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 Goodpasture's 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.

Comprehensive, state-of-the-art review of rare lung diseases 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 Includes supplementary material: sn.pub/extras

Inhalt
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 Haemorrhagic Telangiectasia.- HermanskyPudlak Syndrome.- Alpha-1 Antitrypsin Deficiency.- The 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.