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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
The Changing Face of Cystic Fibrosis
Pages 3-16
Cogen, Jonathan D. (et al.)
Diagnosis of Cystic Fibrosis
Pages 17-33
Thomas, Caroline S. (et al.)
Health Disparities
Pages 35-55
Oates, Gabriela R. (et al.)
Early Cystic Fibrosis Lung Disease
Pages 59-72
Ranganathan, Sarath C.
Bacterial Infections and the Respiratory Microbiome
Pages 73-92
Waters, Valerie J. (et al.)
Fungal Infections and ABPA
Pages 93-126
Mac Aogáin, Micheál (et al.)
Nontuberculous Mycobacterium
Pages 127-160
Ruffles, Thomas (et al.)
Inflammation in CF: Key Characteristics and Therapeutic Discovery
Pages 161-179
Polineni, Deepika (et al.)
Pulmonary Exacerbations
Pages 181-198
Montemayor, Kristina (et al.)
Maintenance of Pulmonary Therapies
Pages 199-213
Paranjape, Shruti M. (et al.)
Advanced Stage Lung Disease
Pages 215-227
Garcia, Bryan (et al.)
Lung Transplantation for Cystic Fibrosis
Pages 229-258
Pilewski, Joseph M.
Exocrine Pancreatic Insufficiency and Nutritional Complications
Pages 261-298
Mandalia, Amar (et al.)
Hepatobiliary Involvement in Cystic Fibrosis
Pages 299-320
Bertolini, Anna (et al.)
Gastrointestinal Complications
Pages 321-334
Stein, Adam C. (et al.)
Cystic Fibrosis-Related Diabetes
Pages 337-353
Granados, Andrea (et al.)
Cystic Fibrosis-Related Bone Disease: Current Knowledge and Future Directions
Pages 355-372
Guérin, Sophie (et al.)
Rheumatologic Manifestations of CF
Pages 373-392
Reis, Amanda (et al.)
Impact of CF on the Kidneys
Pages 393-412
Prayle, Andrew (et al.)
Sexual and Reproductive Health in Cystic Fibrosis
Pages 413-427
Crowley, Erin (et al.)
Mental Health in Cystic Fibrosis
Pages 429-447
Behrhorst, Kathryn L. (et al.)
Understanding Treatment Adherence in Cystic Fibrosis: Challenges and Opportunities
Pages 449-463
Muther, Emily F. (et al.)
Molecular Genetics of Cystic Fibrosis
Pages 467-481
Han, Sangwoo T. (et al.)
Targeting the Underlying Defect in CFTR with Small Molecule Compounds
Pages 483-501
Guimbellot, Jennifer S. (et al.)
Gene Editing for CF
Pages 503-514
Drumm, Mitchell L.
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