title: [Familial, recurring plasma infusion dependent thrombotic thrombocytopenic purpura/hemolytic uremic syndrome].
abstract: Two siblings with chronic relapsing TTP/HUS from early childhood are presented. An onset of the disease was probably neonatal period and it did not involved the organ until puberty. During puberty the course of the disease aggravated with renal involvement and became fulminant in a girl who died disseminated TTP. Her older brother had episode of severe atypical HUS responding to plasma therapy. Now, his renal function is normal but the symptoms are depend an regular, prophylactic plasma infusions.
authors.affiliation: Kliniki Dializoterapii i Transplantacji Nerek Instytut-Pomnik Centrum Zdrowia Dziecka w Warszawie.
authors.forename: M
authors.initials: M
authors.lastname: Litwin
authors.forename: E
authors.initials: E
authors.lastname: Smirska
mesh_terms: D000293:Adolescent; D002648:Child; D002908:Chronic Disease; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics* / Q000628:therapy*; D006801:Humans; D007262:Infusions, Intravenous; D010951:Plasma Exchange / Q000379:methods; D011697:Purpura, Thrombotic Thrombocytopenic / Q000235:genetics* / Q000628:therapy*; D012008:Recurrence