Cystic Fibrosis

Preface
Contributors
Pathophysiology
The Genetics of Cystic Fibrosis Laurence Suaud and Ronald C. Rubenstein
Ion Transport James L. Kreindler and J. Kevin Foskett
Mucus Abnormalities and Ciliary Dysfunction A. Whitney Brown and Scott H. Donaldson
Microbiology in Cystic Fibrosis Paul J. Planet and Lisa Saiman
Inflammation in the Cystic Fibrosis Lung James F. Chmiel and Michael W. Konstan
Modifier Genes of Cystic Fibrosis Mitchell Drumm

Diagnostics
Cystic Fibrosis: Diagnosis, Sweat Testing, and Newborn Screening Brian P. O’Sullivan
Diagnostic Approach to Diseases Associated with Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations Chee Y. Ooi (Keith), Elizabeth Tullis, and Peter R. Durie
Lung Function Testing in Infants Jessica E. Pittman and Stephanie D. Davis
Assessment of Lung Function in Young Children with Cystic Fibrosis Fariba Rezaee and Clement L. Ren
Lung Function Testing in School-Age Children with Cystic Fibrosis Oscar H. Mayer and Julian L. Allen
Thoracic Imaging in Cystic Fibrosis Pulmonary Disease Molly Raske and Alan S. Brody

Clinical Manifestations and Treatment
Pulmonary Manifestations Andrew Bush
Treatment Strategies for Maintaining Pulmonary Health in Cystic Fibrosis Susanna A. McColley
Mucolytic Therapy and Airway Clearance Techniques Felix Ratjen and Craig Lapin
Pulmonary Exacerbations Don B. Sanders and Margaret Rosenfeld
Gastrointestinal Complications of Cystic Fibrosis Maria Mascarenhas and Asim Maqbool
Liver Disease Meghana N. Sathe and Andrew P. Feranchak
Nutrition Asim Maqbool, Kelly Dougherty, Alisha J. Rovner, Suzanne H. Michel, and Virginia A. Stallings
Bone Health and Treatment Nadine G. Haddad and Linda A. DiMeglio
Cystic Fibrosis–Related Diabetes and Management Andrea Kelly and Andrew C. Calabria
Other Extrapulmonary Complications and Treatment Joshua P. Needleman and Suzanne E. Beck
Chronic Respiratory Failure and the Roles of Noninvasive Ventilation and Lung
Transplantation Samuel Goldfarb and Howard B. Panitch
Gene Repair: Past, Present, and Future Christian Mueller and Terence R. Flotte
Restoration of CFTR Function with Small-Molecule Modulators Wynton Hoover and John P. Clancy

Psychosocial Considerations and Care Systems
Quality Improvement in Cystic Fibrosis Care Michael S. Schechter
Cystic Fibrosis and Infection Control Susan Rettig and Susan Coffin
Transition to Adult Care Sherstin G. Truitt and James R. Yankaskas
Reproduction, Sexuality, and Fertility Lisa K. Tuchman and Ioanna K. Gisone
A Biopsychosocial Model of Cystic Fibrosis: Social and Emotional Functioning, Adherence, and Quality of Life David Barker and Alexandra L. Quittner
Palliative and End-of-Life Care in Cystic Fibrosis Walter M. Robinson and Jeffrey C. Klick
Index