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Diseases

Genetic and Rare Diseases Information Center (GARD)

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VLCAD deficiency


Other Names for this Disease
  • Very long-chain acyl-CoA dehydrogenase deficiency
  • VLCADD
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Prognosis


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What is the long-term outlook for an individual with VLCAD deficiency?

The long-term outlook (prognosis) for individuals with VLCAD deficiency is hard to predict and can vary depending on the form of the condition present in the affected individual.

For individuals with the severe, early-onset form, cardiomyopathy and arrhythmias can be lethal. Although the effects of cardiomyopathy may be severe, heart function can be improved with early, intensive, supportive care and specific diet modifications. Normal intellectual outcome has been reported in individuals with this form.

For individuals with the hepatic (liver) or hypoketotic hypoglycemic form, hypoketotic hypoglycemia can cause a loss of consciousness or seizures. Individuals with hypoglycemia associated with a large quantity of ketones reportedly are less likely to have impairment of fatty acid oxidation metabolism than those with a small or undetectable amount of ketones; however, ketones may be present in all individuals with this form.

Individuals with the later-onset episodic myopathic form may only have muscle-related symptoms. Some individuals with this form may not be diagnosed until adulthood. Since the later-onset form can have vague or intermittent symptoms, it is possible that some individuals may have no symptoms during their lifetime.[1]
Last updated: 12/10/2013

References
  1. 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.