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, Giuseppe Remuzzi

Research output: Contribution to journalArticle

50 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)201-206
Number of pages6
JournalClinical Journal of the American Society of Nephrology
Volume4
Issue number1
DOIs
StatePublished - Jan 1 2009

ASJC Scopus subject areas

  • Epidemiology
  • Critical Care and Intensive Care Medicine
  • Nephrology
  • Transplantation

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    Saland, J. M., Shneider, B. L., Bromberg, J. S., Shi, P. A., Ward, S. C., Magid, M. S., Benchimol, C., Seikaly, M. G., Emre, S. H., Bresin, E., & Remuzzi, G. (2009). Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome. Clinical Journal of the American Society of Nephrology, 4(1), 201-206. https://doi.org/10.2215/CJN.02170508