Alpha-1-antitrypsin Deficiency

Biology, Diagnosis, Clinical Significance, and Emerging Therapies

Noor Kalsheker, Robert Andrew Stockley

• Publisher: Academic Press
• Year: 2017
• Print ISBN:
  9780128039427
• eBook ISBN:
  9780128039472
• Topic: Biological Sciences
• Subtopic: Human Anatomy & Physiology
• Index: Biological Sciences

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. - Provides documentation of the variations in clinical presentation and pathology in a single reference - Presents new insights by pulling together the advances in the understanding of the structure and function of alpha1-antitrypsin deficiency with the genetic variants that cause the disease - Allows for easy reference for the diagnosis of AATD to lead to better therapeutics

Table of contents

• Cover
• Title page
• Table of Contents
• Copyright
• List of Contributors
• Preface
• Acknowledgments
• Chapter 1: Historical Perspective
• Chapter 2: The Swedish Alpha-1-Antitrypsin Screening Study: What We Have Learnt From Birth to Adult Life
• Chapter 3: Alpha-1-Antitrypsin Gene Regulation
• Chapter 4: Alpha-1-Antitrypsin Variants
• Chapter 5: Alpha1-Antitrypsin: Structure and Dynamics in Health, Disease and Drug Development
• Chapter 6: Novel Biological Functions of A1AT
• Chapter 7: AATD and Lung Disease
• Chapter 8: Alpha-1-Antitrypsin Deficiency Liver Disease
• Chapter 9: Alpha-1-Antitrypsin Deficiency: Epidemiological Studies and Other AATD Associated Diseases
• Chapter 10: Thoracic CT Imaging in AATD
• Chapter 11: Animal Models of Alpha-1-Antitrypsin Deficiency
• Chapter 12: Establishing European Registries for AATD and Clinical Trials for Lung Disease
• Chapter 13: Establishing the USA Registry: Logistics, Impact, and Early Clinical Trials
• Chapter 14: Gene Therapy
• Chapter 15: The Future of Alpha-1-Antitrypsin Research
• Index