Posttransplant recurrence of atypical hemolytic uremic syndrome

Elisabetta Valoti, Marta Alberti, Marina Noris

Research output: Contribution to journalArticlepeer-review

Abstract

Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It is usually secondary to infections by strains of Escherichia coli (STEC) that produce Shiga-like toxin. In about 10% of patients, no STEC infections are reported. In these cases of atypical HUS (aHUS), mutations in genes encoding proteins of the complement system have been described. Atypical HUS is characterized by poor prognosis and by high risk of posttransplant recurrence which greatly depends on the specific gene mutation involved in the disease. Plasma therapy, eculizumab treatment and, in some cases, combined liver-kidney transplant have been used to prevent and/ or treat posttransplant aHUS recurrences.

Original languageEnglish
Pages (from-to)911-917
Number of pages7
JournalJournal of Nephrology
Volume25
Issue number6
DOIs
Publication statusPublished - Nov 2012

Keywords

  • Atypical hemolytic uremic syndrome (aHUS)
  • Eculizumab
  • Kidney transplant
  • Posttransplant aHUS recurrence

ASJC Scopus subject areas

  • Nephrology

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