Cystic Fibrosis

This is the first source to cover the latest advances for treatment and earlier diagnosis of this chronic and potentially deadly disease. Specialists treating cystic fibrosis (CF) sufferers will find key information needed to improve and extend the lives of their patients. Clinicians need this book to: - note differences when testing lung function in children, adolescents, and adults - find the best pharmacologic treatment options to improve patient quality of life and consider therapies that target the gene mutation that causes CF - avoid and/or manage potential gastrointestinal, cardiac, and neurological complications, as well as the comorbidities of sleep disorders, liver disease, and diabetes - recognize care systems relevant to adult patients with CF   * Dr Julian Allen (Children's Hospital of Philadelphia, Philadelphia, PA, USA) * Dr Ronald Rubenstein (Children's Hospital of Philadelphia, Philadelphia, PA, USA) * Dr Howard Panitch (Children's Hospital of Philadelphia, Philadelphia, PA, USA)  CONTENTS Pathophysiology * Gene Abnormalities, Ronald Rubenstein * Ion Transport, Jim Kreindler * Mucus Abnormalities and Ciliary Dysfunction, Scott Donaldson * Microbiology, Lisa Saiman * Inflammation, Mike Konstan * Modifier Genes, Mitchell Drumm Diagnostics * Sweat Testing and Newborn Screening, Brian O’Sullivan * Diagnostic Approach (Including Nasal Potential Difference) to Non-classical CF and CFTR-opathies, Peter Durie * Lung Function Testing in Infants, Stephanie Davis * Lung function Testing in Preschool Children, Clement Ren * Lung Function in Older Children, Adolescents and Adults, O. Henry Mayer and Julian Allen * Imaging Studies, Alan Brody Clinical Manifestations and Treatment * Pulmonary Manifestations, Andrew Bush * Treatment Strategies for Maintaining Pulmonary Health in CF, Susanna McColley * Airway Clearance: RhDNAse, Inhaled Hypertonic Saline and Chest Physiotherapy, Craig Lapin * Pulmonary Exacerbations of CF and Treatment, Margaret Rosenfeld * Gastrointestinal Complications of CF, Maria Macarenhas * Liver Disease in CF, Drew Feranchak * Nutrition, Virginia Stallings * Bone Health and Treatment, Linda Dimeglio * CF Related Diabetes and Management, Andrea Kelly * Other Extra-Pulmonary Complications and Treatment: Infertility, Metabolic Alkalosis Cardiac, Neurological, Skin, and Sleep Disorders, Joshua Needleman * Chronic Respiratory Failure: Non-Invasive Ventilation and Lung Transplantation, Howard Panitch and Sam Goldfarb * Gene Repair: Present and Future, Terrence Flotte * CFTR Mutation Directed Therapies, John Paul (JP) Clancy Psychosocial Considerations and Care Systems, * CF Care Center Quality Assessment and Improvement, Michael Schecter * Infection Control, Susan Coffin * Transition to Adult Care, James Yankaskis * Reproduction and Sexuality, Lisa Tuchman * Quality of Life, Coping and Depression, Alexandra Quittner * Palliative Care, Walter Robinson and Jeffrey Klick