Conventional Diet Therapy for Hyperammonemia is Risky in the Treatment of Hepatic Encephalopathy Associated with Citrin Deficiency
スポンサーリンク
概要
- 論文の詳細を見る
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.
- JAPAN SOC INTERNAL MEDICINEの論文
著者
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KOBAYASHI Keiko
Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Med
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Fukushima Kazuhiro
The Third Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
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Yazaki Masahide
The Third Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
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Nakamura Mio
Division of Nutrition, Shinshu University School of Medicine
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Tanaka Naoki
The Second Department of Medicine (Gastroenterology), Shinshu University School of Medicine
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Saheki Takeyori
Institute for Health Sciences, Tokushima Bunri University
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Takei Hideki
Department of Internal Medicine, Suwa Red Cross Hospital
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Ikeda Shu-ichi
The Third Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
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Takei Hideki
Department Of Civil Engineering Nagoya Institute Of Technology
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Ikeda Shu-ichi
The Third Department Of Internal Medicine Shinshu University School Of Medicine
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Kobayashi Keiko
Department Of Biochemical Pharmacology Faculty Of Pharmaceutical Sciences Chiba University
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Saheki Takeyori
Department Of Molecular Metabolism And Biochemical Genetics Kagoshima University Graduate School Of
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Saheki Takeyori
Institute For Health Sciences Tokushima Bunri University
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Kobayashi Keiko
Department Of Molecular Metabolism And Biochemical Genetics Kagoshima University Graduate School Of
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Kobayashi Keiko
Dep. Of Molecular Metabolism And Biochemical Genetics Kagoshima Univ. Graduate School Of Medical And
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Ikeda Shu-ihci
Dep. Of Medicine (neurology And Rheumatology) Shinshu Univ. School Of Medicine
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Nakamura Mio
Division Of Infection Control & Prevention And Infection Control Team Akita University Hospital
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