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

Congenital Pulmonary Lymphangiectasia


Synonyms of Congenital Pulmonary Lymphangiectasia
  • CPL
  • PPL
  • Primary Pulmonary Lymphangiectasia
  • Pulmonary Cystic Lymphangiectasis

Disorder Subdivisions



    General Discussion
    Congenital pulmonary lymphangiectasia (CPL) is a rare developmental disorder that is present at birth (congenital). Affected infants have abnormally widened (dilated) lymphatic vessels within the lungs. Functioning as part of the immune system, the lymphatic system helps to protect the body against infection and disease. It consists of a network of tubular channels (lymph vessels) that drain a thin watery fluid known as lymph from different areas of the body into the bloodstream. Lymph accumulates in the tiny spaces between tissue cells and contains proteins, fats, and certain white blood cells known as lymphocytes.

    Infants with CPL often develop severe, potentially life-threatening, respiratory distress shortly after birth. Affected infants may also develop cyanosis, a condition marked by abnormal bluish discoloration of the skin that occurs because of low levels of circulating oxygen in the blood. The exact cause of CPL is unknown.

    CPL can occur as a primary or secondary disorder. Primary pulmonary lymphangiectasia can occur as isolated congenital defect within the lungs or as part of a generalized form of lymphatic vessel malformation (lymphangiectasia) that affects the entire body. Secondary CPL occurs secondary to a variety of heart (cardiac) abnormalities.

    Organizations related to Congenital Pulmonary Lymphangiectasia
    • British Paediatric Orphan Lung Disease (BPOLD)
      None
      None None None
      Phone #: N/A
      800 #: N/A
      e-mail: N/A
      Home page: http://www.bpold.co.uk
    • Lymphatic Research Foundation
      100 Forest Avenue
      East Hills NY 11548
      Phone #: 516-625-9675
      800 #: --
      e-mail: lrf@lymphaticresearch.org
      Home page: http://www.lymphaticresearch.org
    • MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
      150 Custer Court
      Green Bay WI 54301-1243
      Phone #: 920-336-5333
      800 #: 877-336-5333
      e-mail: mums@netnet.net
      Home page: http://www.netnet.net/mums/
    • March of Dimes Birth Defects Foundation
      1275 Mamaroneck Avenue
      White Plains NY 10605
      Phone #: 914-428-7100
      800 #: 888-663-4637
      e-mail: Askus@marchofdimes.com
      Home page: http://www.marchofdimes.com
    • NIH/National Heart, Lung and Blood Institute Information Center
      P.O. Box 30105
      Bethesda MD 20824-0105
      Phone #: 301-592-8573
      800 #: --
      e-mail: nhlbiinfo@rover.nhlbi.nih.gov
      Home page: N/A
    • National Lymphedema Network
      Latham Square Building
      Oakland CA 94612-2138
      Phone #: 510-208-3200
      800 #: 800-541-3259
      e-mail: nln@lymphnet.org
      Home page: http://www.lymphnet.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