stage renal failure at 14 years of age and had a cerebrovascular accident while on dialysis. The cause of HUS was a constitutional deficiency in the von Willebrand factor cleaving protease. Hematological features of HUS significantly improved following bilateral nephrectomy. After renal transplantation, he had an early recurrence of HUS associated with two episodes of retinal and cerebral ischemia. Long-term treatment with fresh-frozen plasma exchanges prevented recurrence of HUS, cerebrovascular attacks, and early loss of the graft. More... »

PAGES

867-870

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00467-002-0944-9

DOI

http://dx.doi.org/10.1007/s00467-002-0944-9

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1004145949

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/12376819


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