| Conventional diet therapy for hyperammonemia is risky in the treatment of hepatic encephalopathy associated with citrin deficiency. | |
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MedLine Citation:
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PMID: 20118603 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Kazuhiro Fukushima; Masahide Yazaki; Mio Nakamura; Naoki Tanaka; Keiko Kobayashi; Takeyori Saheki; Hideki Takei; Shu-ichi Ikeda |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't Date: 2010-02-01 |
Journal Detail:
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Title: Internal medicine (Tokyo, Japan) Volume: 49 ISSN: 1349-7235 ISO Abbreviation: Intern. Med. Publication Date: 2010 |
Date Detail:
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Created Date: 2010-02-01 Completed Date: 2010-05-17 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9204241 Medline TA: Intern Med Country: Japan |
Other Details:
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Languages: eng Pagination: 243-7 Citation Subset: IM |
Affiliation:
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The Third Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Arginine
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therapeutic use* Citrullinemia / chemically induced, complications* Diet, Protein-Restricted / adverse effects* Dietary Supplements* Gene Deletion Hepatic Encephalopathy / complications, diet therapy*, etiology Humans Hyperammonemia / diet therapy* Male Middle Aged Mitochondrial Membrane Transport Proteins / genetics |
| Chemical | |
Reg. No./Substance:
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0/Mitochondrial Membrane Transport Proteins; 0/SLC25A13 protein, human; 74-79-3/Arginine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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