성인 Citrullinemia 환자의 생체 동소성 부분 보조 간이식 수술의 마취 경험 -증례보고

• Published in: Korean journal of anesthesiology Vol. 55; no. 2; pp. 244 - 249
• Main Authors: 백철민, Cheol Min Paek, 정준영, Jun Young Chung, 이재우, Jae Woo Yi, 이봉재, Bong Jae Lee, 김동옥, Dong Ok Kim, 강종만, Jong Man Kang
• Format: Journal Article
• Language: Korean
• Published: 대한마취통증의학회(구 대한마취과학회) 30.08.2008; 대한마취통증의학회
• Subjects: APOLT; citrullinemia; hyperammonemia; Liver transplantation; 마취과학
• ISSN: 2005-6419; 2005-7563

A deficiency of the urea cycle enzyme, argininosuccinate synthetase which is produced in liver, makes citrullinemia, which is an autosomal recessive disorder. As the liver is the only organ which transforms ammonia into urea, liver transplantation has been considered as an effective alternative therapy to classical dietary and medical therapy. We have experienced perioperative anesthetic care for a 27-year-old male with citrullinemia undergoing successful living donor auxiliary partial orthotopic liver transplantation (APOLT). After the liver transplantation, the postoperative clinical courses of the patient were uneventful, and the neurological symptoms were completely resolved. The plasma concentrations of ammonia and citrulline normalized rapidly without any kinds of protein dietary restrictions. We present this case with a brief review of literature. (Korean J Anesthesiol 2008; 55: 244~9)