Alpha 1-antitrypsin-deficient individuals develop severe destructive lung disease much earlier and their lung function declines faster than the general population of individuals with chronic obstructive lung disease. This study is designed to better understand the pathogenesis of lung destruction in alpha 1-antitrypsin deficient individuals and to characterize the pathobiology of early lung destruction. To accomplish this we intend to use bronchoalveolar lavage to determine and quantify the factors that initiate and sustain lung inflammation in alpha 1-antitrypsin deficient individuals with lung function above a force expiratory volume in one second (FEV1) of greater than 50% of predicted.

Condition
Emphysema Lung Diseases, Obstructive alpha 1-Antitrypsin Deficiency

Further Study Details: 

Expected Total Enrollment:  60

Alpha 1-antitrypsin-deficient individuals develop severe destructive lung disease much earlier and their lung function declines faster than the general population of individuals with chronic obstructive lung disease. This study is designed to better understand the pathogenesis of lung destruction in alpha 1-antitrypsin deficient individuals and to characterize the pathobiology of early lung destruction. To accomplish this we intend to use bronchoalveolar lavage to determine and quantify the factors that initiate and sustain lung inflammation in alpha 1-antitrypsin deficient individuals with lung function above a force expiratory volume in one second (FEV1) of greater than 50% of predicted.

Genders Eligible for Study:  Both

Accepts Healthy Volunteers

Criteria

Any alpha 1 antitrypsin-deficient individuals.
18-65 years old.
FEV1 greater than 1 equal to 50 percent of predicted (forced expiratory volume).
Study participation is required for one year.
A total of four bronchoscopies will be performed over a year period.
Methacholine challenge test will be performed at the beginning and end of the study to assess the degree of reactive airways disease.
Pneumococcal and annual influenza vaccine will be given.
No prolastin within one year prior to start of the study.
No oral systemic corticosteroids within 30 days prior to start of study.
No allergy to topical or local anesthetic (i.e., lidocaine).
No pregnancy.
No HIV positive patients.
No Hepatitis B/C virus positive patients.
No patients with any condition associated with immunodeficiency.
No patients with presence of significant cardiac diseases.
No patients with presence of uncorrected blood-clotting disorders.
No patients with any oxygen at home on a regular basis.
No adverse reactions to methacholine.

Maryland
      National Heart, Lung and Blood Institute (NHLBI), 9000 Rockville Pike,  Bethesda,  Maryland,  20892,  United States

Study ID Numbers:  950116; 95-H-0116
Record last reviewed:  August 3, 1999
Last Updated:  December 11, 2002
Record first received:  November 3, 1999
ClinicalTrials.gov Identifier:  NCT00001462
Health Authority: United States: Federal Government
ClinicalTrials.gov processed this record on 2005-04-08