[Familial hemolytic uremic syndrome].
Hemolytic uremic syndrome (HUS) consists of an apparently heterogenous group of disorders resulting from a variety of diseases. Outcome and prognosis are largely determined by the basic disease. In the familial type there is frequently progressive deterioration and poor prognosis. The classic epidemic type usually has a better prognosis. The pathophysiology of familial HUS is unknown. Treatment includes transfusion of fresh frozen plasma or plasma exchanges. We report 2 siblings, male and female infants, who developed HUS at ages 7 and 8.5 months, respectively (but they were born 5 years apart). Their courses were characterized by slow onset, gradual deterioration, hypertension and fatal outcome in 1, and end-stage renal failure in the other.
Segal
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N
Dept. of Pediatrics, Kaplan Hospital, Rehovot.
Sinai
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L
D016913:Blood Component Transfusion; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics* / Q000628:therapy; D006801:Humans; D007223:Infant; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D010949:Plasma; D010951:Plasma Exchange; D011379:Prognosis