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Treatment of Cystic Fibrosis and Other Rare Lung Diseases

  • Book
  • © 2017

Overview

Editors:
  1. Arata Azuma

    1. Div of Pulmonary Medicine, Nippon Medical School Div of Pulmonary Medicine, Tokyo, Japan

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    You can also search for this editor in PubMed   Google Scholar

  2. Michael S. Schechter

    1. Children's Hospital of Richmond, Virginia Commonwealth University Children's Hospital of Richmond, Richmond, USA

    View editor publications

    You can also search for this editor in PubMed   Google Scholar

  • Offers up-to-date information on the research background and treatment of these pulmonary diseases
  • Explores basic molecular biological research as well as clinical applications
  • Primarily aimed at researchers and clinicians

Part of the book series: Milestones in Drug Therapy (MDT)

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Table of contents (13 chapters)

  1. Front Matter

    Pages i-viii
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  2. Etiopathology and Genetics of Rare Lung Diseases

    1. Front Matter

      Pages 1-1
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    2. An Introduction to Clinical Aspects of Cystic Fibrosis

      • Nauman Chaudary, Michael S. Schechter
      Pages 3-20

    3. Diffuse Panbronchiolitis

      • Naoto Keicho, Minako Hijikata
      Pages 21-38

    4. Idiopathic Pulmonary Fibrosis

      • Chiko Shimbori, Pierre-Simon Bellaye, Philipp Kolb, Martin Kolb
      Pages 39-69

    5. Pulmonary Alveolar Proteinosis: A Historic Perspective

      • Koh Nakata, Ryushi Tazawa
      Pages 71-86

    6. Lymphangioleiomyomatosis

      • Kuniaki Seyama
      Pages 87-98

  3. Treatment of Cystic Fibrosis

    1. Front Matter

      Pages 99-99
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    2. CFTR Modulator Therapies in Cystic Fibrosis

      • David R. Spielberg, John P. Clancy, Christopher Siracusa
      Pages 101-118

    3. Drug Therapies that Augment Airway Surface Liquid

      • Evangelia Daviskas, Sheila Sivam, Mark R. Elkins, Tiffany J. Dwyer, Ruth Dentice, Peter T. Bye
      Pages 119-138

    4. Anti-Inflammatory Therapies for Cystic Fibrosis

      • Elliott C. Dasenbrook, James F. Chmiel
      Pages 139-151

    5. Anti-Infective Therapies in Cystic Fibrosis

      • Patrick A. Flume, Donald R. VanDevanter
      Pages 153-169

  4. Treatment of Other Rare Lung Diseases

    1. Front Matter

      Pages 171-171
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    2. Diffuse Panbronchiolitis: Long-Term Low-Dose Macrolide Therapy

      • Mutsuo Yamaya, Arata Azuma, Shoji Kudoh
      Pages 173-188

    3. Idiopathic Pulmonary Fibrosis

      • Paolo Spagnolo
      Pages 189-210

    4. Treatment of Pulmonary Alveolar Proteinosis

      • Muhammad Muhye-ud-din Sheikh, Bruce C. Trapnell
      Pages 211-238

    5. Treatment of Lymphangioleiomyomatosis (LAM)

      • Mariam Anis, Francis X. McCormack
      Pages 239-263
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Keywords

About this book

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.



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)

                

Editors and Affiliations

  • Div of Pulmonary Medicine, Nippon Medical School Div of Pulmonary Medicine, Tokyo, Japan

    Arata Azuma

  • Children's Hospital of Richmond, Virginia Commonwealth University Children's Hospital of Richmond, Richmond, USA

    Michael S. Schechter

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