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Treatment of Cystic Fibrosis and Other Rare Lung Diseases
English
381 b
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This volume describes the pathogenesis and pathophysiology of several§pulmonary diseases as well as their treatment. It also discusses the underlying§genetic and molecular biological basis, which opens the way for new treatments for§these conditions.§§It focuses on the treatment of cystic fibrosis including CFTR (cystic§fibrosis transmembrane-conductance regulator) modulator therapies, drug§therapies that augment airway surface liquid as well as anti-inflammatory and§anti-infective therapies. Further topics include long-term, low-dose macrolide§therapy for diffuse panbronchiolitis; novel agents for previously untreatable§idiopathic pulmonary fibrosis; possible new treatments for pulmonary alveolar§proteinosis (PAP); and multiple novel therapeutic targets for treating§lymphangiomyomatosis. Research into these conditions has led to major advances§in our understanding of the underlying genetic and molecular basis of this§disease, and to dramatic improvements in survival and quality of life for§affected individuals.§§
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