title: Recurrence of hemolytic uremic syndrome after renal transplantation.
abstract: Non-Shiga toxin-associated hemolytic uremic syndrome (non-Stx-HUS) is a rare disease. The clinical outcome is often unfavorable: 50% of patients progress to end-stage renal failure. Several mutations in complement regulatory genes predispose to non-Stx-HUS. Transplantation outcomes are poor among patients with either mutation in the genes encoding complement H or I factors, with 80% graft loss due to HUS recurrence. In contrast, patients with mutation in the gene encoding MCP have no disease relapse after transplantation. There are no treatment guidelines for non-Stx-HUS recurrence. Herein we have presented 8 patients with non-Stx-HUS recurrence after transplantation during the last 10 years in the South of France. HUS recurrence, which occurred early after transplantation in all but 1 patient, was treated by plasma exchange (PE) with substitution by fresh frozen plasma (FFP). Three patients still treated with long-term plasma therapy have no recurrence at 15, 19, or 24 months. An international registry would help to define new guidelines.
authors.affiliation: Hôpital Pasteur, 30 Avenue de la Voie Romaine, 06002 Nice Cedex 1, France. barbara.seitz@wanadoo.fr
authors.forename: B
authors.initials: B
authors.lastname: Seitz
authors.forename: L
authors.initials: L
authors.lastname: Albano
authors.forename: F
authors.initials: F
authors.lastname: Vocila
authors.forename: S
authors.initials: S
authors.lastname: Mzoughi
authors.forename: R
authors.initials: R
authors.lastname: Aoudia
authors.forename: J
authors.initials: J
authors.lastname: Guitard
authors.forename: D
authors.initials: D
authors.lastname: Ribes
authors.forename: H
authors.initials: H
authors.lastname: Vachet-Copponat
authors.forename: G
authors.initials: G
authors.lastname: Mourad
authors.forename: F
authors.initials: F
authors.lastname: Bienaimé
authors.forename: P
authors.initials: P
authors.lastname: Dahan
authors.forename: V
authors.initials: V
authors.lastname: Frémeaux-Bacchi
authors.forename: E
authors.initials: E
authors.lastname: Cassuto
mesh_terms: D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics / Q000601:surgery*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000601:surgery*; D016030:Kidney Transplantation / Q000009:adverse effects*; D008297:Male; D009154:Mutation; D012008:Recurrence; D012086:Reoperation; D012189:Retrospective Studies