Conventional Diet Therapy for Hyperammonemia is Risky in the Treatment of Hepatic Encephalopathy Associated with Citrin Deficiency
Conventional Diet Therapy for Hyperammonemia is Risky in the Treatment of Hepatic Encephalopathy Associated with Citrin Deficiency
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DOI：
10.2169/internalmedicine.49.2712
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发表时间：
2010-01-01
影响因子：
1.2
通讯作者：
Ikeda, Shu-ichi
中科院分区：
文献类型：
--
作者：
Fukushima, Kazuhiro;Yazaki, Masahide;Ikeda, Shu-ichi
由SLC25A13基因突变引起的瓜氨酸缺乏症发展为成人发作的II型瓜氨酸血症（CTLN 2），表现为肝性脑病。最近的研究表明，过量的碳水化合物负荷是有害的柠檬素缺乏的个人。在这里，我们报告了一个CTLN 2的病人谁表现出进一步恶化的脑病后，就业的传统低蛋白饮食治疗慢性肝功能衰竭。由于肝性脑病患者体内碳水化合物含量较高，故应避免采用常规低蛋白饮食治疗。此外，我们的观察可能表明，碳水化合物含量低于每日能量摄入量50%的碳水化合物限制饮食对CTLN 2患者具有治疗效果。
Citrin deficiency caused by SLC25A13 gene mutations develops into adult-onset type II citrullinemia (CTLN2) presenting with hepatic encephalopathy. Recent studies have suggested that excessive loading of carbohydrates is harmful in citrin-deficient individuals. Here we report a CTLN2 patient who showed further deterioration of encephalopathy after the employment of conventional low-protein diet therapy for chronic liver failure. Owing to the high carbohydrate content, the conventional low-protein diet therapy should be avoided in patients with hepatic encephalopathy associated with citrin deficiency. In addition, our observation may suggest that carbohydrate-restricted diet in which the content of carbohydrate is below 50% of daily energy intake can have therapeutic efficacy in CTLN2 patients.