Successful plasma therapy in hemolytic uremic syndrome with factor H deficiency

Pediatr Nephrol. 2001 Jul;16(7):554-6. doi: 10.1007/s004670100609.

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.

Publication types

  • Case Reports

MeSH terms

  • Complement Factor H / deficiency*
  • Creatinine / blood
  • Haptoglobins / metabolism
  • Hemolytic-Uremic Syndrome / genetics
  • Hemolytic-Uremic Syndrome / therapy*
  • Humans
  • Infant
  • Kidney Function Tests
  • Male
  • Pedigree
  • Plasma*

Substances

  • Haptoglobins
  • complement factor H, human
  • Complement Factor H
  • Creatinine