Sickle Cell Anemia: From Basic Science to Clinical Practice

✍ Scribed by Fernando Ferreira Costa, Nicola Conran (eds.)

• Publisher: Springer International Publishing
• Year: 2016
• Tongue: English
• Leaves: 439
• Edition: 1
• Category: Library

✦ Synopsis

Although sickle cell anemia was the first molecular disease to be identified, its complex and fascinating pathophysiology is still not fully understood. A single mutation in the beta-globin gene incurs numerous molecular and cellular mechanisms that contribute to the plethora of symptoms associated with the disease. Our knowledge regarding sickle cell disease mechanisms, while still not complete, has broadened considerably over the last decades. Sickle Cell Anemia: From Basic Science to Clinical Practice aims to provide an update on our current understanding of the disease’s pathophysiology and use this information as a basis to discuss its manifestations in childhood and adulthood. Current therapies and prospects for the development of new approaches for the management of the disease are also covered.

✦ Table of Contents

Front Matter....Pages i-xv
Hemoglobin: Structure, Synthesis and Oxygen Transport....Pages 1-22
Sickle Cell Anemia: History and Epidemiology....Pages 23-47
Overview of Sickle Cell Anemia Pathophysiology....Pages 49-73
Red Blood Cells and the Vaso-Occlusive Process....Pages 75-90
Leukocytes in the Vaso-Occlusive Process....Pages 91-107
Hypercoagulability and Sickle Cell Disease....Pages 109-127
Cardiovascular Adaptations to Anemia and the Vascular Endothelium in Sickle Cell Disease Pathophysiology....Pages 129-175
Inflammation and Sickle Cell Anemia....Pages 177-211
Clinical Manifestations of Sickle Cell Anemia: Infants and Children....Pages 213-229
Treatment of Childhood Sickle Cell Disease....Pages 231-267
Priapism in Sickle Cell Disease: New Aspects of Pathophysiology....Pages 269-283
Clinical Manifestations and Treatment of Adult Sickle Cell Disease....Pages 285-318
Hemoglobin Sβ Thalassemia, SC Disease and SD Disease: Clinical and Laboratorial Aspects....Pages 319-337
Sickle Cell Disease in Africa and the Arabian Peninsula: Current Management and Challenges....Pages 339-370
Genetic Factors Modifying Sickle Cell Disease Severity....Pages 371-397
Future Perspectives for the Treatment of Sickle Cell Anemia....Pages 399-429
Back Matter....Pages 431-435

✦ Subjects

Hematology; Internal Medicine; Medical Biochemistry; Medicine/Public Health, general