@article{oai:soar-ir.repo.nii.ac.jp:00003879, author = {Fukushima, Kazuhiro and Yazaki, Masahide and Nakamura, Mio and Tanaka, Naoki and Kobayashi, Keiko and Saheki, Takeyori and Takei, Hideki and Ikeda, Shu-ichi}, issue = {3}, journal = {INTERNAL MEDICINE}, month = {}, note = {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., Article, INTERNAL MEDICINE. 49(3):243-247 (2010)}, pages = {243--247}, title = {Conventional Diet Therapy for Hyperammonemia is Risky in the Treatment of Hepatic Encephalopathy Associated with Citrin Deficiency}, volume = {49}, year = {2010} }