Type II citrullinemia in an elderly patient treated with living related partial liver transplantation

被引：21

作者：

Takenaka, K ^{[1
]}

Yasuda, I ^{[1
]}

Araki, H ^{[1
]}

Naito, T ^{[1
]}

Fukutomi, Y ^{[1
]}

Ohnishi, H ^{[1
]}

Yamakita, N ^{[1
]}

Hasegawa, T ^{[1
]}

Sato, H ^{[1
]}

Shimizu, Y ^{[1
]}

Matsunami, H ^{[1
]}

Moriwaki, H ^{[1
]}

机构：

[1] Gifu Univ, Sch Med, Dept Internal Med 1, Gifu 5008705, Japan

来源：

INTERNAL MEDICINE | 2000年 / 39卷 / 07期

关键词：

consciousness disturbance; hyperammonemia; argininosuccinate synthetase;

D O I：

10.2169/internalmedicine.39.553

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：553 / 558

页数：6

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