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Successful Isolated Liver Transplantation in a Child with Atypical Hemolytic Uremic Syndrome and a Mutation in Complement Factor H

Lookup NU author(s): Professor Tim Goodship

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Abstract

A male infant was diagnosed with atypical hemolytic uremic syndrome (aHUS) at the age of 5.5 months. Sequencing of the gene (CFH) encoding complement factor H revealed a heterozygous mutation (c.3644G > A, p.Arg1215Gln). Despite maintenance plasmapheresis he developed recurrent episodes of aHUS and vascular access complications while maintaining stable renal function. At the age of 5 years he received an isolated split liver graft following a previously established protocol using pretransplant plasma exchange (PE) and intratransplant plasma infusion. Graft function, renal function and disease remission are preserved 2 years after transplantation. Preemptive liver transplantation prior to the development of end stage renal disease is a valuable option in the management of aHUS associated with CFH mutations.

Publication metadata

Author(s): Haller W, Milford DV, Goodship THJ, Sharif K, Mirza DF, McKiernan PJ

Publication type: Article

Publication status: Published

Journal: American Journal of Transplantation

Year: 2010

Volume: 10

Issue: 9

Pages: 2142-2147

Print publication date: 01/09/2010

ISSN (print): 1600-6135

ISSN (electronic): 1600-6143

Publisher: Wiley-Blackwell Publishing Inc.

URL: http://dx.doi.org/10.1111/j.1600-6143.2010.03228.x

DOI: 10.1111/j.1600-6143.2010.03228.x

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