This book provides a comprehensive overview of the multisystem disease, cystic fibrosis, for both pediatric and adult patients. Written by experts in the field, the text outlines the progressive nature of CF as well as the impact of this autosomal recessive disease on the respiratory, gastrointestinal, endocrine, rheumatologic, and renal systems, as well as the patient's mental health. The book begins with a chapter describing the history of cystic fibrosis and how the face of this life-shortening disease has changed over the past several decades. The following chapters elucidate the pathophysiology of how cystic fibrosis impacts each organ system. Current management and therapeutics are detailed with step-by-step guidelines for clinicians. This book is unique in that it highlights the entire person, not just the respiratory system, with detailed inclusion of the patient perspectives throughout, informing practice standards and considerations. This is an ideal guide for pediatric and adult physicians who care for patients with cystic fibrosis, as well as respiratory therapists, physical therapists, nurses, nutritionists, and pharmacists who care for these patients.

Autorentext

Stephanie D Davis, MD is Professor of Pediatrics, Director of Pediatric Pulmonology, Allergy and Sleep Medicine, and Vice Chair of Clinical Affairs in the Department of Pediatrics at Indiana University School of Medicine. She is volume editor of another series book, Diagnostic Tests in Pediatric Pulmonology (2015).

Margaret Rosenfeld, MD, MPH is Professor of Pediatrics in the Division of Pulmonary Medicine and Associate Director of the Center for Clinical and Translational Research at Seattle Children's Hospital. James Chmiel, MD, MPH is Professor of Pediatrics in the Division of Pediatric Pulmonology, Allergy/Immunology and Sleep Medicine, Director of the Cystic Fibrosis Therapeutics Development Center, Associate Center Director of the LeRoy W. Matthews Cystic Fibrosis Center, and Director of the Pediatric Cystic Fibrosis Program at Case Western Reserve University School of Medicine and Rainbow Babies and Children's Hospital.

Inhalt

The Changing Face of CF.- Diagnosing cystic fibrosis.- SECTION I: Pulmonary Manifestations.- Early lung disease.- Pulmonary complications as an adult.- Treating respiratory complications.- Transplant.- SECTION II: Gastrointestinal Manifestations.- Liver disease.- Pancreatic insufficiency and nutritional complications.- SECTION III: Endocrine Manifestations.- Diabetes.- Bone Disease. SECTION IV: Further Comorbidities.- Rheumatologic Manifestations.- Reproductive Issues.- The Impact of CF on the Kidney.- Mental Health.