Cystic Fibrosis

1. Front Matter

   Pages i-xvii

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2. Introduction

   1. Front Matter

      Pages 1-1

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   2. The Changing Face of Cystic Fibrosis

      • Jonathan D. Cogen, Bonnie W. Ramsey

      Pages 3-16

   3. Diagnosis of Cystic Fibrosis

      • Caroline S. Thomas, Clement L. Ren

      Pages 17-33

   4. Health Disparities

      • Gabriela R. Oates, Michael S. Schechter

      Pages 35-55

3. Pulmonary Manifestations

   1. Front Matter

      Pages 57-57

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   2. Early Cystic Fibrosis Lung Disease

      • Sarath C. Ranganathan

      Pages 59-72

   3. Bacterial Infections and the Respiratory Microbiome

      • Valerie J. Waters, John J. LiPuma

      Pages 73-92

   4. Fungal Infections and ABPA

      • Micheál Mac Aogáin, Céline Vidaillac, Sanjay H. Chotirmall

      Pages 93-126

   5. Nontuberculous Mycobacterium

      • Thomas Ruffles, Claire Wainwright

      Pages 127-160

   6. Inflammation in CF: Key Characteristics and Therapeutic Discovery

      • Deepika Polineni, Dave Nichols, Alex H. Gifford

      Pages 161-179

   7. Pulmonary Exacerbations

      • Kristina Montemayor, Allison A. Lambert, Natalie E. West

      Pages 181-198

   8. Maintenance of Pulmonary Therapies

      • Shruti M. Paranjape, Peter J. Mogayzel Jr

      Pages 199-213

   9. Advanced Stage Lung Disease

      • Bryan Garcia, Jessica Mattson, Patrick A. Flume

      Pages 215-227

   10. Lung Transplantation for Cystic Fibrosis

       • Joseph M. Pilewski

       Pages 229-258

4. Gastrointestinal Manifestations

   1. Front Matter

      Pages 259-259

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   2. Exocrine Pancreatic Insufficiency and Nutritional Complications

      • Amar Mandalia, Matthew J. DiMagno

      Pages 261-298

   3. Hepatobiliary Involvement in Cystic Fibrosis

      • Anna Bertolini, Frank A. J. A. Bodewes, Mordechai Slae, Michael Wilschanski

      Pages 299-320

   4. Gastrointestinal Complications

      • Adam C. Stein, Nicole Green, Sarah Jane Schwarzenberg

      Pages 321-334

5. Other Organ System Manifestations

   1. Front Matter

      Pages 335-335

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This book provides a comprehensive overview of the multisystem disease, cystic fibrosis, for both pediatric and adult patients.  Written by experts in the field, the text outlines the progressive nature of CF as well as the impact of this autosomal recessive disease on the respiratory, gastrointestinal, endocrine, rheumatologic, and renal systems, as well as the patient’s mental health.  The book begins with a chapter describing the history of cystic fibrosis and how the face of this life-shortening disease has changed over the past several decades.  The following chapters elucidate the pathophysiology of how cystic fibrosis impacts each organ system.  Current management and therapeutics are detailed with step-by-step guidelines for clinicians.  This book is unique in that it highlights the entire person, not just the respiratory system, with detailed inclusion of the patient perspectives throughout, informing practice standards and considerations. This is an ideal guide for pediatric and adult physicians who care for patients with cystic fibrosis, as well as respiratory therapists, physical therapists, nurses, nutritionists, and pharmacists who care for these patients.

• cystic fibrosis
• CF
• Respiratory disease
• multisystem disease
• pancreatic insufficiency
• depression
• diabetes

• The University of North Carolina at Chapel Hill, Department of Pediatrics, UNC Children’s Hospital, Chapel Hill, USA

  Stephanie Duggins Davis

• Department of Pediatrics, University of Washington School of Medicine, Division of Pulmonary and Sleep Medicine, Seattle Children’s Hospital, Seattle, USA

  Margaret Rosenfeld

• Department of Pediatrics, Indiana University School of Medicine, Division of Pediatric Pulmonology, Allergy and Sleep Medicine, Riley Hospital for Children at IU Health, Indianapolis, USA

  James Chmiel

Stephanie D Davis, MD is Professor of Pediatrics, Director of Pediatric Pulmonology, Allergy and Sleep Medicine, and Vice Chair of Clinical Affairs in the Department of Pediatrics at Indiana University School of Medicine. She is volume editor of another series book, Diagnostic Tests in Pediatric Pulmonology (2015).

Margaret Rosenfeld, MD, MPH is Professor of Pediatrics in the Division of Pulmonary Medicine and Associate Director of the Center for Clinical and Translational Research at Seattle Children’s Hospital.

James Chmiel, MD, MPH is Professor of Pediatrics in the Division of Pediatric Pulmonology, Allergy/Immunology and Sleep Medicine, Director of the Cystic Fibrosis Therapeutics Development Center, Associate Center Director of the LeRoy W. Matthews Cystic Fibrosis Center, and Director of the Pediatric Cystic Fibrosis Program at Case Western Reserve University School of Medicine and Rainbow Babies and Children’s Hospital.

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