Cystic Fibrosis

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.

• Preface; Pathophysiology; The Genetics of Cystic Fibrosis; Ion Transport; Mucus Abnormalities and Ciliary Dysfunction; Microbiology in Cystic Fibrosis; Inflammation in the Cystic Fibrosis Lung; Modifier Genes of Cystic Fibrosis; Diagnostics; Cystic Fibrosis: Diagnosis, Sweat Testing, and Newborn Screening; Diagnostic Approach to Diseases Associated with Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations; Lung Function Testing in Infants; Assessment of Lung Function in Young Children with Cystic Fibrosis; Lung Function Testing in School-Age Children with Cystic Fibrosis; Thoracic Imaging in Cystic Fibrosis Pulmonary Disease; Clinical Manifestations and Treatment; Pulmonary Manifestations; Treatment Strategies for Maintaining Pulmonary Health in Cystic Fibrosis; Mucolytic Therapy and Airway Clearance Techniques; Pulmonary Exacerbations; Gastrointestinal Complications of Cystic Fibrosis; Liver Disease; Nutrition; Bone Health and Treatment; Cystic Fibrosis-Related Diabetes and Management; Other Extrapulmonary Complications and Treatment; Chronic Respiratory Failure and the Roles of Noninvasive Ventilation and Lung Transplantation; Gene Repair: Past, Present, and Future; Restoration of CFTR Function with Small-Molecule Modulators; Psychosocial Considerations and Care Systems; Quality Improvement in Cystic Fibrosis Care; Cystic Fibrosis and Infection Control; Transition to Adult Care; Reproduction, Sexuality, and Fertility; A Biopsychosocial Model of Cystic Fibrosis: Social and Emotional Functioning, Adherence, and Quality of Life; Palliative and End-of-Life Care in Cystic Fibrosis