2024-03-29T13:58:33Z
https://soar-ir.repo.nii.ac.jp/oai
oai:soar-ir.repo.nii.ac.jp:00003879
2022-12-14T04:40:00Z
461:462
Conventional Diet Therapy for Hyperammonemia is Risky in the Treatment of Hepatic Encephalopathy Associated with Citrin Deficiency
Fukushima, Kazuhiro
Yazaki, Masahide
Nakamura, Mio
Tanaka, Naoki
Kobayashi, Keiko
Saheki, Takeyori
Takei, Hideki
Ikeda, Shu-ichi
citrin deficiency
CTLN2
low protein diet
carbohydrate
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)
journal article
JAPAN SOC INTERNAL MEDICINE
2010
application/pdf
INTERNAL MEDICINE
3
49
243
247
0918-2918
AA10827774
https://soar-ir.repo.nii.ac.jp/record/3879/files/Conventional_Diet_Therapy_Hyperammonemia.pdf
eng
20118603
https://pubmed.ncbi.nlm.nih.gov/20118603
10.2169/internalmedicine.49.2712
https://doi.org/10.2169/internalmedicine.49.2712
Copyright (c) 2010 The Japanese Society of Internal Medicine