Long-term successful liver-kidney transplantation in a child with atypical hemolytic uremic syndrome caused by homozygous factor H deficiency.
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.
['Gonzales|Emmanuel|E|', 'Ulinski|Tim|T|', 'Habes|Dalila|D|', 'Deschênes|Georges|G|', 'Frémeaux-Bacchi|Véronique|V|', 'Bensman|Albert|A|0000-0001-9083-7469']
D065766:Atypical Hemolytic Uremic Syndrome D017242:Complement Factor H D003241:Consanguinity D000081208:Hereditary Complement Deficiency Diseases D006801:Humans D007223:Infant D007674:Kidney Diseases D007676:Kidney Failure, Chronic D016030:Kidney Transplantation D016031:Liver Transplantation D008297:Male D010951:Plasma Exchange D016896:Treatment Outcome
Atypical hemolytic uremic syndrome End-stage renal disease Combined liver–kidney transplantation Complement factor H deficiency