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Cystic Fibrosis

A Multi-Organ System Approach

  • Book
  • © 2020

Overview

Editors:
  1. Stephanie Duggins Davis

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

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  2. Margaret Rosenfeld

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

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  3. James Chmiel

    1. 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

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  • Details cystic fibrosis as a multisystem disease, not just a pulmonary one
  • Includes coverage of both pediatric and adult cases and the care considerations for each
  • Covers mental health, rheumatologic, and renal manifestations of cystic fibrosis
  • Written by leaders in the field

Part of the book series: Respiratory Medicine (RM)

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Table of contents (25 chapters)

  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, CĂ©line 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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Keywords

About this book

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.

Editors and Affiliations

  • 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

About the editors

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.

Bibliographic Information

  • Book Title: Cystic Fibrosis

  • Book Subtitle: A Multi-Organ System Approach

  • Editors: Stephanie Duggins Davis, Margaret Rosenfeld, James Chmiel

  • Series Title: Respiratory Medicine

  • DOI: https://doi.org/10.1007/978-3-030-42382-7

  • Publisher: Humana Cham

  • eBook Packages: Medicine, Medicine (R0)

  • Copyright Information: Springer Nature Switzerland AG 2020

  • Hardcover ISBN: 978-3-030-42381-0Published: 22 May 2020

  • Softcover ISBN: 978-3-030-42384-1Published: 22 May 2021

  • eBook ISBN: 978-3-030-42382-7Published: 21 May 2020

  • Series ISSN: 2197-7372

  • Series E-ISSN: 2197-7380

  • Edition Number: 1

  • Number of Pages: XVII, 526

  • Number of Illustrations: 10 b/w illustrations, 26 illustrations in colour

  • Topics: Pneumology/Respiratory System, Pediatrics

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