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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.
List of contents
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
About the author
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.
Summary
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.
