Diseases & Conditions


A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD)


Synonyms of 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.
    .

    Organizations related to Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD)
    • CLIMB (Children Living with Inherited Metabolic Diseases)
      Climb Building
      Crewe Intl CW2 6BG
      Phone #: +44- 87-0 7700 325
      800 #: --
      e-mail: info@climb.org.uk
      Home page: http://www.CLIMB.org.uk
    • FOD (Fatty Oxidation Disorders) Family Support Group
      2041 Tomahawk
      Okemos MI 48864
      Phone #: 517-381-1940
      800 #: --
      e-mail: deb@fodsupport.org
      Home page: http://www.fodsupport.org
    • Madisons Foundation
      PO Box 241956
      Los Angeles CA 90024
      Phone #: 310-264-0826
      800 #: N/A
      e-mail: getinfo@madisonsfoundation.org
      Home page: http://www.madisonsfoundation.org
    • NIH/National Digestive Diseases Information Clearinghouse
      2 Information Way
      Bethesda MD 20892-3570
      Phone #: 301-654-3810
      800 #: 800-891-5389
      e-mail: nddic@info.niddk.nih.gov
      Home page: http://www.niddk.nih.gov
    • Organic Acidaemias UK
      5 Saxon Road
      Middlesex Intl TW15 1QL
      Phone #: 44--178-4-245989
      800 #: --
      e-mail: davidpriddy@bigfoot.com
      Home page: N/A
    • Organic Acidemia Association
      13210 35th Avenue North
      Plymouth MN 55441
      Phone #: 763-559-1797
      800 #: --
      e-mail: OAANews@aol.com
      Home page: http://www.oaanews.org
    • United Mitochondrial Disease Foundation
      8085 Saltsburg Road
      Pittsburgh PA 15239
      Phone #: 412-793-8077
      800 #: --
      e-mail: info@umdf.org
      Home page: http://www.umdf.org



    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html