Alpha-1-antitrypsin Deficiency: Biology, Diagnosis, Clinical Significance, and Emerging Therapies is the authoritative reference on AATD, providing standards for diagnosis, monitoring, treatment and appropriate avenues of research.
The book covers the disease from basic biology and epidemiology, to clinical impact, and includes the understanding of the natural history of the disease and the significant advances that have been made in the last 20 years, including the three-dimensional structure of the molecule, its broad biological activity and improved therapeutic options, including replacement therapy and gene therapy.
The editors have recruited international experts in the field to contribute evidence-based chapters and insights on future developments in the understanding of this disease.
1. Cover - p. i 2. Title page - p. iii 3. Table of Contents - p. iii 4. Copyright - p. ii 5. List of Contributors - p. iv 6. Preface - p. xii 7. Acknowledgments - p. xiv 8. Chapter 1: Historical Perspective - p. xv 9. Chapter 2: The Swedish Alpha-1-Antitrypsin Screening Study: What We Have Learnt From Birth to Adult Life - p. 14 10. Chapter 3: Alpha-1-Antitrypsin Gene Regulation - p. 24 11. Chapter 4: Alpha-1-Antitrypsin Variants - p. 35 12. Chapter 5: Alpha1-Antitrypsin: Structure and Dynamics in Health, Disease and Drug Development - p. 47 13. Chapter 6: Novel Biological Functions of A1AT - p. 80 14. Chapter 7: AATD and Lung Disease - p. 100 15. Chapter 8: Alpha-1-Antitrypsin Deficiency Liver Disease - p. 116 16. Chapter 9: Alpha-1-Antitrypsin Deficiency: Epidemiological Studies and Other AATD Associated Diseases - p. 131 17. Chapter 10: Thoracic CT Imaging in AATD - p. 158 18. Chapter 11: Animal Models of Alpha-1-Antitrypsin Deficiency - p. 174 19. Chapter 12: Establishing European Registries for AATD and Clinical Trials for Lung Disease - p. 195 20. Chapter 13: Establishing the USA Registry: Logistics, Impact, and Early Clinical Trials - p. 205 21. Chapter 14: Gene Therapy - p. 211 22. Chapter 15: The Future of Alpha-1-Antitrypsin Research - p. 227 23. Index - p. 232
Alpha-1-antitrypsin Deficiency: Biology, Diagnosis, Clinical Significance, and Emerging Therapies is the authoritative reference on AATD, providing standards for diagnosis, monitoring, treatment and appropriate avenues of research.
The book covers the disease from basic biology and epidemiology, to clinical impact, and includes the understanding of the natural history of the disease and the significant advances that have been made in the last 20 years, including the three-dimensional structure of the molecule, its broad biological activity and improved therapeutic options, including replacement therapy and gene therapy.
The editors have recruited international experts in the field to contribute evidence-based chapters and insights on future developments in the understanding of this disease.
1. Cover - p. i 2. Title page - p. iii 3. Table of Contents - p. iii 4. Copyright - p. ii 5. List of Contributors - p. iv 6. Preface - p. xii 7. Acknowledgments - p. xiv 8. Chapter 1: Historical Perspective - p. xv 9. Chapter 2: The Swedish Alpha-1-Antitrypsin Screening Study: What We Have Learnt From Birth to Adult Life - p. 14 10. Chapter 3: Alpha-1-Antitrypsin Gene Regulation - p. 24 11. Chapter 4: Alpha-1-Antitrypsin Variants - p. 35 12. Chapter 5: Alpha1-Antitrypsin: Structure and Dynamics in Health, Disease and Drug Development - p. 47 13. Chapter 6: Novel Biological Functions of A1AT - p. 80 14. Chapter 7: AATD and Lung Disease - p. 100 15. Chapter 8: Alpha-1-Antitrypsin Deficiency Liver Disease - p. 116 16. Chapter 9: Alpha-1-Antitrypsin Deficiency: Epidemiological Studies and Other AATD Associated Diseases - p. 131 17. Chapter 10: Thoracic CT Imaging in AATD - p. 158 18. Chapter 11: Animal Models of Alpha-1-Antitrypsin Deficiency - p. 174 19. Chapter 12: Establishing European Registries for AATD and Clinical Trials for Lung Disease - p. 195 20. Chapter 13: Establishing the USA Registry: Logistics, Impact, and Early Clinical Trials - p. 205 21. Chapter 14: Gene Therapy - p. 211 22. Chapter 15: The Future of Alpha-1-Antitrypsin Research - p. 227 23. Index - p. 232