PMID: 3751563Jul 1, 1986Paper

Riboflavin responsive multiple acyl-CoA dehydrogenation deficiency. Assessment of 3 years of riboflavin treatment

Acta paediatrica Scandinavica
N GregersenS Kølvraa

Abstract

A boy with riboflavin-responsive beta-oxidation deficiency (multiple acyl-CoA dehydrogenation deficiency) was assessed clinically and biochemically after 3 years of continuous riboflavin medication. He was diagnosed at the age of three years after an attack of a Reye's syndrome-like disease. During the 3 years of assessment he has experienced no serious disease; although short episodes of fatigue and loss of appetite have been noted. His mental and physical development has been normal. Biochemically the abnormal excretion of organic acid metabolites, characteristic of the acyl-CoA dehydrogenation deficiency, has been continuously present. Quantitatively there has been a trend to a more simple picture with ethylmalonic acid as the predominant metabolite. However, because of the large within-day variation in the excretion of all the metabolites, changes following diet and riboflavin trials must be interpreted with caution in these patients.

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Citations

Jan 1, 1987·Journal of Inherited Metabolic Disease·H Przyrembel
Jan 1, 1991·Neuromuscular Disorders : NMD·A Papadimitriou, S Servidei
Mar 30, 2000·Pediatric Neurology·B T Darras, N R Friedman
May 11, 2000·Seminars in Perinatology·P T Ozand
Jan 24, 2014·Journal of Child Neurology·Manish Prasad, Shanawaz Hussain
Jun 7, 2003·The American Journal of Clinical Nutrition·Hilary J Powers
Aug 7, 2017·EFSA Journal·UNKNOWN EFSA Panel on Dietetic Products, Nutrition and Allergies (NDA)Monika Neuhäuser-Berthold
Apr 3, 2001·Current Treatment Options in Neurology·Roser Pons, Darryl C. De Vivo

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