Pulmonary Alveolar Proteinosis
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Pulmonary Alveolar Proteinosis 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.
The lung is composed of millions of tiny air sacs (alveoli) with very thin walls that allow oxygen in the air we breathe to pass through into the blood. Surfactant is a natural substance consisting of fat (mostly phospholipids) and a small amount of protein made in alveoli. Normally, a thin layer of surfactant present on the surface of alveoli helps them stay open. This allows air to come in and out as we breathe. Once used, surfactant is removed (cleared) from alveoli by cells called alveolar macrophages. This helps prevent surfactant from building up too much. Alveolar macrophages require a protein called granulocyte/macrophage-colony stimulating factor (GM-CSF) to maintain a normal surfactant level in alveoli (a process called surfactant homeostasis).
Pulmonary alveolar proteinosis (PAP) is a syndrome, a set of symptoms and signs and not just a single disease, in which surfactant slowly builds up in alveoli. This blocks air from entering alveoli and oxygen from passing through into the blood, which results in a feeling of breathlessness (dyspnea). Research has greatly improved our understanding of the diseases that cause PAP and how to identify (diagnose) and treat them. Diseases that cause PAP can occur in men, women, and children of all ages, ethnic backgrounds, and geographic locations. Disease severity varies from mild to severe and depends on which disease is present. Thus, it is important to know which disease is causing PAP in order to determine the best therapy and expected treatment response. Diseases that cause PAP can be grouped into three categories: primary PAP, secondary PAP, and congenital PAP (more accurately called disorders of surfactant production).
American Lung Association
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- Website: http://www.lungusa.org
British Paediatric Orphan Lung Disease
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- Website: http://www.bpold.co.uk
Genetic and Rare Diseases (GARD) Information Center
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NIH/National Heart, Lung and Blood Institute
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Rare Diseases Clinical Research Network
- University of South Florida, Pediatrics Epidemiology Center
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- Tel: (866)533-9104
- Email: RareDiseasesNetwork.org, RDCRN.org
- Website: http://rarediseasesnetwork.epi.usf.edu/registry/
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".
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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
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Last Updated: 1/14/1970
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