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This volume describes the pathogenesis and pathophysiology of severalpulmonary diseases as well as their treatment. It also discusses the underlyinggenetic and molecular biological basis, which opens the way for new treatments forthese conditions.
It focuses on the treatment of cystic fibrosis including CFTR (cysticfibrosis transmembrane-conductance regulator) modulator therapies, drugtherapies that augment airway surface liquid as well as anti-inflammatory andanti-infective therapies. Further topics include long-term, low-dose macrolidetherapy for diffuse panbronchiolitis; novel agents for previously untreatableidiopathic pulmonary fibrosis; possible new treatments for pulmonary alveolarproteinosis (PAP); and multiple novel therapeutic targets for treatinglymphangiomyomatosis. Research into these conditions has led to major advancesin our understanding of the underlying genetic and molecular basis of thisdisease, and to dramatic improvements in survival and quality of life foraffected individuals.