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Alternative names Return to top
Alveolar proteinosisDefinition Return to top
Pulmonary 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 Return to top
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.
Symptoms Return to top
Note: There may be no symptoms.Signs and tests Return to top
The health care provider may hear crackles in the lungs. Often, the physical examination is normal.Treatment Return to top
Treatment 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) Return to top
Spontaneous remission occurs in some, while progressive respiratory failure develops in others.Calling your health care provider Return to top
Call 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: 5/7/2003 Updated by: David A. Kaufman, M.D., Pulmonary & Critical Care Medicine, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |