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KMID : 0356920080550020244
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Korean Journal of Anesthesiology
2008 Volume.55 No. 2 p.244 ~ p.249
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Anesthetic care for living donor auxiliary partial orthotopic liver transplantation in the treatment of adult-onset type II citrullinemia
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Paek Cheol-Min
Chung Jun-Young
Yi Jae-Woo
Lee Bong-Jae
Kim Dong-Ok
Kang Jong-Man
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Abstract
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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)
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KEYWORD
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APOLT, citrullinemia, hyperammonemia, liver transplantation
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