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Posttransplantation cytomegalovirus-induced recurrence of atypical hemolytic uremic syndrome associated with a factor H mutation: Successful treatment with intensive plasma exchanges and ganciclovir

https://doi.org/10.1053/j.ajkd.2004.09.012Get rights and content

Atypical hemolytic uremic syndrome (HUS) can recur after renal transplantation and often leads to graft loss. In some series of familial HUS, the risk of early graft loss due to recurrence of HUS approaches 100% despite any therapy. This led some authors to claim that kidney transplantation is contraindicated in those patients. The authors describe an 8-year-old girl with end-stage renal failure owing to familial atypical HUS with a factor H mutation who underwent successful transplantation using continuous prophylactic plasma exchange (PE). Twenty-four months after transplantation, plasma creatinine level is 1.2 mg/dL (106 μmol/L) despite 2 recurrences of HUS contemporaneous to 2 cytomegalovirus infections, which resolved with PE intensification and ganciclovir. This strongly suggests that cytomegalovirus infection may trigger posttransplant recurrent HUS. The feasibility of kidney transplantation in case of atypical HUS related to factor H mutation using continuous prophylactic PE intensified during relapses should be confirmed in prospective studies.

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Case report

The patient belongs to a family of 5 children, 3 of whom have presented with atypical HUS. The eldest affected child has lost 2 allografts to recurrent HUS and is currently treated by hemodialysis. The identical twin sister of the patient described in this report has normal kidney function 3 years after presentation. She is treated with regular prophylactic PE. In all 3 siblings, von Willebrand factor-cleaving protease activity is normal and no evidence of complement activation, as assessed by

Discussion

CMV infection in renal transplant recipients has both renal and systemic effects. 15 CMV can impair allograft function through the development of a transplant glomerulopathy 16, 17 and by predisposing to acute rejection and chronic allograft nephropathy. 18, 19 De novo thrombotic microangiopathy in association with CMV infection has also been reported in 3 adult transplant recipients 12, 20; in one, graft function did not recover. CMV increases the expression of endothelial adhesion molecules

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  • Cited by (0)

    Dr Goodship is supported by the National Kidney Research Fund of the United Kingdom.

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