Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD)

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It is possible that the main title of the report Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD) is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.


  • nonketotic hypoglycemia caused by deficiency of acyl-CoA dehydrogenase

Disorder Subdivisions

  • None

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 clear 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).

Classically, two forms of VLCAD have been described: 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). In reality, patients may present with a combination of symptoms and the disease is best though of as being a continuum. Since the advent of expanded newborn screening programs using tandem mass spectrometry technology, most VLCAD infants in the United States are being detected neonatal period.


CLIMB (Children Living with Inherited Metabolic Diseases)
Climb Building
176 Nantwich Road
Crewe, CW2 6BG
United Kingdom
Tel: 4408452412173
Fax: 4408452412174

United Mitochondrial Disease Foundation
8085 Saltsburg Road Suite 201
Pittsburgh, PA 15239
United States
Tel: (412)793-8077
Fax: (412)793-6477
Tel: (888)317-8633

Organic Acidemia Association
P.O. Box 1008
Pinole, CA 94564
Tel: (763)559-1797
Fax: (763)694-0017

Organic Acidaemias UK
5, Saxon Road
Middlesex, TW15 1QL
United Kingdom
Tel: 4401784245989

NIH/National Institute of Diabetes, Digestive & Kidney Diseases
Office of Communications & Public Liaison
Bldg 31, Rm 9A06
31 Center Drive, MSC 2560
Bethesda, MD 20892-2560
Tel: (301)496-3583

FOD (Fatty Oxidation Disorders) Family Support Group
PO Box 54
Okemos, MI 48864
Tel: (517)381-1940
Fax: (866)290-5206

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223

14 Pembroke Street
Medford, MA 02155
Tel: (888)648-6228
Fax: (888)648-6228

Childhood Liver Disease Research and Education Network
c/o Joan M. Hines, Research Administrator
Children's Hospital Colorado
13123 E 16th Ave. B290
Aurora, CO 80045
Tel: (720)777-2598
Fax: (720)777-7351

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It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

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Last Updated:  6/27/2013
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