Procurement and Analysis of Specimens From Individuals With Pulmonary Fibrosis

This study is currently recruiting participants. (see Contacts and Locations)
Verified May 2014 by National Institutes of Health Clinical Center (CC)
Sponsor:
Information provided by:
National Institutes of Health Clinical Center (CC)
ClinicalTrials.gov Identifier:
NCT00084305
First received: June 9, 2004
Last updated: June 21, 2014
Last verified: May 2014
  Purpose

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.


Condition
Pulmonary Fibrosis

Study Type: Observational
Official Title: Procurement and Analysis of Specimens From Individuals With Pulmonary Fibrosis

Resource links provided by NLM:


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

Estimated Enrollment: 500
Study Start Date: June 2004
Detailed Description:

The etiology of pulmonary fibrosis (PF) is unknown. Analyses 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 the pathogenic mechanisms of this disorder. The purpose of this protocol is to obtain blood, genomic DNA, and lung specimens from research subjects by bronchoscopy or lung biopsy, or lung tissue available either in the setting of 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 beobtained from relatives of subjects with familial pulmonary fibrosis or healthy research volunteers. Comparison of specimens from affected patients and healthy research volunteers will further the understanding of the pathogenesis of pulmonary fibrosis. Blood and lung specimens will be examined for genetic, molecular, and cellular abnormalities that may contribute to accumulation of extracellular matrix proteins and pneumocyte proliferation. In addition, genomic DNA will be procured and analyzed to investigate genotype/phenotype correlations.

  Eligibility

Ages Eligible for Study:   18 Years and older
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   Yes
Criteria
  • INCLUSION 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.

  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: NCT00084305

Contacts
Contact: Bernadette R Gochuico, M.D. (301) 451-7979 gochuicb@mail.nih.gov

Locations
United States, Maryland
National Institutes of Health Clinical Center, 9000 Rockville Pike Recruiting
Bethesda, Maryland, United States, 20892
Contact: For more information at the NIH Clinical Center contact Patient Recruitment and Public Liaison Office (PRPL)    800-411-1222 ext TTY8664111010    prpl@mail.cc.nih.gov   
Sponsors and Collaborators
Investigators
Principal Investigator: Bernadette R Gochuico, M.D. National Human Genome Research Institute (NHGRI)
  More Information

Additional Information:
Publications:
ClinicalTrials.gov Identifier: NCT00084305     History of Changes
Other Study ID Numbers: 040211, 04-HG-0211
Study First Received: June 9, 2004
Last Updated: June 21, 2014
Health Authority: United States: Federal Government

Keywords provided by National Institutes of Health Clinical Center (CC):
Bronchoscopy
Pulmonary Fibrosis
Lung Biopsy (Clinically-Indicated)
Hereditary Pulmonary Fibrosis
Healthy Volunteer
HV

Additional relevant MeSH terms:
Fibrosis
Pulmonary Fibrosis
Pathologic Processes
Lung Diseases
Respiratory Tract Diseases

ClinicalTrials.gov processed this record on August 19, 2014