A novel nutritional management regimen for very long-chain acyl-CoA dehydrogenase deficiency

• Haruki Hiroyo

  Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine

• Kawai Motoharu

  Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine

• Ogasawara Jun-ichi

  Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine

• Koga Michiaki

  Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine

• Negoro Kiyoshi

  Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine

• Kanda Takashi

  Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine

Bibliographic Information

Other Title

• 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

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

• Rinsho Shinkeigaku

  Rinsho Shinkeigaku 50 (3), 172-174, 2010

  Societas Neurologica Japonica

Keywords

• very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency
• nutritional management
• medium chain triglyceride (MCT) oil
• recurrent rhabdomyolysis

Details

• CRID: 1390001205035328128
• NII Article ID: 130004504752
• NII Article ID: 10026291634
• NII Book ID: AN00253207
• DOI: 10.5692/clinicalneurol.50.172
• ISSN: 18820654
• ISSN: 0009918X
• NDL BIB ID: 10608829
• PubMed: 20235487
• Web Site: https://ndlsearch.ndl.go.jp/books/R000000004-I10608829
• Web Site: https://search.jamas.or.jp/link/ui/2010143949
• Text Lang: ja
• Data Source:
  • JaLC
  • NDL
  • Crossref
  • PubMed
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed