[Novel Nutritional Management Regimen for Very Long-Chain acyl-CoA Dehydrogenase Deficiency]

Rinsho Shinkeigaku. 2010 Mar;50(3):172-4. doi: 10.5692/clinicalneurol.50.172.
[Article in Japanese]

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 (300 kcal 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.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Acyl-CoA Dehydrogenase, Long-Chain / deficiency*
  • Dietary Carbohydrates / administration & dosage
  • Female
  • Humans
  • Rhabdomyolysis / diet therapy*
  • Rhabdomyolysis / etiology*
  • Rhabdomyolysis / prevention & control
  • Secondary Prevention
  • Treatment Outcome
  • Triglycerides / administration & dosage
  • Triglycerides / chemistry
  • Young Adult

Substances

  • Dietary Carbohydrates
  • Triglycerides
  • Acyl-CoA Dehydrogenase, Long-Chain