Successful Split Liver-Kidney Transplant for Factor H Associated Hemolytic Uremic Syndrome

Background and objectives: A male infant with a family history of thrombotic microangiopathy developed atypical hemolytic uremic syndrome (aHUS). Design, setting, participants, & measurements: Case report. Results: Genetic analysis demonstrated a heterozygous mutation (S1191L) of CFH, the gene coding complement factor H (CFH). The child suffered many episodes of HUS, each treated with plasma exchange. In time, despite initiation of a prophylactic regimen of plasma exchange, his renal function declined significantly. At the age of 4 yr he received a (split liver) combined liver-kidney transplant (LKT) with preoperative plasma exchange and enoxaparin anticoagulation. Initial function of both grafts was excellent and is maintained for nearly 2 yr. Conclusions: This report adds to the small but growing number of individuals in whom LKT has provided a favorable outcome for aHUS associated with CFH mutation, expands the technique of using a split liver graft, and describes the unique histologic features of subclinical liver disease in HUS. Clin J Am Soc Nephrol 4: 201–206, 2009. doi: 10.2215/CJN.02170508 I ndividuals who suffer ESRD due to atypical hemolyticuremic syndrome (aHUS) have poor outcomes after kid-ney transplant because of high rates of disease recurrence (1–3). In the case of carriers of a CFH [complement factor H (CFH)] mutation, the recurrence risk is over 80%, usually ac