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.
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Authors | S Nathanson, V Frémeaux-Bacchi, G Deschênes |
Journal | Pediatric nephrology (Berlin, Germany)
(Pediatr Nephrol)
Vol. 16
Issue 7
Pg. 554-6
(Jul 2001)
ISSN: 0931-041X [Print] Germany |
PMID | 11465803
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- CFH protein, human
- Haptoglobins
- Complement Factor H
- Creatinine
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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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