Cystic Fibrosis in the 21st Century / Edition 1

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Cystic fibrosis used to be thought of as a respiratory and digestive disease, with a uniformly and rapidly fatal outcome. The spectrum of the disease has broadened into the mild atypical case, presenting in middle age, with the potential for complications in virtually every system of the body. In the past few years there has been an explosion of knowledge of the basic science of the defect. The editors have therefore invited the leading scientists and clinicians in the field of cystic fibrosis to describe the recent advances in this disease. Although there are many 'Recent Advances' texts, previous books have been selective in their choice of topics. This book is the first to cover the entire field of this complex disease, and encompasses the rapidly moving topics of the basic molecular and cellular biology as well as the recent multi-system, multi-disciplinary advances in the clinical care of patients. The authors have been charged with writing only about new developments and not to rehash old literature. The bulk of the references is therefore less than five years old. This book addresses all professionals who treat cystic fibrosis and want to have an update of new findings in the field, particularly of those outside their immediate specialisation. It will also be useful for basic researchers interested in related scientific areas and the clinical context of their work.

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Product Details

  • ISBN-13: 9783805579605
  • Publisher: Karger, S. Inc.
  • Publication date: 1/28/2006
  • Series: Progress in Respiratory Research Series
  • Edition number: 1
  • Pages: 330
  • Product dimensions: 8.40 (w) x 11.20 (h) x 0.70 (d)

Table of Contents

Ch. 1 The CFTR gene : structure, mutations and specific therapeutic approaches 2
Ch. 2 Exquisite and multilevel regulation of CFTR expression 11
Ch. 3 Intracellular processing of CFTR 21
Ch. 4 Structure of the cystic fibrosis transmembrane conductance regulator 29
Ch. 5 Function of CFTR protein : ion transport 38
Ch. 6 Cystic fibrosis : function of CFTR protein : regulatory functions 45
Ch. 7 Function of CFTR protein : developmental role 54
Ch. 8 Genotype-phenotype correlations in cystic fibrosis 61
Ch. 9 Diagnosis of cystic fibrosis, CFTR-related disease and screening 69
Ch. 10 CF modifier genes 77
Ch. 11 Animal models 84
Ch. 12 In vitro/ex vivo fluorescence assays of CFTR chloride channel function 93
Ch. 13 In vivo measurement of airway potential difference to assess CFTR function in man 102
Ch. 14 Arrays and proteomics 109
Ch. 15 Pathogenesis of pulmonary disease in cystic fibrosis 116
Ch. 16 Infection versus inflammation 122
Ch. 17 Pseudomonas aeruginosa : clinical research 131
Ch. 18 Pseudomonas aeruginosa : basic research 138
Ch. 19 Other gram-negative organisms : Burkholderia cepacia complex and Stenotrophomonas maltophilia 145
Ch. 20 Staphylococcus aureus and MRSA 153
Ch. 21 Nontuberculous mycobacterial lung disease in patients with cystic fibrosis 160
Ch. 22 Respiratory fungal infections and allergic bronchopulmonary aspergillosis 166
Ch. 23 Advanced disease management and advances in transplant medicine 173
Ch. 24 Current and novel antimicrobial approaches 180
Ch. 25 Anti-inflammatory agents : a clinical perspective 187
Ch. 26 Recent advances in infant and pre-school lung function 195
Ch. 27 Recent advances in imaging 205
Ch. 28 New pharmacological approaches for treatment of cystic fibrosis 212
Ch. 29 Gene and stem cell therapy 221
Ch. 30 Gut disease : clinical manifestations, pathophysiology, current and new treatments 232
Ch. 31 Pancreatic involvement : clinical manifestations, pathophysiology and new treatments 242
Ch. 32 Cystic fibrosis : liver disease 251
Ch. 33 Fertility, contraception, incontinence and pregnancy 264
Ch. 34 Arthritis, vasculitis and bone disease 270
Ch. 35 Diabetes in cystic fibrosis 278
Ch. 36 Challenges for nurses 286
Ch. 37 Dietetics 293
Ch. 38 Physiotherapy 301
Ch. 39 Psychological interventions 309
Ch. 40 The future 316
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