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Pediatric and Adult Nutrition in Chronic Diseases, Developmental Disabilities, and Hereditary Metabolic DisordersPrevention, Assessment, and Treatment$
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Shirley W. Ekvall and Valli K. Ekvall

Print publication date: 2017

Print ISBN-13: 9780199398911

Published to Oxford Scholarship Online: April 2017

DOI: 10.1093/acprof:oso/9780199398911.001.0001

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PRINTED FROM OXFORD SCHOLARSHIP ONLINE (www.oxfordscholarship.com). (c) Copyright Oxford University Press, 2017. All Rights Reserved. Under the terms of the licence agreement, an individual user may print out a PDF of a single chapter of a monograph in OSO for personal use (for details see http://www.oxfordscholarship.com/page/privacy-policy).date: 15 December 2017

Isovaleric Acidemia and 3-Methylcrotonyl Glycinuria

Isovaleric Acidemia and 3-Methylcrotonyl Glycinuria

Chapter:
(p.305) 43 Isovaleric Acidemia and 3-Methylcrotonyl Glycinuria
Source:
Pediatric and Adult Nutrition in Chronic Diseases, Developmental Disabilities, and Hereditary Metabolic Disorders
Author(s):

Dianne Frazier

Publisher:
Oxford University Press
DOI:10.1093/acprof:oso/9780199398911.003.0043

This chapter discusses isovaleric acidemia (IVA) and 3-methylcrotonyl glycinuria, including clinical features and biochemical abnormalities of isovaleryl coenzyme A dehydrogenase deficiency (in IVA) and 3-methylcrotonyl-coenzyme A carboxylase deficiency (3-MCCD). Factors to be considered in nutritional evaluation and treatment, dietary management, monitoring, and follow-up care are discussed.

Keywords:   isovaleric acidemia, 3-methylcrotonyl glycinuria, 3-MCCD, dietary management, medical foods, screening

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