Characterization of the Pathobiology of Early Lung Destruction in Alpha 1-Antitrypsin Deficient Individuals

Emphysema Clinical Trial

Official title:

Characterization of the Pathobiology of Early Lung Destruction in Alpha 1-Antitrypsin Deficient Individuals

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT00001462
• Other study ID #: 950116
• Secondary ID: 95-H-0116
• Status: Completed
• Phase: N/A
• First received: November 3, 1999
• Last updated: March 3, 2008
• Start date: May 1995
• Est. completion date: April 2000

Study information

• Verified date: August 1999
• Source: National Institutes of Health Clinical Center (CC)
• Contact: n/a
• Is FDA regulated: No
• Health authority: United States: Federal Government
• Study type: Observational

Clinical Trial Summary

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.

Description:

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 60
• Est. completion date: April 2000
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: Both
• Age group: N/A and older

Eligibility

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.

Study Design

N/A

Related Conditions & MeSH terms

• Alpha 1-Antitrypsin Deficiency
• Emphysema
• Lung Diseases
• Lung Diseases, Obstructive

Locations

Country	Name	City	State
United States	National Heart, Lung and Blood Institute (NHLBI)	Bethesda	Maryland

Sponsors (1)

Lead Sponsor	Collaborator
National Heart, Lung, and Blood Institute (NHLBI)

Country where clinical trial is conducted

United States, 

References & Publications (3)

Buist AS. Alpha 1-antitrypsin deficiency in lung and liver disease. Hosp Pract (Off Ed). 1989 May 15;24(5):51-9. Review. — View Citation

Crystal RG. Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy. J Clin Invest. 1990 May;85(5):1343-52. Review. — View Citation

Gadek JE, Pacht ER. The protease-antiprotease balance within the human lung: implications for the pathogenesis of emphysema. Lung. 1990;168 Suppl:552-64. Review. — View Citation