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Pulmonary alveolar proteinosis
Alternative namesAlveolar proteinosis
DefinitionPulmonary alveolar proteinosis is a disease in which a phospholipid (a compound widely distributed in living cells) accumulates within spaces in the lungs where oxygen and carbon dioxide are exchanged.
Causes, incidence, and risk factors
In some cases, the cause of pulmonary alveolar proteinosis is unknown. In others, it is associated with infection or immune deficiency.
This rare disorder generally affects people 30 to 50 years old and is seen in men more often than in women.
Signs and testsThe health care provider may hear crackles in the lungs. Often, the physical examination is normal.
TreatmentTreatment consists of periodic whole-lung lavage, a washing out of the phospholipids from the lung, performed by specially trained physicians. Lung transplantation has been performed for this disease as well.
Expectations (prognosis)Spontaneous remission occurs in some, while progressive respiratory failure develops in others.
Calling your health care providerCall your health care provider if symptoms develop. Progressive worsening of shortness of breath indicates that an urgent or emergency condition may be developing.
Update Date: 5/7/2003David A. Kaufman, M.D., Pulmonary & Critical Care Medicine, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT