Hodson and Geddes' Cystic Fibrosis (5版)

Cystic Fibrosis has seen dramatic advances in treatment since the last edition, including targeted cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators for most CFTR gene abnormalities. This new fifth edition is an update and expansion of the rapid clinical and scientific advances in improving prognosis, and the impact of COVID-19, all of which has transformed conventional models of care. It covers basic science, such as how detailed understanding of the biology of the CFTR gene and protein has led to novel and beneficial therapies, as well as all aspects of clinical management in high-, middle- and low-income settings and the voices of individuals with CF from across the world. It will be a useful reference for clinicians, including all levels of trainees, across the whole multidisciplinary team, scientists and students. Key Features • Follows an appealing organization of chapters, by developing fundamental knowledge of the reader before moving on to more complex or developing topics. • Presents a comprehensive, authoritative and up-to-date text, integrating fundamental science and clinical aspects of cystic fibrosis providing an attractive read for clinicians, trainee doctors and scientists. • Draws on global expertise and reflects best evidence-based practice from experts conducting cutting-edge clinical and basic science research from around the world.

Ch 1 Journey from Macromolecular Pathology to Molecular Therapies Bonnie W. Ramsey and Christiane (Kris) De Boeck Ch 2 Global epidemiology of CF: High income and low/middle income countries (global harmonisation registry countries) Ahmad M. Hider and Samya Z. Nasr Ch 3 Voices of Patients and Families Lorna Allen Ch 4 Patient Organisations Nettie Burke Ch 5 Molecular Biology of CFTR: From the Gene to the Protein Deborah M. Cholon, Andrei A. Aleksandrov and Martina Gentzsch Ch 6 Biology of the CF airway epithelium Tahir Idris, Marc Chanson and Mehdi Badaoui Ch 7 The physiology of epithelial ion and fluid transport: beyond CFTR modulators Livia Delpiano and Michael A. Gray Ch 8 Inflammation in Cystic Fibrosis Deepika Polineni and James F. Chmiel Ch 9 Model Organisms of Cystic Fibrosis Daniel P. Cook and David A. Stoltz Ch 10 Systems Biology and the New Omics Margarida D. Amaral, Luka A. Clarke, Carlos M. Farinha, Hugo M. Botelho Ch 11 Genotype: phenotype correlations Karen S. Raraigh and Garry R. Cutting Ch 12 Gene Environment Interactions Shivanthan Shanthikumar Ch 13 Demographic, socioeconomic, and environmental contributions to health in cystic fibrosis John B. Palla, Meghan E. McGarry and Susanna A. McColley Ch 14 Drug Discovery Platforms for CFTR modulators Nicoletta Pedemonte Ch 15 CFTR modulator drug discovery and translation into the clinic Marlou Bierlaagh, Jeffrey Beekman and Kors van der Ent Ch 16 Newborn and Carrier Screening for CF John Massie and Juerg Barben Ch 17 Diagnostic tests: Sweat testing, epithelial potential differences and genetic testing Elke De Wachter and Caroline Raynal Ch 18 Immediate Management of The Newly Screened Positive Baby Jessica L. Saunders and Clement L. Ren Ch 19 Diagnosis of the symptomatic patient Dorota Sands and Nicholas J. Simmonds Ch 20 Blurred boundaries: CRMS/ CFSPID and CFTR related disorders Nicholas J. Simmonds and Jane C. Davies Ch 21 Respiratory Disease across the Lifecourse Noah Lechtzin and Peter J. Mogayzel, Jr. Ch 22 Respiratory effects of the new CFTR modulators Jennifer L. Taylor-Cousar, Alex H. Gifford, Dominic Hughes, John A King and Jane C. Davies Ch 23 Epidemiology and microbiology of cystic fibrosis pulmonary infections Christina Thornton, Ranjani Somayaji and Valerie Waters Ch 24 New methods for detecting and identifying bacteria Eshwar Mahenthiralingam Ch 25 Non-tuberculous Mycobacterial Infections in Cystic Fibrosis Jerry A. Nick and Claire E Wainwright Ch 26 Fungal diseases in CF Carsten Schwarz Ch 27 Molecular microbiology