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

Jeffrey M. Saland^*,, Benjamin L. Shneider, Jonathan S. Bromberg^,, Patricia A. Shi^||,¶, Stephen C. Ward^¶, Margret S. Magid^¶, Corinne Benchimol^*,, Mouin G. Seikaly^**, Sukru H. Emre, Elena Bresin^,, and Giuseppe Remuzzi^,

^* Department of Pediatrics, Recanati/Miller Transplantation Institute, Departments of Surgery, Gene and Cell Medicine, Center for Immunobiology, ^|| Department of Medicine, and ^¶ Department of Pathology, The Mount Sinai Medical Center, New York, New York; Department of Pediatrics, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania; ^** Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas; Department of Surgery, Yale University School of Medicine, New Haven, Connecticut; Department of Medicine and Transplantation, Ospedali Riuniti di Bergamo, Bergamo, Italy; and "Mario Negri" Institute for Pharmacological Research, Bergamo, Italy

Correspondence: Dr. Jeffrey M. Saland, Department of Pediatrics, Box 1664, The Mount Sinai Medical Center, One Gustave L. Levy Place, New York, NY 10029. Phone: 212-241-6187; Fax: 212-426-1972; E-mail: Jeff.Saland{at}MSSM.EDU

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.

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