Other Names for this Disease
- Very long chain acyl-CoA dehydrogenase deficiency
- Very long-chain acyl-CoA dehydrogenase deficiency
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Management of VLCAD deficiency depends on the form of the condition present and the specific signs and symptoms in the affected individual. Options may include intravenous glucose as an energy source, treatment of heart rhythm abnormalities, and monitoring for rhabdomyolysis (breakdown of muscle fibers). Heart problems may be reversible with early, intensive supportive care and diet modifications. Individuals with severe forms of the condition are typically placed on a low-fat formula, with supplemental calories provided through medium-chain triglycerides (MCT). Acute rhabdomyolysis may be treated with hydration and alkalization of the urine (decreasing the amount of acid you take in) to protect kidney function and to prevent acute kidney failure. Affected individuals are generally advised to avoid fasting, myocardial irritation (such as cardiac catheterization), dehydration, and a high fat diet.
Last updated: 12/10/2013
- Nancy D Leslie, Brad T Tinkle, Arnold W Strauss, Kerry Shooner, and Kejian Zhang. Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency. GeneReviews. September 22, 2011; http://www.ncbi.nlm.nih.gov/books/NBK6816/. Accessed 12/10/2013.
- GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.
- Genetic Metabolic Dietitians International (GMDI) has developed nutrition guidelines for this condition.
- Orphanet Emergency Guidelines is an article which is expert-authored and peer-reviewed that is intended to guide health care professionals in emergency situations involving this condition.
- ClinicalTrials.gov lists trials that are studying or have studied VLCAD deficiency. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.