Cystic Fibrosis in the 21st Century Progress in Respiratory Research 1st Edition by Andrew Bush, Eric W F W Alton, Jane C Davies, Uta Griesenbach, Adam Jaffe ISBN 3805579608 9783805579605

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ISBN 10: 3805579608

ISBN 13: 9783805579605 

Author: Andrew Bush, Eric W. F. W. Alton, Jane C. Davies, Uta Griesenbach, Adam Jaffe

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.

Table of contents:

1. The CFTR Gene: Structure, Mutations and Specific Therapeutic Approaches

2. Exquisite and Multilevel Regulation of CFTR Expression

3. Intracellular Processing of CFTR

4. Structure of the Cystic Fibrosis Transmembrane Conductance Regulator

5. Function of CFTR Protein: Ion Transport

6. Cystic Fibrosis: Function of CFTR Protein: Regulatory Functions

7. Function of CFTR Protein: Developmental Role

8. Genotype-Phenotype Correlations in Cystic Fibrosis

9. Diagnosis of Cystic Fibrosis, CFTR-Related Disease and Screening

10. CF Modifier Genes

11. Animal Models

12. In Vitro/Ex Vivo Fluorescence Assays of CFTR Chloride Channel Function

13. In Vivo Measurement of Airway Potential Difference to Assess CFTR Function in Man

14. Arrays and Proteomics

15. Pathogenesis of Pulmonary Disease in Cystic Fibrosis

16. Infection versus Inflammation

17. Pseudomonas aeruginosa: Clinical Research

18. Pseudomonas aeruginosa: Basic Research

19. Other Gram-Negative Organisms: Burkholderia cepacia complex and Stenotrophomonas maltophilia

20. Staphylococcus aureus and MRSA

21. Nontuberculous Mycobacterial Lung Disease in Patients with Cystic Fibrosis

22. Respiratory Fungal Infections and Allergic Bronchopulmonary Aspergillosis

23. Advanced Disease Management and Advances in Transplant Medicine

24. Current and Novel Antimicrobial Approaches

25. Anti-Inflammatory Agents: A Clinical Perspective

26. Recent Advances in Infant and Pre-School Lung Function

27. Recent Advances in Imaging

28. New Pharmacological Approaches for Treatment of Cystic Fibrosis

29. Gene and Stem Cell Therapy

30. Gut Disease: Clinical Manifestations, Pathophysiology, Current and New Treatments

31. Pancreatic Involvement: Clinical Manifestations, Pathophysiology and New Treatments

32. Cystic Fibrosis: Liver Disease

33. Fertility, Contraception, Incontinence and Pregnancy

34. Arthritis, Vasculitis and Bone Disease

35. Diabetes in Cystic Fibrosis

36. Challenges for Nurses

37. Dietetics

38. Physiotherapy

39. Psychological Interventions

40. The Future

41. Author Index

42. Subject Index

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Tags: Andrew Bush, Eric W F W Alton, Jane C Davies, Uta Griesenbach, Adam Jaffe, Cystic Fibrosis, 21st Century, Progress, Respiratory Research