544 Pages 100 B/W Illustrations
    by CRC Press

    544 Pages 100 B/W Illustrations
    by CRC Press

    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 the past, current and forthcoming advancements in cystic fibrosis research and clinical care.

    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

    Biography

    Julian L. Allen, Howard B. Panitch, Ronald C. Rubenstein, all from the University of Pennsylvania School of Medicine, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, U.S.A.