Hodson and Geddes' Cystic Fibrosis

Description

Hodson and Geddes' Cystic Fibrosis provides everything the respiratory clinician, pulmonologist or health professional treating patients needs in a single manageable volume. This international and authoritative work brings together current knowledge and has become established in previous editions as a leading reference in the field. This fourth edition includes a wealth of new information, figures, useful videos, and a companion eBook.

The basic science that underlies the disease and its progression is outlined in detail and put into a clinical context. Diagnostic and clinical aspects are covered in depth, as well as promising advances such as gene therapies and other novel molecular based treatments. Patient monitoring and the importance of multidisciplinary care are also emphasized.

This edition:

• Features accessible sections reflecting the multidisciplinary nature of the cystic fibrosis care team
• Contains a chapter written by patients and families about their experiences with the disease
• Includes expanded coverage of clinical areas, including chapters covering sleep, lung mechanics and the work of breathing, upper airway disease, insulin deficiency and diabetes, bone disease, and sexual and reproductive issues
• Discusses management both in the hospital and at home
• Includes a new section on monitoring and discusses the use of databases to improve patient care
• Covers monitoring in different age groups, exercise testing and the outcomes of clinical trials in these areas
• Includes chapters devoted to nursing, physiotherapy, psychology, and palliative and spiritual care

Throughout, the emphasis is on providing an up-to-date and balanced review of both the clinical and basic science aspects of the subject and reflecting the multidisciplinary nature of the cystic fibrosis care team.

Features

• Covers the clinical areas that reflect the multidisciplinary nature of the condition
• Presents new chapters on Living with Cystic Fibrosis, Growing Old with Cystic Fibrosis, Quality Improvement, and Outcome of Clinical Trials
• Includes expanded information on animal models, infection and inflammation of the airway affected by cystic fibrosis, management of the screened patient, and pulmonary exacerbations
• Includes a complimentary eBook containing many more references, links to videos, and references within PubMed
• Presents information in a completely redesigned format so that the key facts can be found at a glance

Table of Contents

INTRODUCTION: WHAT IS CYSTIC FIBROSIS?

Introduction: from the Discovery of the CFTR Gene in 1989 through to 2014; Kris De Boeck

Epidemiology of Cystic Fibrosis; Stephanie J. MacNeill

Living with Cystic Fibrosis: The Voices of Patients and Families; Matt Benn, Helen Benn, Jess Harrison, Kevin Passey, Emily Hoyle, Rupert Pearce Gould, and Frances Pearce Gould

BASIC SCIENCE FOR THE CLINICIAN

Molecular Biology of Cystic Fibrosis: CFTR Processing and Functions and Classes of Mutations; Pascale Fanen and Isabelle Sermet-Gaudelus

Underlying Concepts of the Pathophysiology of Cystic Fibrosis in the Sweat Gland, GI Tract, and Lung; Richard C. Boucher

What Have We Learned from Animal Models? Uta Griesenbach and Eric W.F.W. Alton

Interactions Between Infection and Inflammation in the Cystic Fibrosis Airway; Sanjay H. Chotirmall, Michelle A. Murray, Kevin Molloy, and Noel G. McElvaney

CFTR and Interactions with Modifier Genes and the Environment: Genotype–Phenotype Correlations and Modifier Genes; Nicholas J. Simmonds, R.J. Darrah, and M.L. Drumm

CFTR and Interactions with Modifier Genes and the Environment: Environment; Joshua N. Freedman and Michael S. Schechter

DIAGNOSTIC ASPECTS OF CYSTIC FIBROSIS

Diagnosis of the Symptomatic Patient; Colin Wallis and Nicholas J. Simmonds

Screening: What Tests? Kevin Southern

Pathway of Pain: Your "Normal" Baby Has a Life-Threatening Disease; Siân Bentley, Nicola Collins, Mary Jurd, Michele Puckey, Pat Stringer, and Andrew Bush

Microbiology of Cystic Fibrosis: Epidemiology of Cystic Fibrosis Pathogens and Clinical Microbiology Laboratory Methods; Christina Gagliardo and Lisa Saiman

