Segmental Bronchoalveolar Lavage

This protocol proposes to perform bronchoalveolar lavage, bronchial brushing and bronchial
wall biopsy in research volunteers and in patients with pulmonary disease to evaluate the
cellular components of normal and diseased lungs. This research study will help to improve
our understanding of pathogenic mechanisms in the lung during progression of pulmonary
disease.

- INCLUSION CRITERIA:

Patients and research volunteers will be accepted for bronchoscopy and bronchoalveolar
lavage only if their heath status will not be compromised by the procedure. Examples of
disease that may be studied under this protocol include alpha-1 antitrypsin deficiency;
lymphangioleiomyomatosis and the cystic lung disease; pulmonary fibrosis; and sarcoidosis.

EXCLUSION CRITERIA:

Patients or research volunteers with abnormalities that contraindicate, or increase the
risk of, fiberoptic bronchoscopy and bronchoalveolar lavage, including a history of allergy
to atropine, lidocaine, or other topical anesthetics or premedications; FEV(1) less than
0.8 liters; PaO(2), on supplemental 02, of less than 70 mmHg or PaCO(2) greater than 45
mmHg; presence of uncorrected clotting disorder, uncontrolled hypertension, significant
cardiac disease, renal or liver failure; metastatic disease; hematologic disorders such as
severe anemia (hemoglobin less than or equal to 7 g/ml), granulocytopenia, or platelet
disorders.

Patients or research volunteers with a positive serum test for human immunodeficiency virus
or hepatitis B or C. The rationale for excluding participation on the basis of HIV
seropositivity is because of the known effects of HIV on the lungs.

Patients or research volunteers who are pregnant or lactating.

Patients or research volunteers incapable of giving informed consent.