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Pediatric Nephrology

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01-12-2016 | Brief Report

Long-term successful liver–kidney transplantation in a child with atypical hemolytic uremic syndrome caused by homozygous factor H deficiency

Authors: Emmanuel Gonzales, Tim Ulinski, Dalila Habes, Georges Deschênes, Véronique Frémeaux-Bacchi, Albert Bensman

Published in: Pediatric Nephrology | Issue 12/2016

Abstract

Background

Rational options for the treatment of end-stage renal disease (ESRD) due to atypical hemolytic uremic syndrome (aHUS) in children are still open to discussion. In the case of human complement factor H (CFH) deficiency, the choice is either kidney transplantation in combination with eculizumab, a humanized anti-C5 monoclonal antibody, or a combined liver–kidney transplantation.

Case-Diagnosis/treatment

A child with a homozygous CFH deficiency underwent a successful liver–kidney transplantation. CFH levels normalized within days. After 6 years of follow-up, the graft function (Cockroft clearance 100 ml min⁻¹ 1.73 m⁻²) and the liver functions were normal.

Results and Conclusions

The results of this long-term follow-up confirm that combined liver–kidney transplantation remains a reasonable option in patients with ESRD due to aHUS when an identified genetic abnormality of the C3 convertase regulator synthesized in the liver has been identified.

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Title: Long-term successful liver–kidney transplantation in a child with atypical hemolytic uremic syndrome caused by homozygous factor H deficiency
Authors: Emmanuel Gonzales
Tim Ulinski
Dalila Habes
Georges Deschênes
Véronique Frémeaux-Bacchi
Albert Bensman
Publication date: 01-12-2016
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Nephrology / Issue 12/2016
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-016-3511-5

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Long-term successful liver–kidney transplantation in a child with atypical hemolytic uremic syndrome caused by homozygous factor H deficiency

Abstract

Background

Case-Diagnosis/treatment

Results and Conclusions

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Springer Medicine

Abstract

Background

Case-Diagnosis/treatment

Results and Conclusions

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