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

Autor:	Hiroyo, Haruki, Motoharu, Kawai, Jun-Ichi, Ogasawara, Michiaki, Koga, Kiyoshi, Negoro, Takashi, Kanda
Rok vydání:	2010
Předmět:	medicine.medical_specialty business.industry Acyl-CoA Dehydrogenase Long-Chain Gastroenterology Rhabdomyolysis Young Adult Treatment Outcome Internal medicine Dietary Carbohydrates Secondary Prevention medicine Humans Female Neurology (clinical) business Triglycerides
Zdroj:	Rinsho Shinkeigaku. 50:172-174
ISSN:	1882-0654 0009-918X
DOI:	10.5692/clinicalneurol.50.172
Popis:	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.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::79d0c5dd460321d2d3f1649b473a9455 https://doi.org/10.5692/clinicalneurol.50.172 Zobrazit plný text záznamu