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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Acknowledgments
We thank Christine Grapin and Virginie Fouquet for the kidney and liver transplantation, respectively, Catherine Baujard Simon and Philippe Roulleau for anesthesiology, Philippe Durand for intensive care management and Dominique Debray for helpful support.
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Gonzales, E., Ulinski, T., Habes, D. et al. Long-term successful liver–kidney transplantation in a child with atypical hemolytic uremic syndrome caused by homozygous factor H deficiency. Pediatr Nephrol 31, 2375–2378 (2016). https://doi.org/10.1007/s00467-016-3511-5
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DOI: https://doi.org/10.1007/s00467-016-3511-5