Internal Medicine, 2010, Vol.49(3), pp.243-247
2010
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- Autor(en) / Beteiligte
- Titel
- Conventional Diet Therapy for Hyperammonemia is Risky in the Treatment of Hepatic Encephalopathy Associated with Citrin Deficiency
- Ist Teil von
- Internal Medicine, 2010, Vol.49(3), pp.243-247
- Ort / Verlag
- Japan: The Japanese Society of Internal Medicine
- Erscheinungsjahr
- 2010
- Quelle
- Free E-Journal (出版社公開部分のみ）
- Beschreibungen/Notizen
- 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.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 0918-2918, 1349-7235eISSN: 1349-7235DOI: 10.2169/internalmedicine.49.2712Titel-ID: cdi_proquest_miscellaneous_733525059
- Format
- –
- Schlagworte
- Arginine - therapeutic use, carbohydrate, Carbohydrates, citrin deficiency, Citrullinemia - chemically induced, Citrullinemia - complications, CTLN2, Diet, Protein-Restricted - adverse effects, Dietary Supplements, Diets, Encephalopathy, Energy intake, Gene Deletion, Hepatic encephalopathy, Hepatic Encephalopathy - complications, Hepatic Encephalopathy - diet therapy, Hepatic Encephalopathy - etiology, Humans, hyperammonemia, Hyperammonemia - diet therapy, Liver diseases, Low protein diet, Male, Middle Aged, Mitochondrial Membrane Transport Proteins - genetics, Point mutation