Disease Information for VLCHAD/Very Long chain OH-CoA dehydrogenase def

Synonyms:

VLCHAD Very Long chain OH CoA dehydrogenase def

Definition:

Very long chain hydroxy-CoA dehydrogenase deficiency is essentially like long chain disease but more rare;

Long chain 3-Hydroxacyl-CoA dehydrogenase Deficiency metabolic enzyme defect; Hereditary extremely rare mitochondrial defect in all body cells; Severe fat dietary

restriction is indicated; Presentation often as failure to thrive infants; Family history positive;---------------------------------.Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD)

ACADL

LCAD Deficiency

Long Chain Acyl-CoA Dehydrogenase Deficiency

Nonketotic Hypoglycemia Caused by Deficiency of Acyl-CoA Dehydrogenase

VLCAD

Disorder Subdivisions

General Discussion

Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a rare genetic disorder of fatty acid metabolism that is transmitted as an autosomal recessive trait. It occurs when an enzyme needed to break down certain very long-chain fatty acids is missing or not working properly. VLCAD is one of the metabolic diseases known as fatty acid oxidation (FOD) diseases. In the past, the name long-chain acyl-CoA dehydrogenase deficiency (LCAD) was applied to one such disease, but today it is believed that all cases once thought to be LCAD are actually VLCAD.

The breakdown of fatty acids takes place in the mitochondria found in each cell. The mitochondria are small, well-defined bodies that are found in the cytoplasm of cells and in which the body generates energy from the breakdown of complex substances into simpler ones (mitochondrial oxidation); There appear to be two forms of VLCAD: an early-onset, severe form which, if unrecognized and undiagnosed, may lead to extreme weakness of the heart muscles (cardiomyopathy) and may be life-threatening (VLCAD-C), and a later-onset, milder form, sometimes referred to as VLCAD-H, that is characterized by repeated bouts of low blood sugar (hypoglycemia); Since the advent of expanded newborn screening programs using tandem mass spectrometry technology, more VLCAD infants are being detected earlier in the course of the disorder than in the past;[NORD 2005]

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