Treatment of Cystic Fibrosis and Other Rare Lung Diseases (2017) (PDF) by Arata Azuma

Description

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.

User’s Reviews

“The purpose is to focus on recent advances in understanding the molecular basis of these diseases, and how this understanding has helped develop novel targeted therapies. … The audience is clinicians, students, and researchers who want a deeper understanding of the pathogenesis and recent advances in the therapy of these rare diseases. … It should serve as a useful addition to the personal library of any interested pulmonologist or lung disease researcher.” (Santosh Dhungana, Doody’s Book Reviews, April, 2017)

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