Complete neurological recovery of an adult patient with type II citrullinemia after living related partial liver transplantation

Transplantation
M YazakiT Saheki

Abstract

Type II citrullinemia is an adult-onset hepatocerebral disease caused by a deficiency of argininosuccinate synthetase in liver. A 25-year-old Japanese man suddenly developed encephalopathy, showing disorientation and flapping tremor. Plasma concentrations of ammonia and citrulline were extremely high, and hepatic argininosuccinate synthetase activity was deficient. The patient's condition deteriorated rapidly in spite of intensive medications. Therefore, we performed a partial liver transplantation using a graft obtained from his healthy 61-year-old father. After surgery, his neurological symptoms soon disappeared and plasma levels of ammonia and citrulline were normalized within 3 months after operation. Type II citrullinemia is one fulminant form of various liver-based metabolic diseases, and immediate liver transplantation is necessary to rescue patients with this disease. As liver transplantation from cadaveric donor is still not possible in Japan, it seems justifiable to use living related partial liver transplantation for our patient.

References

Mar 1, 1992·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·S TodoW A Fenton
Sep 1, 1989·The Journal of Pediatrics·C LargillièreJ P Farriaux
Jan 1, 1987·Reviews of Physiology, Biochemistry and Pharmacology·T SahekiI Inoue
Feb 5, 1981·Clinica Chimica Acta; International Journal of Clinical Chemistry·T SahekiT Katsunuma
Jan 1, 1982·Advances in Experimental Medicine and Biology·T SahekiT Katsunuma

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Citations

Jan 4, 2001·Journal of the Neurological Sciences·H MaruyamaN Sunohara
Nov 8, 2001·Journal of Pediatric Gastroenterology and Nutrition·F Lacaille, E Sokal
Oct 19, 2001·Journal of Neurology, Neurosurgery, and Psychiatry·S IkedaT Saheki
Jun 8, 2011·Journal of Transplantation·Hui-Hui TanChee-Kiat Tan
Oct 16, 2007·Chest·Alison S Clay, Bryan E Hainline
Jan 2, 2007·Journal of the Neurological Sciences·Ko-ichi TazawaShu-ichi Ikeda
Mar 30, 2004·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Masahide YazakiShu-ichi Ikeda
Aug 14, 2012·Hepatology Research : the Official Journal of the Japan Society of Hepatology·Masahide YazakiShu-Ichi Ikeda
May 25, 2013·Hepatology Research : the Official Journal of the Japan Society of Hepatology·Takayuki KogureTooru Shimosegawa
Aug 1, 1997·Clinics in Liver Disease·C O Esquivel
May 17, 2011·The Biochemical Journal·Stefan Bröer, Manuel Palacín
Jul 1, 2003·Expert Review of Neurotherapeutics·Shu-Ichi IkedaYasuhiko Hashikura
Mar 9, 2013·Journal of Nippon Medical School = Nippon Ika Daigaku Zasshi·Eiichi IshiiYuh Fukuda
Jan 5, 2005·Journal of Hepatology·Koichi Tanaka, Takako Yamada
Sep 6, 2003·Lancet·Koji UmeshitaUNKNOWN Japanese Liver Transplantation Society
Feb 28, 2017·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Amy TanThomas D Schiano
Aug 1, 1998·Journal of Inherited Metabolic Disease·P F WhitingtonJ M Millis

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