1. Management of Fatty Acid Oxidation Disorders
- Author
-
Rani H. Singh and Java O Solis
- Subjects
medicine.medical_specialty ,Nutrition and Dietetics ,business.industry ,Diet therapy ,Metabolic disorder ,medicine.disease ,Bioinformatics ,Dehydrogenase deficiency ,Clinical research ,Endocrinology ,Internal medicine ,Medicine ,Treatment strategy ,Nutritional care ,business ,Beta oxidation ,Food Science - 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.
- Published
- 2002