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Korean Journal of Anesthesiology 2008;55(2):244-249.
DOI: https://doi.org/10.4097/kjae.2008.55.2.244   
Anesthetic care for living donor auxiliary partial orthotopic liver transplantation in the treatment of adult-onset type II citrullinemia: A case report.
Cheol Min Paek, Jun Young Chung, Jae Woo Yi, Bong Jae Lee, Dong Ok Kim, Jong Man Kang
Department of Anesthesiology and Pain Medicine, East-West Neo Medical Center, College of Medicine, Kyung Hee University, Seoul, Korea. kjm@khnmc.or.kr
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.
Key Words: APOLT; citrullinemia; hyperammonemia; liver transplantation


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