title: Management of fatty acid oxidation disorders: a survey of current treatment strategies.
abstract: Standardization of the nutritional care for patients with fatty-acid oxidation disorders is lacking. A literature review and national survey of metabolic dietitians describes the range of therapeutic strategies currently employed in the U.S. to treat patients with fatty-acid oxidation disorders. Questionnaire responses provided by dietitians specializing in metabolic disorders evaluated practices used for treatment of fatty acid oxidation disorders, medium-chain acyl-CoA dehydrogenase deficiency (MCAD), very-long-chain acyl-CoA dehydrogenase deficiency (VLCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD), long-chain acyl-CoA dehydrogenase deficiency (LCAD), and Trifunctional Protein deficiency (TFP). This survey reveals a significant lack of evidence supporting the protocols in use. Recent advances in tandem mass spectrometry technology promises an increase in the number of identified patients with fatty-acid oxidation disorders, which reinforces the need for comprehensive, clinical research studies to determine optimal care for patients with these genetic disorders.
authors.affiliation: Sarah Lawrence College, Bronxville, NY, USA.
authors.forename: Java O
authors.initials: JO
authors.lastname: Solis
authors.forename: Rani H
authors.initials: RH
authors.lastname: Singh
mesh_terms: D015094:3-Hydroxyacyl CoA Dehydrogenases / Q000378:metabolism*; D000102:Acetyl-CoA C-Acyltransferase / Q000378:metabolism*; D000328:Adult; D019748:Carbon-Carbon Double Bond Isomerases / Q000378:metabolism*; D002648:Child; D004746:Enoyl-CoA Hydratase / Q000378:metabolism*; D006801:Humans; D007223:Infant; D008659:Metabolic Diseases / Q000178:diet therapy*; D019998:Racemases and Epimerases / Q000378:metabolism*; D011795:Surveys and Questionnaires
chemical_list: C038784:fatty acid oxidation complex; D015094:3-Hydroxyacyl CoA Dehydrogenases; D000102:Acetyl-CoA C-Acyltransferase; D004746:Enoyl-CoA Hydratase; D019998:Racemases and Epimerases; D019748:Carbon-Carbon Double Bond Isomerases