title: Fulminant recurrence of atypical hemolytic uremic syndrome during a calcineurin inhibitor-free immunosuppression regimen.
abstract: Recurrence of hemolytic uremic syndrome (HUS) after kidney transplantation is frequent, occurring almost exclusively in patients with atypical HUS, which is not caused by Escherichia coli gastroenteritis and in which diarrhea is absent. Calcineurin inhibitors are associated with recurrence of HUS. In two children who underwent living donor kidney transplantation for atypical HUS, we pre-emptively employed sirolimus in a calcineurin inhibitor-free immunosuppression regimen. Both children had excellent early graft function, yet both developed severe recurrent disease and subsequently lost their grafts. Avoidance of calcineurin inhibitors did not prevent recurrence of severe HUS and graft loss. Transplantation for severe atypical HUS remains problematic.
authors.affiliation: The Recanati/Miller Transplantation Institute and Department of Pediatric Nephrology, The Mount Sinai School of Medicine, New York, New York, USA. sandy.florman@mountsinai.org
authors.forename: Sander
authors.initials: S
authors.lastname: Florman
authors.forename: Corinne
authors.initials: C
authors.lastname: Benchimol
authors.forename: Kenneth
authors.initials: K
authors.lastname: Lieberman
authors.forename: Lewis
authors.initials: L
authors.lastname: Burrows
authors.forename: Jonathan S
authors.initials: JS
authors.lastname: Bromberg
mesh_terms: D019703:Calcineurin; D002675:Child, Preschool; D005260:Female; D006084:Graft Rejection / Q000209:etiology; D006463:Hemolytic-Uremic Syndrome / Q000150:complications* / Q000601:surgery*; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects*; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000601:surgery; D016030:Kidney Transplantation; D019520:Living Donors; D008297:Male; D012008:Recurrence; D012307:Risk Factors
chemical_list: D007166:Immunosuppressive Agents; D019703:Calcineurin