Characterization of the Pathobiology of Early Lung Destruction in Alpha 1-Antitrypsin Deficient Individuals
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Purpose
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 |
|---|
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Emphysema Lung Diseases, Obstructive Alpha 1-Antitrypsin Deficiency |
| Study Type: | Observational |
| Official Title: | Characterization of the Pathobiology of Early Lung Destruction in Alpha 1-Antitrypsin Deficient Individuals |
| Estimated Enrollment: | 60 |
| Study Start Date: | May 1995 |
| Estimated Study Completion Date: | April 2000 |
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.
Eligibility| Genders Eligible for Study: | Both |
| Accepts Healthy Volunteers: | Yes |
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.
Contacts and Locations More Information
Publications:
| ClinicalTrials.gov Identifier: | NCT00001462 History of Changes |
| Other Study ID Numbers: | 950116, 95-H-0116 |
| Study First Received: | November 3, 1999 |
| Last Updated: | March 3, 2008 |
| Health Authority: | United States: Federal Government |
Keywords provided by National Institutes of Health Clinical Center (CC):
|
Alpha 1-Antitrypsin Deficiency Emphysema Genetic Diseases |
Inflammation Nitric Oxide Protease |
Additional relevant MeSH terms:
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Emphysema Pulmonary Emphysema Lung Diseases Alpha 1-Antitrypsin Deficiency Lung Diseases, Obstructive Pathologic Processes Respiratory Tract Diseases Liver Diseases Digestive System Diseases Genetic Diseases, Inborn |
Subcutaneous Emphysema Alpha 1-Antitrypsin Protein C Inhibitor Trypsin Inhibitors Serine Proteinase Inhibitors Protease Inhibitors Enzyme Inhibitors Molecular Mechanisms of Pharmacological Action Pharmacologic Actions |
ClinicalTrials.gov processed this record on May 16, 2013