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 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, aswell as future therapeutic targets and directions. Disorders ranging from theMarfan 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 Lung Disease: Insights from Rare Disorders is a comprehensive and invaluable resource that sheds new light on the molecular mechanisms influencing the clinical presentation and treatment strategies for these debilitating disorders. Comprehensive, state-of-the-art review of rare lungdiseases Thoroughly sheds light on the molecular mechanisms that evoke the clinical presentation and engender treatment strategies for each rare lung disease Covers all aspects of such disorders as pulmonary alveolar proteinosis (PAP), pulmonary alveolar microlithiasis (PAM), inherited disorders of surfactant metabolism, and pulmonary arterial hypertension, to name just several Chaptersauthored by a combined clinician and basic scientist team approach, providinga synthesis of expertise in each disease topic and underlying molecular defect Invaluable resource for pulmonary clinicians and scientists alike INDICE: 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.

• ISBN: 978-1-58829-963-5
• Editorial: Humana
• Encuadernacion: Cartoné
• Páginas: 434
• Fecha Publicación: 01/04/2010
• Nº Volúmenes: 1
• Idioma: Inglés