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J Korean Surg Soc. 2011 Jun;80(Suppl 1):S51-S54. English. Case Report. https://doi.org/10.4174/jkss.2011.80.Suppl1.S51
Kim BS , Joo SH , Lee SH , Lee JI , Kim HC , Nam DH , Park HC .
Department of Surgery, Kyung Hee University Gangdong Hospital, Kyung Hee University School of Medicine, Seoul, Korea. sunhyung@chol.com
Department of Internal Medicine, Kyung Hee University Gangdong Hospital, Kyung Hee University School of Medicine, Seoul, Korea.
Department of Radiology, Kyung Hee University Gangdong Hospital, Kyung Hee University School of Medicine, Seoul, Korea.
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.

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