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Title Molecular basis of pulmonary disease : insights from rare lung disorders / edited by Francis X. McCormack, Ralph J. Panos, Bruce C. Trapnell
Published New York : Humana, ©2010

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Description 1 online resource (xi, 438 pages) : illustrations (some color)
Series Respiratory medicine (New York, N.Y.)
Respiratory medicine (New York, N.Y.)
Contents 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 haemorrhagic telangiectasia -- Hermansky-Pudlak Syndrome -- Alpha-1 antitrypsin deficiency -- Marfan syndrome -- Surfactant deficiency disorders: SP-B and ABCA3 -- Pulmonary capillary hemangiomatosis -- Anti-glomerular basement disease: Goodpasture's Syndrome -- Primary ciliary dyskinesia -- Pulmonary alveolar microlithiasis -- Cystic fibrosis -- Pulmonary Langerhans' cell histiocytosis: advances in the understanding of a true dendritic cell lung disease -- Sarcoidosis -- Scleroderma lung disease
Summary 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
Bibliography Includes bibliographical references and index
Notes Print version record
In Springer eBooks
Subject Lungs -- Diseases -- Molecular aspects
Lung Diseases -- genetics
Molecular Biology
MEDICAL -- Pulmonary & Thoracic Medicine.
Médecine.
Form Electronic book
Author McCormack, Francis X
Trapnell, Bruce C
Panos, Ralph J
ISBN 9781597453844
1597453846