The median age of survival for those with cystic fibrosis has risen considerably in recent years. This text thoroughly examines the developments and breakthroughs which have led to this improvement in life expectancy. With a focus on the latest discoveries in the diagnosis and treatment of the disease, this book provides a comprehensive overview of

chapter 1|10 pages

The Genetics of Cystic Fibrosis

ByLaurence Suaud, Ronald C. Rubenstein

chapter 2|13 pages

Ion Transport

ByJames L. Kreindler, J. Kevin Foskett

chapter 3|12 pages

Mucus Abnormalities and Ciliary Dysfunction

ByA. Whitney Brown, Scott H. Donaldson

chapter 4|21 pages

Microbiology in Cystic Fibrosis

ByPaul J. Planet, Lisa Saiman

chapter 5|21 pages

Inflammation in the Cystic Fibrosis Lung

ByJames F. Chmiel, Michael W. Konstan

chapter 6|12 pages

Modifier Genes of Cystic Fibrosis

ByMitchell Drumm

chapter 7|13 pages

Cystic Fibrosis: Diagnosis, Sweat Testing, and Newborn Screening

ByBrian P. O’Sullivan

chapter 8|20 pages

Diagnostic Approach to Diseases Associated with Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations

ByChee Y. Ooi (Keith), Elizabeth Tullis, and Peter R. Durie

chapter 9|25 pages

Lung Function Testing in Infants

ByJessica E. Pittman, Stephanie D. Davis

chapter 10|13 pages

Assessment of Lung Function in Young Children with Cystic Fibrosis

ByFariba Rezaee, Clement L. Ren

chapter 11|21 pages

Lung Function Testing in School-Age Children with Cystic Fibrosis

ByOscar H. Mayer, Julian L. Allen

chapter 12|16 pages

Thoracic Imaging in Cystic Fibrosis Pulmonary Disease

ByMolly Raske, Alan S. Brody

chapter 13|26 pages

Pulmonary Manifestations

ByAndrew Bush

chapter 14|12 pages

Treatment Strategies for Maintaining Pulmonary Health in Cystic Fibrosis

BySusanna A. McColley

chapter 15|15 pages

Mucolytic Therapy and Airway Clearance Techniques

ByFelix Ratjen, Craig Lapin

chapter 16|15 pages

Pulmonary Exacerbations

ByDon B. Sanders, Margaret Rosenfeld

chapter 17|19 pages

Gastrointestinal Complications of Cystic Fibrosis

ByMaria Mascarenhas, Asim Maqbool

chapter 18|23 pages

Liver Disease

ByMeghana N. Sathe, Andrew P. Feranchak

chapter 19|20 pages


ByAsim Maqbool, Kelly Dougherty, Alisha J. Rovner

chapter 20|13 pages

Bone Health and Treatment

ByNadine G. Haddad and Linda A. DiMeglio

chapter 21|15 pages

Cystic Fibrosis–Related Diabetes and Management

ByAndrea Kelly, Andrew C. Calabria

chapter 22|16 pages

Other extrapulmonary Complications and Treatment

ByJoshua P. Needleman, Suzanne E. Beck

chapter 23|18 pages

Chronic Respiratory Failure and the Roles of Noninvasive Ventilation and Lung Transplantation

BySamuel Goldfarb, Howard B. Panitch

chapter 24|16 pages

Gene Repair: Past, Present, and Future

ByChristian Mueller, Terence R. Flotte

chapter 25|18 pages

Restoration of CFTR Function with Small-Molecule Modulators

ByWynton Hoover, John P. Clancy

chapter 26|16 pages

Quality IMprovement in Cystic Fibrosis Care

ByMichael S. Schechter

chapter 27|11 pages

Cystic Fibrosis and Infection Control

BySusan Rettig, Susan Coffin

chapter 28|6 pages

Transition to Adult Care

BySherstin G. Truitt, James R. Yankaskas

chapter 29|11 pages

Reproduction, Sexuality, and Fertility

ByLisa K. Tuchman, Ioanna K. Gisone

chapter 31|15 pages

Palliative and End-of-Life Care in Cystic Fibrosis

ByWalter M. Robinson, Jeffrey C. Klick