Auxiliary partial orthotopic liver transplantation for adult onset type II citrullinemia / 대한외과학회지
Journal of the Korean Surgical Society
;
: S51-S54, 2011.
Artigo
em Inglês
| WPRIM
| ID: wpr-164432
ABSTRACT
Adult-onset type II citrullinemia (CTLN2) is a disorder caused by an inborn error of metabolism affecting the liver. CTLN2 is an autosomal recessive disorder characterized by recurrent encephalopathy with hyperammonemia due to highly elevated plasma levels of citrulline and ammonia, caused by a deficiency of argininosuccinate synthetase in the liver. A small number of patients have undergone liver transplantation with favorable results. In Korea, the limitations of the deceased donor pool have made living donor liver transplantation a common alternative treatment option. We report the case of a patient with type II citrullinemia who was treated successfully with auxiliary partial orthotopic liver transplantation (APOLT) from a living donor. This is the first description of an APOLT for a patient with adult onset type II citrullinemia in Korea.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Argininossuccinato Sintase
/
Plasma
/
Doadores de Tecidos
/
Transplante de Fígado
/
Citrulina
/
Doadores Vivos
/
Citrulinemia
/
Hiperamonemia
/
Amônia
/
Coreia (Geográfico)
Limite:
Adulto
/
Humanos
País/Região como assunto:
Ásia
Idioma:
Inglês
Revista:
Journal of the Korean Surgical Society
Ano de publicação:
2011
Tipo de documento:
Artigo
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