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Brief Title † | Specimen Procurement From People With Pulmonary Fibrosis | ||||||||
Official Title † | Specimen Procurement From Individuals With Pulmonary Fibrosis | ||||||||
Brief Summary | This study will collect tissue specimens from healthy subjects and from patients with hereditary forms of pulmonary fibrosis (lung scarring) to learn about substances in the blood, urine, or lung that might change as a result of this disease. Cells and fluid from the blood, urine, and lung will be analyzed for measurements of genetic material and proteins. Healthy volunteers, patients with Hermansky-Pudlak syndrome or familial pulmonary fibrosis, and relatives of patients with hereditary pulmonary fibrosis who are 18 years of age or older may be eligible for this study. Participants are admitted to the hospital for 3 to 7 days for the following tests and procedures:
Subjects undergo these tests to determine their level of lung inflammation. Before the procedures begin, the subject's mouth, nose and throat are numbed with an anesthetic. Drugs are given to reduce mouth secretions, coughing and wheezing. A pain reliever and sedative may also be given. A thin tube (bronchoscope) is passed through the nose or mouth into the bronchi (large breathing tubes) of the lungs. A salt-water solution is infused and then suctioned out. A small brush may be passed through the tube to brush an area of the airway wall to collect some cells. Then the bronchoscope is removed and the procedure is finished. Oxygen is administered and an EKG monitors heart function throughout the procedure. The procedure takes 20 to 40 minutes. Patients are observed overnight in the hospital. Patients with Hermansky-Pudlak syndrome may receive the drug desmopressin before the procedure to reduce the chance of bleeding.
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Detailed Description | The etiology of pulmonary fibrosis is unknown. Analysis of blood, genomic DNA, and specimens procured by bronchoscopy, lung biopsy, lung transplantation, or post-mortem examination from individuals with this disorder may contribute to our understanding of pathogenic mechanisms of pulmonary fibrosis. The purpose of this protocol is to obtain blood, genomic DNA, and specimens by bronchoscopy, lung biopsy, lung transplantation, or post-mortem examination from subjects with pulmonary fibrosis. In addition, blood, genomic DNA, as well as bronchoscopy and post-mortem examination specimens may be obtained from relatives of subjects with familial pulmonary fibrosis or healthy research volunteers. |
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Study Phase | |||||||||
Study Type † | Observational | ||||||||
Study Design † | |||||||||
Primary Outcome Measure † | |||||||||
Secondary Outcome Measure † | |||||||||
Condition † | Pulmonary Fibrosis | ||||||||
Intervention † | |||||||||
MEDLINE PMIDs | 9562579, 8145011, 9787100 | ||||||||
Links | NIH Clinical Center Detailed Web Page  | ||||||||
Recruitment Information Fields | |||||||||
Recruitment Status † | Recruiting | ||||||||
Enrollment † | 500 | ||||||||
Start Date † | June 2004 | ||||||||
Completion Date | |||||||||
Eligibility Criteria † |
Individuals who are 18 years of age or older with any of the following: Idiopathic pulmonary fibrosis (defined by either an open lung biopsy demonstrating pulmonary fibrosis and/or HRCT scan findings consistent with idiopathic pulmonary fibrosis as outlined by the American Thoracic Society/European Respiratory Society guidelines), Familial pulmonary fibrosis (defined as idiopathic pulmonary fibrosis in two or more first-degree relatives) Relatives of patients with hereditary pulmonary fibrosis, Hermansky-Pudlak syndrome (diagnosed by paucity or deficiency of platelet dense bodies on whole mount electron microscopy), Pulmonary fibrosis associated with rheumatoid arthritis [defined by 1987 American College of Rheumatology Revised Criteria for the Classification of RA], or Healthy research volunteers by history and indicated tests (individuals without history of chronic pulmonary disorder, collagen vascular disease, or bleeding disorder). EXCLUSION CRITERIA: Individuals with any of the following: Significant Inhalational exposure to fibrogenic fibers or dusts (i.e., asbestos, silica, coal, beryllium) or exposure to drugs associated with pulmonary fibrosis, Uncontrolled ischemic heart disease, Other collagen vascular disorders (i.e. systemic lupus erythematosus, scleroderma, polymyositis, mixed connective tissue disease), Uncontrolled ischemic heart disease Other collagen vascular disorders (i.e., systemic lupus erythematosus, scleroderma, polymyositis, mixed connective tissue disease) Uncorrectable bleeding diathesis, Pregnancy or lactation, or Inability to give informed consent. |
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Gender | Both | ||||||||
Ages | 18 Years and older | ||||||||
Accepts Healthy Volunteers | Yes | ||||||||
Contacts †† |
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Location Countries † | United States | ||||||||
Administrative Information Fields | |||||||||
NCT ID † | NCT00084305 | ||||||||
Organization ID | 040211 | ||||||||
Secondary IDs †† | 04-HG-0211 | ||||||||
Study Sponsor † | National Human Genome Research Institute (NHGRI) | ||||||||
Collaborators †† | |||||||||
Investigators † | |||||||||
Information Provided By | National Institutes of Health Clinical Center (CC) | ||||||||
Verification Date | December 2008 | ||||||||
First Received Date † | June 9, 2004 | ||||||||
Last Updated Date | December 23, 2008 |