Type II Citrullinemia in an Elderly Patient Treated with Living Related Partial Liver Transplantation

Kiyoyuki TAKENAKA; Ichiro YASUDA; Hiroshi ARAKI; Tomoo NAITO; Yasushi FUKUTOMI; Hiroo OHNISHI; Noriyoshi YAMAKITA; Takeshi HASEGAWA; Hirohide SATO; Yasunobu SHIMIZU; Hidetoshi MATSUNAMI; Hisataka MORIWAKI

doi:10.2169/internalmedicine.39.553

Kiyoyuki TAKENAKA, Ichiro YASUDA, Hiroshi ARAKI, Tomoo NAITO, Yasushi FUKUTOMI, Hiroo OHNISHI, Noriyoshi YAMAKITA, Takeshi HASEGAWA, Hirohide SATO, Yasunobu SHIMIZU, Hidetoshi MATSUNAMI, Hisataka MORIWAKI

Author information

Kiyoyuki TAKENAKA
the First Department of Internal Medicine, Gifu University School of Medicine
Ichiro YASUDA
the First Department of Internal Medicine, Gifu University School of Medicine
Hiroshi ARAKI
the First Department of Internal Medicine, Gifu University School of Medicine
Tomoo NAITO
the First Department of Internal Medicine, Gifu University School of Medicine
Yasushi FUKUTOMI
the First Department of Internal Medicine, Gifu University School of Medicine
Hiroo OHNISHI
the First Department of Internal Medicine, Gifu University School of Medicine
Noriyoshi YAMAKITA
Matsunami General Hospital
Takeshi HASEGAWA
Matsunami General Hospital
Hirohide SATO
Matsunami General Hospital
Yasunobu SHIMIZU
Matsunami General Hospital
Hidetoshi MATSUNAMI
Matsunami General Hospital
Hisataka MORIWAKI
the First Department of Internal Medicine, Gifu University School of Medicine

Keywords: consciousness disturbance, hyperammonemia, argininosuccinate synthetase

JOURNAL FREE ACCESS

2000 Volume 39 Issue 7 Pages 553-558

DOI https://doi.org/10.2169/internalmedicine.39.553

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Abstract

A 60-year-old woman was admitted to our hospital for repeated consciousness disturbance. Blood examination showed hyperammonemia, and plasma amino acid analysis revealed a marked increase in the citrulline level. To establish a diagnosis, a percutaneous needle biopsy of the liver was performed. The determination of the urea cycle enzyme activities revealed a selective marked decrease in argininosuccinate synthetase activity, indicating the final diagnosis of type II citrullinemia. The mean survival period of this disease after the appearance of symptoms has been reported as 26.4 months, and most conservative treatments are not effective. We performed a living related partial liver transplantation. Over the subsequent 13-month follow-up, the patient's condition has remained fairly good.
(Internal Medicine 39: 553-558, 2000)

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