Shiga toxin-associated hemolytic uremic syndrome: absence of recurrence after renal transplantation

Abstract

We evaluated the relationship between the acute phase and the development of end-stage renal disease (ESRD) and the outcome of renal transplant in patients with Shiga toxin-associated hemolytic uremic syndrome (Stx-HUS). A 20-year retrospective study was performed of 66 renal transplants in 62 patients with Stx-HUS compared with 189 renal allografts in 178 children with other diseases. Of 62 patients, 61 had >7 days of oliguria during the acute phase. Stx-HUS patient survival was not different from controls (92% vs. 83% 15 years after renal transplantation). In the cyclosporine (CsA) era, survival of grafts from living related (LRD) and cadaver (CD) donors in Stx-HUS and control patients was 83% versus 70% ( P<0.03) and 77% versus 49% ( P<0.05) at 10 years. Graft survival in Stx-HUS and dysplasia/obstructive uropathy patients was 79% versus 76% ( P=NS), but it was different from that of other diseases (79% vs. 58%, P<0.001). There was no clinical or histopathological evidence of Stx-HUS recurrence. In conclusion, in Stx-HUS patients the duration of the acute oliguric period was a good predictor for the progression to ESRD. Use of CsA and the absence of recurrence of the disease influenced the excellent prognosis in Stx-HUS patients after renal transplantation. The development of ESRD in Stx-HUS could be mediated by non-immunological factors.

The risk of recurrence of hemolytic uremic syndrome after renal transplantation in children

“…In most series, no recurrence was observed [4,19,20,21,22]. We omitted 3 patients reported by Eijenraam et al [23] with possible recurrence of HUS, because the authors considered the diagnosis between HUS recurrence and rejection as uncertain.…”

“…The mean graft survival reported by Repetto [4] in 1997 was 7.16 years in HUS patients and 8.55 years in non-HUS patients. In the series of Ferraris et al [22] of patients treated with cyclosporine, survival of grafts from living-related and cadaver donors in VTEC-induced HUS and control patients was 83% versus 70% (P<0.03) and 77% versus 49% (P<0.05) at 10 years, respectively. Graft survival in VTEC-induced HUS compared with dysplasia/obstructive uropathy patients was 79% versus 76% (NS), but it was better than that of other diseases (79% versus 58%, P<0.001).…”

Outcome of Renal Transplantation in Patients with Non–Shiga Toxin–Associated Hemolytic Uremic Syndrome

“…It is generally accepted that renal transplantation is an effective and safe treatment for patients who have Stx-HUS and have progressed to ESRD. In children with Stx-HUS, the incidence of disease recurrence in the graft ranges from 0 to 10% (33,34), and graft survival at 10 yr is better than that in children who receive a transplant for other causes of ESRD (35). In contrast, disease recurrence and transplantation failure are common in patients with non-Stx-HUS (36 -38), even though the incidence varies widely in the literature.…”

Renal transplantation in patients with hemolytic uremic syndrome: high rate of recurrence and increased incidence of acute rejections1

“…It is well established that the outcome of renal transplantation is good in patients with childhood-onset, diarrheaassociated HUS, with recurrence rates ranging from 0% to 10% (6,13). Notably, prognosis is less favorable in children with atypical HUS, with recurrences occurring in over 50% of patients and graft failure developing in all of them (9).…”