J Korean Surg Soc.  2011 Jun;80(Suppl 1):S51-S54. 10.4174/jkss.2011.80.Suppl1.S51.

Auxiliary partial orthotopic liver transplantation for adult onset type II citrullinemia

• Kim BS
• Joo SH
• Lee SH
• Lee JI
• Kim HC
• Nam DH
• Park HC

• KMID: 2096651
• DOI: http://doi.org/10.4174/jkss.2011.80.Suppl1.S51

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.

Keyword

Citrullinemia; Auxiliary partial orthotopic liver transplantation

MeSH Terms

Adult
Ammonia
Argininosuccinate Synthase
Citrulline
Citrullinemia
Humans
Hyperammonemia
Korea
Liver
Liver Transplantation
Living Donors
Plasma
Tissue Donors
Ammonia
Argininosuccinate Synthase
Citrulline

Figure

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  Fig. 1 On postoperative day 7, abdominal computed tomography shows orthotopically transplanted small left lobe graft with well-enhanced pattern.

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  Fig. 2 (A) Direct portography shows the stenosis of left portal vein (white arrow heads). (B) The embolization of 8th segmental branch of right portal vein was successfully performed. (C) Self-expandable stent placement for left portal vein. After stent placement, the left portal flow was improved.

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  Fig. 3 On post stent insertion day 7, computed tomography volume of the graft is increased to 250 mL.

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