Management of fatty acid oxidation disorders: a survey of current treatment strategies
- PMID: 12487544
- DOI: 10.1016/s0002-8223(02)90386-x
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.
Similar articles
-
Differentiation of long-chain fatty acid oxidation disorders using alternative precursors and acylcarnitine profiling in fibroblasts.Mol Genet Metab. 2006 Jan;87(1):40-7. doi: 10.1016/j.ymgme.2005.09.018. Epub 2005 Nov 16. Mol Genet Metab. 2006. PMID: 16297647
-
Exercise testing in metabolic myopathies.Phys Med Rehabil Clin N Am. 2012 Feb;23(1):173-86, xii. doi: 10.1016/j.pmr.2011.11.011. Epub 2011 Dec 11. Phys Med Rehabil Clin N Am. 2012. PMID: 22239882 Review.
-
Incidence and short-term outcome of children with symptomatic presentation of organic acid and fatty acid oxidation disorders in Germany.Pediatrics. 2002 Dec;110(6):1204-11. doi: 10.1542/peds.110.6.1204. Pediatrics. 2002. PMID: 12456920
-
Differential induction of genes in liver and brown adipose tissue regulated by peroxisome proliferator-activated receptor-alpha during fasting and cold exposure in acyl-CoA dehydrogenase-deficient mice.Mol Genet Metab. 2005 Jan;84(1):39-47. doi: 10.1016/j.ymgme.2004.09.010. Epub 2004 Nov 11. Mol Genet Metab. 2005. PMID: 15639194
-
[Mass Screening for Inborn Errors of Metabolism].Rinsho Byori. 2015 Apr;63(4):441-9. Rinsho Byori. 2015. PMID: 26536777 Review. Japanese.
Cited by
-
Clinical outcome, biochemical and therapeutic follow-up in 14 Austrian patients with Long-Chain 3-Hydroxy Acyl CoA Dehydrogenase Deficiency (LCHADD).Orphanet J Rare Dis. 2015 Feb 22;10:21. doi: 10.1186/s13023-015-0236-7. Orphanet J Rare Dis. 2015. PMID: 25888220 Free PMC article.
-
Very long-chain acyl-CoA dehydrogenase deficiency: the effects of accidental fat loading in a patient detected through newborn screening.J Inherit Metab Dis. 2009 Dec;32 Suppl 1:S187-90. doi: 10.1007/s10545-009-1143-7. Epub 2009 Apr 4. J Inherit Metab Dis. 2009. PMID: 19333779
-
Importance of acylcarnitine profile analysis for disorders of lipid metabolism in adolescent patients with recurrent rhabdomyolysis: Report of two cases.Ann Indian Acad Neurol. 2014 Oct;17(4):437-40. doi: 10.4103/0972-2327.144031. Ann Indian Acad Neurol. 2014. PMID: 25506168 Free PMC article.
-
Advances in genetics: what are the benefits for patients?J Med Genet. 2006 Jul;43(7):555-6. doi: 10.1136/jmg.2005.039966. Epub 2005 Dec 9. J Med Genet. 2006. PMID: 16339848 Free PMC article. Review. No abstract available.
-
MCT oil-based diet reverses hypertrophic cardiomyopathy in a patient with very long chain acyl-coA dehydrogenase deficiency.Indian J Hum Genet. 2011 Jan;17(1):29-32. doi: 10.4103/0971-6866.82190. Indian J Hum Genet. 2011. PMID: 21814341 Free PMC article.
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical
Miscellaneous
