Cystic Fibrosis in the 21st Century

Portada
Andrew Bush
Karger Medical and Scientific Publishers, 2006 - 329 páginas
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 up-date 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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Índice

Chapter 2 Exquisite and Multilevel Regulation of CFTR Expression
11
Chapter 3 Intracellular Processing of CFTR
21
Chapter 4 Structure of the Cystic Fibrosis Transmembrane Conductance Regulator
29
Ion Transport
38
Chapter 6 Cystic Fibrosis
45
Developmental Role
54
Chapter 8 GenotypePhenotype Correlations in Cystic Fibrosis
61
Chapter 9 Diagnosis of Cystic Fibrosis CFTRRelated Disease and Screening
69
Chapter 23 Advanced Disease Management and Advances in Transplant Medicine
173
Chapter 24 Current and Novel Antimicrobial Approaches
180
Chapter 25 AntiInflammatory Agents
187
Chapter 26 Recent Advances in Infant and PreSchool Lung Function
195
Chapter 27 Recent Advances in Imaging
205
Chapter 28 New Pharmacological Approaches for Treatment of Cystic Fibrosis
212
Chapter 29 Gene and Stem Cell Therapy
221
Clinical Manifestations Pathophysiology Current and New Treatments
232

Chapter 10 CF Modifier Genes
77
Chapter 11 Animal Models
84
Chapter 12 In VitroEx Vivo Fluorescence Assays of CFTR Chloride Channel Function
93
Chapter 13 In Vivo Measurement of Airway Potential Difference to Assess CFTR Function in
102
Chapter 14 Arrays and Proteomics
109
Chapter 15 Pathogenesis of Pulmonary Disease in Cystic Fibrosis
116
Chapter 16 Infection versus Inflammation
122
Clinical Research
131
Basic Research
138
Chapter 19 Other GramNegative Organisms
145
Chapter 20 Staphylococcus aureus and MRSA Conway S Denton M
160
Chapter 22 Respiratory Fungal Infections and Allergic Bronchopulmonary
166
Clinical Manifestations Pathophysiology and New Treatments
242
Liver Disease
251
Chapter 33 Fertility Contraception Incontinence and Pregnancy
264
Chapter 34 ArthritisVasculitis and Bone Disease
270
Chapter 35 Diabetes in Cystic Fibrosis
278
Chapter 36 Challenges for Nurses
286
Chapter 37 Dietetics
293
Chapter 38 Physiotherapy
301
Chapter 39 Psychological Interventions
309
Chapter 40 The Future
316
BoydA C
323
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