Successful plasma therapy in hemolytic uremic syndrome with factor H deficiency.
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.
Nathanson
S
S
Service de Néphrologie Pédiatrique, Hĵpital Armand-Trousseau, Paris, France.
Frémeaux-Bacchi
V
V
Deschênes
G
G
D017242:Complement Factor H / Q000172:deficiency*; D003404:Creatinine / Q000097:blood; D006242:Haptoglobins / Q000378:metabolism; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics / Q000628:therapy*; D006801:Humans; D007223:Infant; D007677:Kidney Function Tests; D008297:Male; D010375:Pedigree; D010949:Plasma
C493495:CFH protein, human; D006242:Haptoglobins; D017242:Complement Factor H; D003404:Creatinine