A novel nutritional management regimen for very long-chain acyl-CoA dehydrogenase deficiency
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- Haruki Hiroyo
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine
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- Kawai Motoharu
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine
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- Ogasawara Jun-ichi
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine
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- Koga Michiaki
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine
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- Negoro Kiyoshi
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine
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- Kanda Takashi
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine
Bibliographic Information
- Other Title
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- 極長鎖アシルCoA脱水素酵素(VLCAD)欠損症の22歳女性例に対する食事療法の試み
- ゴク チョウサ アシル CoA ダツスイソ コウソ VLCAD ケッソンショウ ノ 22サイ ジョセイレイ ニ タイスル ショクジ リョウホウ ノ ココロミ
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Abstract
We report a novel regimen of nutritional management in 22-year-old woman with myopathic form of very-long-chain acyl-CoA dehydrogenase deficiency. This regimen is based on avoidance of fasting by frequent intake of carbohydrates and substitution of medium chain triglyceride for long- and very long-chain fatty acids. Oral intake of medium amount of long-chain fatty acid (300kcal daily) was allowed, to facilitate compliance and to escape pigmentary retinopathy. After this nutritional management and lifestyle guidance about prevention of fatigue and starvation, the patient was free from severe rhabdomyolysis for more than three years, which had forced her to hospital management nine times in seven years.<br>
Journal
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- Rinsho Shinkeigaku
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Rinsho Shinkeigaku 50 (3), 172-174, 2010
Societas Neurologica Japonica
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Keywords
Details 詳細情報について
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- CRID
- 1390001205035328128
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- NII Article ID
- 130004504752
- 10026291634
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- NII Book ID
- AN00253207
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- ISSN
- 18820654
- 0009918X
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- NDL BIB ID
- 10608829
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- PubMed
- 20235487
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- Text Lang
- ja
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- Data Source
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- JaLC
- NDL
- Crossref
- PubMed
- CiNii Articles
- KAKEN
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- Abstract License Flag
- Disallowed