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

This study has been completed.
Sponsor:
Information provided by:
National Institutes of Health Clinical Center (CC)
ClinicalTrials.gov Identifier:
NCT00001462
First received: November 3, 1999
Last updated: March 3, 2008
Last verified: August 1999
  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
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

Resource links provided by NLM:


Further study details as provided by National Institutes of Health Clinical Center (CC):

Estimated Enrollment: 60
Study Start Date: May 1995
Estimated Study Completion Date: April 2000
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.

  Eligibility

Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   Yes
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.

  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT00001462

Locations
United States, Maryland
National Heart, Lung and Blood Institute (NHLBI)
Bethesda, Maryland, United States, 20892
Sponsors and Collaborators
  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:
Lung Diseases
Emphysema
Alpha 1-Antitrypsin Deficiency
Lung Diseases, Obstructive
Respiratory Tract Diseases
Pathologic Processes
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 September 22, 2014