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Successful plasma therapy in hemolytic uremic syndrome with factor H deficiency.

Abstract
A patient with homozygous factor H deficiency presented with hemolytic uremic syndrome (HUS) at the age of 7 months. After a 2-year period of stability, renal failure and erythrocyte fragmentation recurred between the age of 3 and 4 years. Fresh frozen plasma infusions allowed renal function to be improved and erythrocyte fragmentation to be stopped. Withdrawal of plasma therapy led to a relapse of the biological signs of HUS.
AuthorsS Nathanson, V Frémeaux-Bacchi, G Deschênes
JournalPediatric nephrology (Berlin, Germany) (Pediatr Nephrol) Vol. 16 Issue 7 Pg. 554-6 (Jul 2001) ISSN: 0931-041X [Print] Germany
PMID11465803 (Publication Type: Case Reports, Journal Article)
Chemical References
  • CFH protein, human
  • Haptoglobins
  • Complement Factor H
  • Creatinine
Topics
  • Complement Factor H (deficiency)
  • Creatinine (blood)
  • Haptoglobins (metabolism)
  • Hemolytic-Uremic Syndrome (genetics, therapy)
  • Humans
  • Infant
  • Kidney Function Tests
  • Male
  • Pedigree
  • Plasma

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