Translational research projects studying interstitial lung disease examine the pathogenesis of pulmonary fibrosis within subpopulations of patients, focussing on genetic effects. Diseases of interest include sporadic and genetic subtypes of pulmonary fibrosis. Thus far, at least two hundred patients have been enrolled in four clinical research protocols (i.e., 99-HL-0056, 99-HL-0068, 99-HL-0069, 04-H-0211). Analyses of patient-derived specimens (e.g., genomic DNA, serum, plasma, bronchoalveolar lavage cells and fluid, lung tissue) are being performed to study and compare genetic polymorphisms, gene and protein expression, and clinical findings (e.g., lung physiology, chest radiography, DTPA lung clearance scan). These pulmonary fibrosis protocols have generated collaborations with several intramural and extramural investigators. High-resolution computed tomography and aerosolized DTPA lung clearance scan findings are being examined by NIH Clinical Center researchers, Drs. Nilo Avila and Clara Chen, respectively. Dr. Ronald Goldstein, Boston University, and Dr. Ivan Rosas, University of Pittsburgh, will analyze biomarkers of fibrosis. Collaborations with Dr. Jonathan Orens, Johns Hopkins University, and Dr. Steven D.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Intramural Research (Z01)
Project #
1Z01HL002543-08
Application #
7321601
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
8
Fiscal Year
2006
Total Cost
Indirect Cost
Name
U.S. National Heart Lung and Blood Inst
Department
Type
DUNS #
City
State
Country
United States
Zip Code
Gahl, William A; Brantly, Mark; Troendle, James et al. (2002) Effect of pirfenidone on the pulmonary fibrosis of Hermansky-Pudlak syndrome. Mol Genet Metab 76:234-42
Gochuico, B R (2001) Potential pathogenesis and clinical aspects of pulmonary fibrosis associated with rheumatoid arthritis. Am J Med Sci 321:83-8