Complete neurological recovery of an adult patient with type II citrullinemia after living related partial liver transplantation.
Complete neurological recovery of an adult patient with type II citrullinemia after living related partial liver transplantation.
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一名患有 II 型瓜氨酸血症的成年患者在活体部分肝移植后完全神经功能恢复。
DOI：
10.1097/00007890-199612150-00027
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发表时间：
1996
期刊：
影响因子：
6.2
通讯作者：
Takeyori Saheki
中科院分区：
文献类型：
--
作者：
Masahide Yazaki;Shu;Y. Takei;Nobuo Yanagisawa;H. Matsunami;Y. Hashikura;Seiji Kawasaki;Masatoshi Makuuchi;Keiko Kobayashi;Takeyori Saheki
II型瓜氨酸血症是一种由肝脏中的氨基琥珀酸合成酶缺乏引起的成人发病的肝脑疾病。一名25岁的日本男子突然出现脑病，表现为定向障碍和扑翼震颤。氨和瓜氨酸的血浆浓度极高，肝脏氨基琥珀酸合成酶活性不足。尽管进行了强化药物治疗，但患者的病情迅速恶化。因此，我们使用从他健康的61岁父亲那里获得的移植物进行了部分肝移植。手术后，他的神经系统症状很快消失，血浆氨和瓜氨酸水平在术后3个月内恢复正常。II型瓜氨酸血症是各种以肝脏为基础的代谢性疾病的一种暴发性形式，需要立即进行肝移植来挽救患有这种疾病的患者。由于在日本还不能进行尸体供者的肝移植，我们的病人采用活体部分肝移植似乎是合理的。
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.