Molecular Basis of Pulmonary Disease Insights from Rare Lung Disorders [Hardcover]

The study of rare lung disorders enhances our understanding of common pulmonary diseases such as fibrosis and emphysema. Molecular Basis of Lung Disease: Insights from Rare Disorders brings together a panel of distinguished clinicians and molecular scientists who are experts in a range of rare lung diseases and their underlying molecular defects. Each chapter focuses on the pathogenic mechanisms and therapeutic targets suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models, clinical presentation, diagnostic approaches, conventional management and treatment strategies, as well as future therapeutic targets and directions. Disorders ranging from the Marfan and Goodpastures syndromes to Sarcoidosis and alpha one titrypsin deficiency are treated in detail. Written for pulmonary clinicians and scientists alike, Molecular Basis of Lung Disease: Insights from Rare Disorders is a comprehensive and invaluable nesource that sheds new light on the molecular mechanisms influencing the clinical presentation and treatment strategies for these debilitating disorders.Chapter 1. A Clinical Approach to Rare Lung DiseasesRalph Panos, M.D.Chapter 2. Clinical Trials for Rare Lung DiseasesJeffrey Krischer, Ph.D.Chapter 3. Idiopathic and Familial Pulmonary Arterial HypertensionJean M. Elwing, M.D., Gail Deutsch, M.D., William C. Nichols, Ph.D., and Timothy LeCras, Ph.D.,Chapter 4. LymphangioleiomyomatosisFrancis X. McCormack, M.D, and Elizabeth P. Henske, M.D., Ph.D.Chapter 5. Autoimmune Pulmonary Alveolar ProteinosisBruce Trapnell, M.D., Koh Nakata, M.D., Ph.D., and Yoshikazu Inoue, M.D., Ph.D.Chapter 6. Mutations in Surfactant Protein C and Interstitial Lung DiseaseJames P. Bridges, Ph. D. and Ralph Panos, M.D.Chapter 7. Hereditary Hemorrhagic TelangiectasiaClaire Shovlin, M.D and S. Paul Oh, Ph.D.Chapter 8. Hermansky Pudlak SyndromeLisa Young, M.D. and Bill Gahl,ls4