Part I. Etiopathology and Genetics of Rare Lung Diseases
1 An Introduction to Clinical Aspects of Cystic Fibrosis ....... 3
Nauman Chaudary and Michael S. Schechter
2 Diffuse Panbronchiolitis .................................... 21
Naoto Keicho and Minako Hijikata
3 Idiopathic Pulmonary Fibrosis ............................... 39
Chiko Shimbori, Pierre-Simon Bellaye, Philipp Kolb, and
Martin Kolb
4 Pulmonary Alveolar Proteinosis: A Historic Perspective ...... 71
Koh Nakata and Ryushi Tazawa
5 Lymphangioleiomyomatosis .................................... 87
Kuniaki Seyama
Part II. Treatment of Cystic Fibrosis
6 CFTR Modulator Therapies in Cystic Fibrosis ................ 101
David R. Spielberg, John P. Clancy, and Christopher
Siracusa
7 Drug Therapies that Augment Airway Surface Liquid .......... 119
Evangelia Daviskas, Sheila Sivam, Mark R. Elkins,
Tiffany J. Dwyer, Ruth Dentice, and Peter T. Bye
8 Anti-Inflammatory Therapies for Cystic Fibrosis ............ 139
Elliott C. Dasenbrook and James F. Chmiel
9 Anti-Infective Therapies in Cystic Fibrosis ................ 153
Patrick A. Flume and Donald R. VanDevanter
Part III. Treatment of Other Rare Lung Diseases
10 Diffuse Panbronchiolitis: Long-Term Low-Dose Macrolide
Therapy .................................................... 173
Mutsuo Yamaya, Arata Azuma, and Shoji Kudoh
11 Idiopathic Pulmonary Fibrosis .............................. 189
Paolo Spagnolo
12 Treatment of Pulmonary Alveolar Proteinosis ................ 211
Muhammad Muhye-ud-din Sheikh and Bruce C. Trapnell
13 Treatment of Lymphangioleiomyomatosis (LAM) ................ 239
Mariam Anis and Francis X. McCormack
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