Pulmonary Alveolar Proteinosis (PAP)
In this unusual disease of unknown cause, accumulation
of a viscous protein coating on the air sacs
(alveoli) of the lung progressively impairs the
exchange of oxygen and carbon dioxide.
Because symptoms may include a progressive decrease
in exercise tolerance, shortness of breath and
cough, Pulmonary Alveolar Proteinosis (PAP) can mimic many other lung diseases.
A diagnosis usually can be made with a combination
of tests, including radiographic lung images,
pulmonary function tests and bronchoscopy. A
surgical lung biopsy may sometimes be necessary
for definitive diagnosis.
The treatment of PAP involves cleansing the
lungs with a sterile salt solution (whole-lung
lavage) under general anesthesia. Fortunately,
the vast majority of patients obtain significant
improvement in symptoms after this therapy.
As the mid-Atlantic regional referral center
for this disease, Penn has performed whole-lung
lavage for more than 25 years and has a very
active research program for PAP. In addition
to establishing definitive diagnoses in difficult
cases and treating this disorder using whole-lung
lavage, our specialists are dedicated to improving
our understanding of the cause and development
of PAP.
Location
Hospital
of the University of Pennsylvania
3 Ravdin, Suite F
3400 Spruce Street
Philadelphia, PA 19104
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