Familial Occurrence of the Haemolytic Uraemic Syndrome

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Abstract
A family is described in which the hemolytic uremic syndrome (HUS) occurred in 2 generations. Both juvenile and adult onset of this syndrome were observed in this family. Those affected were all women, 3 developed HUS in the postpartum period, 1 towards the end of pregnancy and 1 as a 5 yr old child. Because 5 cases were observed over 16 yr, exposure to the same infectious agent is highly unlikely. Although the transmission of a dormant virus cannot be excluded, the occurrence of HUS in 2 generations of the same family seems compatible with the hypothesis that susceptibility to the disease is transmitted as an autosomal dominant characteristic. A genetic influence apparently exists for the development of HUS, possibly in conjunction with other factors, such as infectious agents, pregnancy and/or delivery.