Molecular Basis of Pulmonary Disease Insights from Rare Lung Disorders /

The study of rare lung disorders enhances our understanding of common pulmonary diseases such as fibrosis and emphysema. Molecular Basis of Pulmonary Disease: Insights from Rare Lung Disorders brings together a panel of distinguished clinicians and molecular scientists who are experts in a range of...

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Bibliographic Details
Corporate Author: SpringerLink (Online service)
Other Authors: McCormack, Francis X. (Editor), Panos, Ralph J. (Editor), Trapnell, Bruce C. (Editor)
Format: Electronic
Language:English
Published: Totowa, NJ : Humana Press, 2010.
Series:Respiratory Medicine
Subjects:
Online Access:https://ezaccess.library.uitm.edu.my/login?url=http://dx.doi.org/10.1007/978-1-59745-384-4
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505 0 # |a A Clinical Approach to Rare Lung Diseases -- Clinical Trials for Rare Lung Diseases -- Idiopathic and Familial Pulmonary Arterial Hypertension -- Lymphangioleiomyomatosis -- Autoimmune Pulmonary Alveolar Proteinosis -- Mutations in Surfactant Protein C and Interstitial Lung Disease -- Hereditary Hemorrhagic Telangiectasia -- Hermansky Pudlak Syndrome -- Alpha One Antitrypsin Deficiency -- The Marfan Syndrome -- Surfactant Deficiency Disorders SP-B and ABCA3 -- Pulmonary Capillary Hemangiomatosis -- Goodpastures Syndrome -- Primary Ciliary Diskinesia -- Pulmonary Alveolar Microlithiasis -- Cystic Fibrosis -- Pulmonary Langerhans Cell Histiocytosis -- Sarcoidosis -- Scleroderma Lung Disease. 
520 # # |a The study of rare lung disorders enhances our understanding of common pulmonary diseases such as fibrosis and emphysema. Molecular Basis of Pulmonary Disease: Insights from Rare Lung 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 Goodpasture s syndromes to Sarcoidosis and alpha one antitrypsin deficiency are treated in detail. Written for pulmonary clinicians and scientists alike, Molecular Basis of Pulmonary Disease: Insights from Rare Lung 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. 
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