Inherited hemolytic uremic syndrome in adults.
Familial hemolytic uremic syndrome (HUS) in children may be due to environmental factors or may be an inherited trait, usually with autosomal recessive inheritance. Familial HUS in adults is less well described, and can be associated with autosomal recessive or dominant inheritance. We report a family with autosomal dominant inheritance of HUS. The proband was an adult male. His adult sister developed HUS while taking oral contraceptives, and their mother developed HUS during the third trimester of pregnancy. The prognosis for these patients is poor. Pregnancy and oral contraceptive use may be risk factors for development of HUS in adult women with a family history of HUS or the related disorder thrombotic thrombocytopenic purpura (TTP).
Berns
J S
JS
Division of Nephrology and Hypertension, Graduate Hospital, Philadelphia, PA 19146.
Kaplan
B S
BS
Mackow
R C
RC
Hefter
L G
LG
D000328:Adult; D003276:Contraceptives, Oral / Q000009:adverse effects*; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000453:epidemiology / Q000209:etiology / Q000235:genetics*; D006801:Humans; D007668:Kidney / Q000473:pathology; D008297:Male; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000453:epidemiology; D011379:Prognosis; D012307:Risk Factors
D003276:Contraceptives, Oral