Cystic Fibrosis (2nd edition)

ISBN : 9780198702948

Alex Horsley; Steve Cunningham; J. Alastair Innes
208 ページ
129 x 197 mm
Oxford Respiratory Medicine Library

Cystic Fibrosis (CF) is a multi-system disorder, requiring not just respiratory expertise but also management of nutrition, diabetes, musculoskeletal and psychosocial issues. This pocketbook is a concise companion for all health care professionals who manage patients with CF. The book covers all aspects of care, including both paediatric and adult-specific issues, and summarizes up-to-date literature in a concise and focussed style. There is an emphasis on the practical aspects of management with separate chapters covering the effects of CF in the lung, the microbiology of pulmonary CF, and management of exacerbations. Psychosocial aspects of CF care, end of life care and lung transplantation are also covered, and potential future therapies reviewed. This second edition has been extensively updated to reflect the UK CF Trust Standards of Care, treatment guidelines and Cochrane reviews. There are updates on emerging organisms; an expanded section on physiotherapy; and a new Pharmacopeia that covers all common CF medications.


1. Genetics and Pathophysiology
2. Diagnosis and Process of Care
3. Microbiology of CF lung disease
4. Management of stable CF lung disease
5. Management of Respiratory Exacerbations
6. Respiratory complications and management of severe CF lung disease
7. Gastrointestinal Disease and Nutrition
8. Cystic Fibrosis Related Diabetes
9. Metabolic and Musculoskeletal Effects of Cystic Fibrosis
10. Psychological aspects of CF care
11. Lung Transplantation
12. Fertility, contraception and pregnancy
13. Future Treatments
14. Pharmacy and the CF Pharmacopoeia


Alex Horsley, MA, MBChB, MRCP, PhD. NIHR Clinician Scientist at the University of Manchester and Honorary Consultant at the Manchester Adult CF Centre. Dr Horsley graduated from Cambridge and Edinburgh Universities and finished his respiratory training in Leeds and Edinburgh. He completed a PhD on lung physiology and lung clearance index at Edinburgh University with the CF Gene Therapy Consortium and has published and spoken on this widely. His research focuses on the development of early change in the CF lung, lung physiology technologies and the application of these to clinical practice.; Dr Steve Cunningham is Consultant Respiratory Paediatrician at the Royal Hospital for Sick Children, Edinburgh, UK. Steve trained in Respiratory Paediatrics in Edinburgh, Great Ormond Street, London, and Sydney, Australia. He has previously published on models for Retinopathy of Prematurity, but is now more active within the United Kingdom Cystic Fibrosis Gene Therapy Consortium assessing clinical indicators of disease as outcome factors. In addition, Steve has a focus on the role of oxygen as a therapeutic agent in bronchiolitis. Steve is Chair of the Scottish Intercollegiate Guideline Network (SIGN91) Bronchiolitis Guideline, and also a contributing member to the Pharmacology section of the SIGN/BTS Living Asthma Guideline, and the British Thoracic Society Non-CF Bronchiectasis Guideline. He is also Chair of the Asthma UK Scottish Advisory Group.; Dr Alastair Innes, University of Edinburgh. Dr Alastair Innes is Clinical Director of Respiratory Medicine for NHS Lothian and Honorary Reader in Respiratory Medicine at the University of Edinburgh. As a member of the Strategy Group, he heads the clinical part of the CFGT research team in Edinburgh. Alastair qualified in Edinburgh, and trained in Newcastle, London and Los Angeles before returning to a consultant post at the Western General Hospital in Edinburgh. He has worked in Adult Cystic Fibrosis care for 18 years, during which time he was involved in initial trials of nasal CF gene therapy in Edinburgh. Alastair's principal research interests are in novel clinical physiological measurements of airways disease, and their use to monitor disease processes and response to therapy.