Respiratory Medicine
Series Editors
Sharon I. S. Rounds
Alpert Medical School of Brown University, Providence, RI, USA
Anne Dixon
University of Vermont, Larner College of Medicine, Burlington, VT, USA
Lynn M. Schnapp
University of Wisconsin - Madison, Madison, WI, USA
More information about this series at http://www.springer.com/series/7665 Respiratory Medicine offers clinical and research-oriented resources for pulmonologists and other practitioners and researchers interested in respiratory care. Spanning a broad range of clinical and research issues in respiratory medicine, the series covers such topics as COPD, asthma and allergy, pulmonary problems in pregnancy, molecular basis of lung disease, sleep disordered breathing, and others. The series editors are Sharon Rounds, MD, Professor of Medicine and of Pathology and Laboratory Medicine at the Alpert Medical School at Brown University, Anne Dixon, MD, Professor of Medicine and Director of the Division of Pulmonary and Critical Care at Robert Larner, MD College of Medicine at the University of Vermont, and Lynn M. Schnapp, MD, George R. And Elaine Love Professor and Chair of Medicine at the University of Wisconsin-Madison School of Medicine and Public Health.
Editors
Stephanie Duggins Davis , Margaret Rosenfeld and James Chmiel
Cystic Fibrosis
A Multi-Organ System Approach
Editors
Stephanie Duggins Davis
The University of North Carolina at Chapel Hill, Department of Pediatrics, UNC Childrens Hospital, Chapel Hill, NC, USA
Margaret Rosenfeld
Department of Pediatrics, University of Washington School of Medicine, Division of Pulmonary and Sleep Medicine Seattle Childrens Hospital, Seattle, WA, USA
James Chmiel
Department of Pediatrics, Indiana University School of Medicine, Division of Pediatric Pulmonology, Allergy and Sleep Medicine, Riley Hospital for Children at IU Health, Indianapolis, IN, USA
ISSN 2197-7372 e-ISSN 2197-7380
Respiratory Medicine
ISBN 978-3-030-42381-0 e-ISBN 978-3-030-42382-7
https://doi.org/10.1007/978-3-030-42382-7
Springer Nature Switzerland AG 2020
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Preface
This is an exciting time in the care of people with cystic fibrosis (CF). There are now more adults than children living with CF, and highly effective modulator therapies are poised to dramatically improve outcomes. We offer this book to medical students, residents, and seasoned clinicians wishing for state-of-the-art reviews on all aspects of CF care. Unique features of the book include patients and parents perspectives in many of the chapters, as well as chapters on health disparities and mental health. The book covers major pulmonary and non-pulmonary manifestations of CF as well as advanced lung disease and lung transplantation. There is also a section on the molecular genetics of CF and treatment of the basic defect. Because many excellent reviews on CFTR modulators are readily available, this section covers gene editing and small molecules instead. We dedicate this book to our patients and their families who have inspired and taught us so much.
Stephanie Duggins Davis
Margaret Rosenfeld
James Chmiel
Chapel Hill, NC, USA Seattle, WA, USA Indianapolis, IN, USA
Acknowledgments
I am truly grateful for Sam, Harrison, Jackson, and Parker for their incredible love and support.
-Stephanie Duggins Davis
To my patients for helping me to understand life with CF, and to my colleagues and family for their enduring support.
-Margaret Rosenfeld
To Jennifer, Abigail, James, Michael, my siblings, and my parents for all of the love and support.
-James Chmiel
Contents
Part IIntroduction
Jonathan D. Cogen and Bonnie W. Ramsey
Caroline S. Thomas and Clement L. Ren
Gabriela R. Oates and Michael S. Schechter
Part IIPulmonary Manifestations
Sarath C. Ranganathan
Valerie J. Waters and John J. LiPuma
Michel Mac Aogin , Cline Vidaillac and Sanjay H. Chotirmall
Thomas Ruffles and Claire Wainwright
Deepika Polineni , Dave Nichols and Alex H. Gifford
Kristina Montemayor , Allison A. Lambert and Natalie E. West
Shruti M. Paranjape and Peter J. Mogayzel Jr
Bryan Garcia , Jessica Mattson and Patrick A. Flume
Joseph M. Pilewski
Amar Mandalia and Matthew J. DiMagno
Part IIIGastrointestinal Manifestations
Anna Bertolini , Frank A. J. A. Bodewes , Mordechai Slae and Michael Wilschanski
Adam C. Stein , Nicole Green and Sarah Jane Schwarzenberg
Part IVEndocrine Manifestations
Andrea Granados and Kara S. Hughan
Sophie Gurin , Isabelle Durieu and Isabelle Sermet-Gaudelus
Amanda Reis and Andrew J. White
Andrew Prayle and Bradley S. Quon
Erin Crowley and Cynthia D. Brown
Kathryn L. Behrhorst , Robin S. Everhart and Michael S. Schechter
Emily F. Muther , Jennifer L. Butcher and Kristin A. Riekert
Sangwoo T. Han and Garry R. Cutting
Part VTherapies Directed at the Basic Defect
Jennifer S. Guimbellot and Steven M. Rowe
Mitchell L. Drumm
Contributors
Michel Mac Aogin BSc, PhD
Translational Respiratory Research Laboratory, Lee Kong Chian School of Medicine, Nanyang Technological University, Singapore, Singapore
Kathryn L. Behrhorst MS
Virginia Commonwealth University, Department of Psychology, Richmond, VA, USA