Successful isolated liver transplantation in a child with atypical hemolytic uremic syndrome and a mutation in complement factor H.
Am J Transplant
; 10(9): 2142-7, 2010 Sep.
Article
em En
| MEDLINE
| ID: mdl-20738267
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Transplante de Fígado
/
Fator H do Complemento
/
Síndrome Hemolítico-Urêmica
/
Mutação
Tipo de estudo:
Etiology_studies
/
Guideline
/
Risk_factors_studies
Limite:
Humans
/
Male
/
Newborn
Idioma:
En
Ano de publicação:
2010
Tipo de documento:
Article