Familial hemolytic-uremic syndrome in three generations

Am J Kidney Dis. 1998 Dec;32(6):1063-7. doi: 10.1016/s0272-6386(98)70084-8.


The hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are complex entities characterized by microangiopathic hemolytic anemia, thrombocytopenia, and variable impairment of renal function, occasionally complicated by neurological symptoms. In both syndromes, rare instances of familial forms have been reported. We present the case of a family in which signs and symptoms of HUS/TTP appeared in three generations. We also briefly review the literature on inherited forms of HUS/TTP and discuss the outcome of renal transplantation in adult patients with this syndrome.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Hemolytic-Uremic Syndrome / diagnosis
  • Hemolytic-Uremic Syndrome / genetics*
  • Hemolytic-Uremic Syndrome / therapy
  • Humans
  • Kidney Transplantation
  • Male
  • Middle Aged
  • Treatment Outcome