5th Edition
Hodson and Geddes' Cystic Fibrosis
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
Biography
Andrew Bush MD FHEA FRCP FRCPCH FERS FAPSR ATSF
Professor of Paediatrics and Paediatric Respirology, National Heart and Lung Institute, and Imperial Centre for Paediatrics and Child Health, Imperial College
Consultant Paediatric Chest Physician, Royal Brompton & Harefield NHS Foundation Trust
NIHR Senior Investigator Emeritus
Margarida D. Amaral
Professor
BioISI – Biosystems & Integrative Sciences Institute
Faculty of Sciences, University of Lisboa, Portugal
Jane C. Davies
Professor in Paediatric Respirology & Experimental Medicine.
Imperial College London
Honorary Consultant in Paediatric Respiratory Medicine
Royal Brompton Hospital, part of Guy’s & St Thomas’ NHS Foundation Trust
London
United Kingdom
Nicholas Simmonds MD(Res) FRCP
Associate Director | Consultant Respiratory Physician
Adult Cystic Fibrosis Centre, Royal Brompton Hospital
Professor of Practice (Respiratory Medicine)
National Heart and Lung Institute, Imperial College London
Adult Cystic Fibrosis Centre, Royal Brompton Hospital
Sydney Street, London
United Kingdom
Jennifer L. Taylor-Cousar, MD, MSCS, ATSF
President, Medical Staff
Medical Director, Clinical Research Services
Co-Director and CF TDC Director, Adult CF Program
Professor, Departments of Medicine and Pediatrics,
Divisions of Pulmonary, Critical Care and Sleep Medicine and Pediatric Pulmonary Medicine
Divisions of Pulmonary Sciences and Critical Care Medicine and Pediatric Pulmonology
University of Colorado Anschutz Medical Campus
Sarath Ranganathan MBChB MRCP FRCPH FRACP PhD FThorSoc ATSF
Stevenson Chair and Head, Department of Paediatrics | Academic Director of Clinical Education Strategy and Risk
University of Melbourne
Consultant, Respiratory and Sleep Medicine | Royal Children’s Hospital
Head, Respiratory Diseases Research Group | Infection and Immunity | Murdoch Children’s Research Institute
