1. The role of genetics and cigarette smoking in the development of pulmonary fibrosis
- Author
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Aretha Herron, Thomas A. Sporn, Momen M. Wahidi, Susan H. Slifer, Marcy C. Speer, H P McAdams, Kevin K. Brown, Marvin I. Schwarz, Lauranell H. Burch, John Phillips, David A. Schwartz, Jim C. Loyd, and Mark P. Steele
- Subjects
Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Pathology ,Genetic heterogeneity ,business.industry ,Autopsy ,Disease ,Environmental exposure ,medicine.disease ,DLCO ,Internal medicine ,Pulmonary fibrosis ,medicine ,Sibling ,business ,Idiopathic interstitial pneumonia - Abstract
Several lines of evidence suggest that genetic factors and environmental exposure play a role in the development of pulmonary fibrosis. The authors evaluated families with familial pulmonary fibrosis (FPF), defined as the presence of two or more cases of idiopathic interstitial pneumonia (IIP) in first degree family members. In total, 56 families with FPF were evaluated with 193 affected and 196 unaffected individuals. The presence of FPF was based on clinical history, DLCO, and CXR in 27 (14%); clinical history and HRCT scan in 102 (53%); clinical history and SLB in 55 (28%); and clinical history and autopsy in nine (5%). Older age (67.8 vs. 53.6; P
- Published
- 2006
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