of the CF gut and lung Sarath Ranganathan Ch 28 Pulmonary Exacerbations in Cystic Fibrosis: Epidemiology, Treatment, Outcomes, and Future Research Don B. Sanders and Natalie E. West Ch 29 Infection Prevention and Control in Cystic Fibrosis Charles S. Haworth and R. Andres Floto Ch 30 Technology in Cystic Fibrosis Therapies Christina M. Mingora and Patrick A. Flume Ch 31 Upper Airway Disease in Cystic Fibrosis Cassie Shipp, John Brewington, Daniel Beswick and Raouf Amin Ch 32 Gastrointestinal Disease in CF Zachary M. Sellers and Michael Wilschanski Ch 33 Cystic Fibrosis Liver Disease Julia M Boster and Michael R Narkewicz Ch 34 Cystic Fibrosis-Related Diabetes Melissa S. Putman, Sarah Collins and Kara S Hughan Ch 35 Growth in Cystic Fibrosis: Is chloride transport the key? Michael S Stalvey Ch 36 Bone Disease in Cystic Fibrosis Peter Simm Ch 37 Cancer and cystic fibrosis Daniel Peckham and Rebecca Birch Ch 38 Other cystic fibrosis-related diseases and complications Michal Shteinberg, Mirjam Stahl Ch 39 Extrapulmonary benefits of the new CFTR modulator drugs Nicole Green, Lina Merjaneh, Steven D. Freedman and Margaret Rosenfeld Ch 40 Sexual health, fertility, and pregnancy in people with cystic fibrosis Raksha Jain, Susan M Sawyer and Malena Cohen-Cymberknoh Ch 41 Mental Health Issues in Cystic Fibrosis Janice Abbott and Trudy Havermans Ch 42 Adherence and Self-Management in Cystic Fibrosis Care Gregory Sawicki Ch 43 Transplantation Siddhartha G. Kapnadak and Kathleen J. Ramos Ch 44: Working with cystic fibrosis Scott C. Bell, Beth S. Sufian and James A. Passamano Ch 45 Growing Old with Cystic Fibrosis Andrew M Jones Ch 46 Imaging of Cystic Fibrosis Lung Disease Harm AWM Tiddens, Marcel van Straten, Mirjam Stahl and Pierluigi Ciet Ch 47 Lung function testing including Multiple Breath Washout Author: Paul D Robinson Ch 48 Infant Pulmonary Function Tests Lucy Perrem, Stephanie D Davis Ch 49 Exercise testing in cystic fibrosis Don Urquhart and Thomas Radtke Ch 50 Bronchoscopy and bronchoalveolar lavage in cystic fibrosis Isaac Martin and Felix Ratjen Ch 51 Patient-derived cell-based models for theratyping and individual CFTR modulator assessment Shannon M.A Smits, Lisa W. Rodenburg, Gimano D. Amatngalim and Jeffrey M. Beekman Ch 52 Minimally Invasive Investigations for Biomarkers of Airways Disease Joshua D. Chandler and Charles R. Esther Jr Ch 53 Currrent And Future Interventional Trial Designs To Support The Cf Therapeutic Pipeline Nicole Mayer-Hamblett and Bradley Quon Ch 54 Gene and RNA-based therapies Marie E Egan and Patrick T Harrison Ch 55 Cystic fibrosis in limited resource settings Silva Filho LVRF, Marco Zampoli2, Malena Cohen-Cymberknoh3, Sushil K Kabra Ch 56 Traveling With Cystic Fibrosis Meg Dvorak, Lcsw & Kate Yablonsky, Lcsw Ch 57 Organisation of Cystic Fibrosis Centre Care A Whitney Brown and Bruce C. Marshall Ch 58 The role of the CF nurse specialist Paula Lomas, Niccola Burke and Jill Fliege Ch 59 Physiotherapy Eleanor Main, Emma Dixon, Nicky Murray Ch 60 The role of the Dietitian Inês Asseiceira, Chris Smith and Joaquim Calvo-Lerma Ch 61 The role of the pharmacist as part of the multidisciplinary team caring for CF patients Martin J. Hug, Ph.D. Ch 62 The Role of a Psychologist on the Cystic Fibrosis Care Team Christina Jayne Bathgate, Sonia Graziano and Emily Muther Ch 63 Adolescent Health and Transition in Cystic Fibrosis Ryan Perkins, Gregory S. Sawicki and Traci M. Kazmerski Ch 64 Palliative and supportive care Stephen J. Bourke and Rachel Quibell Ch 65 Using registries & databases to drive up quality Oliver J. McElvaney and Christopher H. Goss Ch 66 Digital Transformations within CF Healthcare Tamara Vagg, Kevin F Deasy and Barry J Plant Ch 67 Personalized Medicine for Cystic Fibrosis in the 21st Century Kris De Boeck and Margarida D Amaral Ch 68 CF Research Priorities for the Future Jan-Christoph Thomassen, John Sheridan, Silke van Koningsbruggen-Rietschel, JP Clancy