New Methods for Detecting and Identifying Bacteria; Geraint B. Rogers and Gerd Döring

Infection Control; Andrew M. Jones

CLINICAL ASPECTS OF CYSTIC FIBROSIS

Respiratory Disease: Infectious Complications; J. Stuart Elborn, Ian M. Balfour-Lynn, and Diana Bilton

Pulmonary Exacerbations; Patrick A. Flume and Donald R. VanDevanter

Respiratory Disease: Noninfectious Complications; Margaret Hodson and Andrew Bush

Sleep, Lung Mechanics and Work of Breathing, Including Noninvasive Ventilation; Brigitte Fauroux and Sonia Khirani

Delivering Therapy to the Cystic Fibrosis Lung; Bruce K. Rubin and Ronald W. Williams

Upper Airway Disease; Romana Kuchai and William E. Grant

Gastrointestinal Disease in Cystic Fibrosis; Alan Steel and David Westaby

The Liver in Cystic Fibrosis; Shahid A. Khan and David Westaby

Insulin Deficiency and Cystic Fibrosis-Related Diabetes; Christopher Sheldon and Rachel Rowe

Growth and Puberty; Nicola Bridges

Bone Disease; Jennifer L. Goralski and Robert M. Aris

Other Cystic Fibrosis-Related Disease; Khin Ma Gyi and Margaret Hodson

Sexual and Reproductive Issues; J. Guy Thorpe-Beeston and Susan Madge

Transplantation; Martin Carby, Andre Simon, and Paul Aurora

Growing Old with Cystic Fibrosis; Nicholas J. Simmonds

MONITORING

Quality Improvement: Using Databases to Improve Cystic Fibrosis Care; Christopher H. Goss, Bradley S. Quon, and Diana Bilton

Infant and Preschool Lung Function; Sarath Ranganathan

Role of Bronchoscopy and Bronchoalveolar Lavage in Infants with Cystic Fibrosis; Claire Wainwright and Felix Ratjen

Clinical and Radiological Aspects of Imaging the Lungs; Samatha Sonnappa and Catherine M. Owens

Physiological Monitoring of Older Children and Adults; Mark Rosenthal

Exercise: Testing and Use in Therapy; Hiran Selvadurai and David Orenstein

Outcome of Clinical Trials: Electricity, Induced Sputum, and Breath; Michael Waller, Eric W.F.W. Alton, and Jane C. Davies

Chest Computed Tomography and Clinical Trials in Cystic Fibrosis; Harm A.W.M. Tiddens, Marcel van Straten, Arlette Odink, and Stephen M. Stick

Outcomes of Clinical Trials: Multiple Breath Washout Tests in Cystic Fibrosis; Padmaja Subbarao and Felix Ratjen

What Have we Learned Over the Last 5 Years? J. Stuart Elborn, Jane C. Davies, and Diana Bilton

MULTIDISCIPLINARY CARE

Cystic Fibrosis Center Care; Susan Madge, Jackie Francis, and Diana Bilton

Nursing; Susan Madge and Kamilla Dack

Physiotherapy; Penny Agent, Nicola Collins, and Helen Parrott

Nutritional Aspects; Sue Wolfe and Sarah Collins

Psychology; Alistair J.A. Duff and Helen Oxley

Palliative and Spiritual Care; Finella Craig and Anna-Marie Stevens

CYSTIC FIBROSIS: THE FUTURE

Basic Science: What Will It Deliver? Gene and Stem Cell Therapy; Uta Griesenbach and Eric W.F.W. Alton

Basic Science: What Will It Deliver? Non-Gene Therapy Treatments; Jane C. Davies and Felix Ratjen

The Future: How Will Management Change? Andrew Bush and Duncan Geddes

APPENDICES

Appendix A: History of Cystic Fibrosis; James M. Littlewood

Appendix B: How to Manage the Screened Patient; Philip Robinson

Index

Author(s)

Andrew Bush, Imperial College and Royal Brompton and Harefield NHS Foundation Trust, London, UK
Diana Bilton, Royal Brompton and Harefield NHS Foundation Trust, London, UK
Margaret Hodson, Imperial College and Royal Brompton and Harefield NHS Foundation Trust, London, UK