Carreras L, Romero R, Requesens C, Oliver A J, Carrera M, Clavo M, Alsina J
JAMA. 1981 Feb 13;245(6):602-4.
We describe a familial, relapsing type of hemolytic uremic syndrome (HUS) that has a possible genetic origin. Three members of a family, aged between 15 months and 34 years, evidenced HUS. Histological examination disclosed thrombotic microangiopathy. Recurrences, persistent hypocomplementemia, and the HLA haplotype A3,B7 were a common factor. We studied 17 members of this family and found a low C3 level from activation of the alternative pathway in the haplotype A3,B7 carriers. These three patients with HUS probably had a congenital and hereditary condition in which immunologic factors played a role in its pathogenesis.
