Congenital Pulmonary Lymphangiectasia
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Congenital Pulmonary Lymphangiectasia 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.
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. The lymphatic system helps the immune system in protecting 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, usually associated with generalized lymphedema. Secondary CPL occurs secondary to a variety of heart (cardiac) abnormalities, and/or lymphatic obstructive forms.
British Paediatric Orphan Lung Disease
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Genetic and Rare Diseases (GARD) Information Center
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- Gaithersburg, MD 20898-8126
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- Tel: (888)205-2311
- Website: http://rarediseases.info.nih.gov/GARD/
Lymphatic Education & Research Network
- 261 Madison Avenue
- 9th Floor
- New York, NY 10016
- Tel: 516-625-9675
- Fax: 516-625-9410
- Email: LERN@LymphaticNetwork.org
- Website: http://www.lymphaticnetwork.org
March of Dimes Birth Defects Foundation
- 1275 Mamaroneck Avenue
- White Plains, NY 10605
- Tel: (914)997-4488
- Fax: (914)997-4763
- Website: http://www.marchofdimes.com
NIH/National Heart, Lung and Blood Institute
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National Lymphedema Network, Inc.
- 116 New Montgomery Street
- Suite 235
- San Francisco, CA 94105
- Tel: (415)908-3681
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- Website: http://www.lymphnet.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
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.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email firstname.lastname@example.org
Last Updated: 12/27/1969
Copyright 2012 National Organization for Rare Disorders, Inc.
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