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

Tracking Information
First Received Date ^ICMJE	November 3, 1999
Last Updated Date	March 3, 2008
Start Date ^ICMJE	May 1995
Primary Completion Date
Current Primary Outcome Measures ^ICMJE
Original Primary Outcome Measures ^ICMJE
Change History	Complete list of historical versions of study NCT00001462 on ClinicalTrials.gov Archive Site
Current Secondary Outcome Measures ^ICMJE
Original Secondary Outcome Measures ^ICMJE

Descriptive Information
Brief Title ^ICMJE	Characterization of the Pathobiology of Early Lung Destruction in Alpha 1-Antitrypsin Deficient Individuals
Official Title ^ICMJE	Characterization of the Pathobiology of Early Lung Destruction in Alpha 1-Antitrypsin Deficient Individuals
Brief 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.
Detailed 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.
Study Phase
Study Type ^ICMJE	Observational
Study Design ^ICMJE
Condition ^ICMJE	Emphysema Lung Diseases, Obstructive Alpha 1-Antitrypsin Deficiency
Intervention ^ICMJE
Study Arms / Comparison Groups
Publications *	Buist AS. Alpha 1-antitrypsin deficiency in lung and liver disease. Hosp Pract (Off Ed). 1989 May 15;24(5):51-9. Review. No abstract available. 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. No abstract available. 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.
* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information
Recruitment Status ^ICMJE	Completed
Enrollment ^ICMJE	60
Completion Date	April 2000
Primary Completion Date
Eligibility Criteria ^ICMJE	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.
Gender	Both
Ages
Accepts Healthy Volunteers	Yes
Contacts ^ICMJE	Contact information is only displayed when the study is recruiting subjects
Location Countries ^ICMJE	United States

Administrative Information
NCT ID ^ICMJE	NCT00001462
Responsible Party
Study ID Numbers ^ICMJE	950116, 95-H-0116
Study Sponsor ^ICMJE	National Heart, Lung, and Blood Institute (NHLBI)
Collaborators ^ICMJE
Investigators ^ICMJE
Information Provided By	National Institutes of Health Clinical Center (CC)
Verification Date	August 1999
^ICMJE Data element required by the International Committee of Medical Journal Editors and the World Health Organization ICTRP