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Inherited hemolytic uremic syndrome in adults
J S Berns1, B S Kaplan, R C Mackow
1Division of Nephrology and Hypertension, Graduate Hospital, Philadelphia, PA 19146.
Summary
Familial hemolytic uremic syndrome (HUS) can be inherited. This study describes a family with autosomal dominant HUS, where pregnancy and oral contraceptives were identified as potential triggers in adult women.
Area of Science:
- Nephrology
- Genetics
- Hematology
Background:
- Familial hemolytic uremic syndrome (HUS) is often autosomal recessive in children.
- Familial HUS in adults is less understood, with possible autosomal dominant or recessive inheritance patterns.
Observation:
- A family presented with autosomal dominant inheritance of HUS.
- The proband was an adult male.
- His sister developed HUS while using oral contraceptives, and their mother developed HUS during pregnancy.
Findings:
- This family exhibits autosomal dominant HUS.
- Adult women with a family history of HUS or TTP may face increased risk from oral contraceptives and pregnancy.
Implications:
- Pregnancy and oral contraceptives are potential risk factors for HUS in susceptible adult women.
- Early identification and risk assessment are crucial for families with a history of HUS or TTP.