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

Study Overview
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Brief Summary
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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
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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.

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

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

Participation Criteria
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Eligibility Criteria
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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.
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Alpha 1-Antitrypsin Deficiency, Emphysema, Genetic Diseases, Inflammation, Nitric Oxide, Protease