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Favorable Long‐Term Outcome after Liver‐Kidney Transplant for Recurrent Hemolytic Uremic Syndrome Associated with a Factor H Mutation
Author(s) -
Saland J. M.,
Emre S. H.,
Shneider B. L.,
Benchimol C.,
Ames S.,
Bromberg J. S.,
Remuzzi G.,
Strain L.,
Goodship T. H. J.
Publication year - 2006
Publication title -
american journal of transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.89
H-Index - 188
eISSN - 1600-6143
pISSN - 1600-6135
DOI - 10.1111/j.1600-6143.2006.01375.x
Subject(s) - medicine , atypical hemolytic uremic syndrome , dialysis , kidney disease , kidney , renal function , risk factor , gastroenterology , liver disease , transplantation , liver transplantation , kidney transplantation , complement system , immunology , antibody
A male child initially presented with atypical hemolytic uremic syndrome (HUS) at the age of 4 months and progressed within weeks to end stage renal disease (ESRD). At the age of 2 years he received a live‐related kidney transplant from his mother, which, despite initial good function, was lost to recurrent disease after 2 weeks. Complement factor H analysis showed low serum levels and the presence of two mutations on different alleles (c.2918G > A, Cys973Tyr and c.3590T > C, Val1197Ala). His survival on dialysis was at risk because of access failure and recurrent bacteremic episodes. Therefore, at the age of 5 years he received a combined liver‐kidney transplant with pre‐operative plasma exchange. Initial function of both grafts was excellent and this has been maintained for over 2 years. This report suggests that despite setbacks in previous experience, combined liver‐kidney transplantation offers the prospect of a favorable long‐term outcome for patients with HUS associated with complement factor H mutations.