Alpha-1-antitrypsin Deficiency. Biology, Diagnosis, Clinical Significance, and Emerging Therapies

Description

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. Content: Front matter,Copyright,List of Contributors,Preface,AcknowledgmentsEntitled to full textChapter 1 - Historical Perspective, Pages 1-14 Chapter 2 - The Swedish Alpha-1-Antitrypsin Screening Study: What We Have Learnt From Birth to Adult Life, Pages 15-24 Chapter 3 - Alpha-1-Antitrypsin Gene Regulation, Pages 25-35 Chapter 4 - Alpha-1-Antitrypsin Variants, Pages 37-47 Chapter 5 - Alpha1-Antitrypsin: Structure and Dynamics in Health, Disease and Drug Development, Pages 49-80 Chapter 6 - Novel Biological Functions of A1AT, Pages 81-100 Chapter 7 - AATD and Lung Disease, Pages 101-116 Chapter 8 - Alpha-1-Antitrypsin Deficiency Liver Disease, Pages 117-131 Chapter 9 - Alpha-1-Antitrypsin Deficiency: Epidemiological Studies and Other AATD Associated Diseases, Pages 133-158 Chapter 10 - Thoracic CT Imaging in AATD, Pages 159-177 Chapter 11 - Animal Models of Alpha-1-Antitrypsin Deficiency, Pages 179-195 Chapter 12 - Establishing European Registries for AATD and Clinical Trials for Lung Disease, Pages 197-205 Chapter 13 - Establishing the USA Registry: Logistics, Impact, and Early Clinical Trials, Pages 207-211 Chapter 14 - Gene Therapy, Pages 213-227 Chapter 15 - The Future of Alpha-1-Antitrypsin Research, Pages 229-232 Index, Pages 233-244