Cystic Fibrosis

This international and authoritative work, which brings together current knowledge in the field of cystic fibrosis, has become established in previous editions as a leading reference in the field. The third edition continues to provide everything that the clinician or allied health professional treating patients with cystic fibrosis will need in a single manageable volume. Thoroughly revised and updated throughout, it reflects the significant advances that have been made in the field since the second edition published in 2000.



Cystic Fibrosis evaluates in detail the basic science that underlies the disease and its progression, putting it into a clinical context. Diagnostic and clinical aspects are covered in depth, as are monitoring the condition and the importance of multi-disciplinary care, reflected in the sections into which the new edition has been sub-divided to improve accessibility. Future developments, including novel therapies, are covered in a concluding section. The clinical areas have been much expanded, with the introduction of separate chapters covering sleep, lung mechanics and the work of breathing, upper airway disease, insulin deficiency and diabetes, bone disease, and sexual and reproductive issues. A new section on monitoring discusses the use of databases to improve patient care, and covers monitoring in different age groups, exercise testing and the outcomes of clinical trials in these areas. Separate chapters are devoted to paramedical issues, including 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 sciences aspects of the subject, and to reflect the multi-disciplinary nature of the cystic fibrosis care team. Drawing on the expertise of a team of international specialists from a variety of backgrounds, the third edition of Cystic Fibrosiswill continue to find a broad readership among respiratory physicians, paediatricians, specialist nurses and other health professionals working with patients with cystic fibrosis.


International and authoritative
Provides comprehensive coverage appropriate for the clinician and health care professional in a single readable volume
Recognizes the importance of the basic science aspects, putting them into a context appropriate to the readership
Many new chapters expand coverage of all areas, including clinical aspects, monitoring and future developments
Reflects the multi-disciplinary nature of the cystic fibrosis care team
Covers management in the hospital and at home



Table of Contents:
PART 1 INTRODUCTION: WHAT IS CYSTIC FIBROSIS?
1 History of cystic fibrosis
2 Epidemiology of cystic fibrosis
PART 2 BASIC SCIENCE FOR THE CLINICIAN
3 Molecular biology of cystic fibrosis: CFTR processing and functions, and classes of mutations
4 Pathophysiology: epithelial cell biology and ion channel function in the lung, sweat gland and pancreas
5 Immunology of cystic fibrosis
6a Genotype–phenotype correlations and modifier genes
6b Variability of clinical course in cystic fibrosis
PART 3 DIAGNOSTIC ASPECTS OF CYSTIC FIBROSIS
7 Diagnosis of cystic fibrosis
8a The challenge of screening newborn infants for cystic fibrosis
8b How to manage the screened patient
9 Microbiology of cystic fibrosis
PART 4 CLINICAL ASPECTS OF CYSTIC FIBROSIS
10 Respiratory disease: infection
11 Respiratory disease: non-infectious complications
12 Sleep, lung mechanics and work of breathing, including NIPPV 175
13 Delivery of therapy to the cystic fibrosis lung
14 The upper airway in cystic fibrosis
15a Gastrointestinal disease in cystic fibrosis
15b Liver, biliary and pancreatic disease
16 Insulin deficiency and diabetes related to cystic fibrosis
17 Growth and puberty
18 Cystic-fibrosis-related low bone mineral density
19 Other system disorders in cystic fibrosis
20 Sexual and reproductive health
21 Transplantation
PART 5 MONITORING
22 Using databases to improve care
23a Infant and pre-school children: lung function
23c Infant and pre-school children: imaging the lungs
5a Exercise: testing
25b Exercise: use in therapy
26 Clinical outcome measures to assess new treatments for cystic fibrosis lung disease PART 6 MULTIDISCIPLINARY CARE
28 Nursing care
29 Physiotherapy
31 Psychological aspects of cystic fibrosis 433
Alistair J. A. Duff and Helen Oxley
32 Palliative care in cystic fibrosis PART 7 CYSTIC FIBROSIS: THE FUTURE
33b Non-gene therapy treatments: what will they deliver?

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