Auxiliary partial orthotopic liver transplantation for adult onset type II citrullinemia / 대한외과학회지
Journal of the Korean Surgical Society
; : S51-S54, 2011.
Article
在 En
| WPRIM
| ID: wpr-164432
Responsible library:
WPRO
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.
Key words
全文:
1
索引:
WPRIM
主要主题:
Argininosuccinate Synthase
/
Plasma
/
Tissue Donors
/
Liver Transplantation
/
Citrulline
/
Living Donors
/
Citrullinemia
/
Hyperammonemia
/
Ammonia
/
Korea
限制:
Adult
/
Humans
国家/地区名称主题:
Asia
语言:
En
期刊:
Journal of the Korean Surgical Society
年:
2011
类型:
Article