Characterization of the Pathobiology of Early Lung Destruction in Alpha 1-Antitrypsin Deficient Individuals
Data Collection
Digestive System Diseases+10
+ Liver Diseases
+ Lung Diseases
Summary
Study start date: May 1, 1995
Actual date on which the first participant was enrolled.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.
Protocol
This section provides details of the study plan, including how the study is designed and what the study is measuring.60 patients to be enrolled
Total number of participants that the clinical trial aims to recruit.Eligibility
Researchers look for people who fit a certain description, called eligibility criteria: person's general health condition or prior treatments.Any sex
Biological sex of participants that are eligible to enroll.Healthy volunteers allowed
If individuals who are healthy and do not have the condition being studied can participate.Conditions
Pathology
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.
Study Centers
These are the hospitals, clinics, or research facilities where the trial is being conducted. You can find the location closest to you and its status.This study has 1 location
National Heart, Lung and Blood Institute (NHLBI)
Bethesda, United StatesOpen National Heart, Lung and Blood Institute (NHLBI) in Google Maps