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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.
List of contents
Chapter 1 Hemoglobin:Structure, Synthesis and Oxygen Transport.-
Chapter 2 Sickle cell Anemia: History and Epidemiology.-
Chapter 3 Overviewof Sickle Cell Anemia Pathophysiology.-
Chapter 4 Red Blood Cells and the Vaso-occlusive Process.-
Chapter5 Leukocytes in the vaso-occlusive process.-
Chapter 6 Hypercoagulability andSickle Cell Disease.-
Chapter 7 Cardiovascular Adaptations to Anemia and theRole of Vascular Endothelium in Sickle Cell Disease Pathophysiology.-
Chapter 8Inflammation and Sickle CellAnemia.-
Chapter 9 Clinical Manifestations of Sickle Cell Anemia: Infants and Children.-
Chapter 10 Treatment of Childhood Sickle Cell Disease.-
Chapter 11 Priapism inSickle Cell Disease: New aspects of pathophysiology.-
Chapter 12 ClinicalManifestations and Treatment of Adult Sickle Cell.-
Chapter 13 Hemoglobin SbetaThalassemia, SC Disease and SD Disease: Clinical and Laboratorial Aspects.-
Chapter 14 Sickle Cell Disease in Africa and the Arabian Peninsula: CurrentManagement and Challenges.-
Chapter 15 Genetic Factorsmodifying sickle cell disease severity.-
Chapter 16 Future Perspectives for theTreatment of Sickle Cell.
About the author
Fernando F. Costa - graduated (1974) and obtained both his MSc (1979) and PhD (1981) degrees from the Ribeirão Preto School of Medicine of the University of São Paulo, where he also served as a faculty member (1985-1989). After completing a postdoctoral fellowship at the Yale School of Medicine (1987-1989), he joined the Department of Internal Medicine of the School of Medical Sciences of the University of Campinas (1990) and became a Full Professor of Hematology and Hemotherapy (1996). Member of several national and international academic societies, Professor Costa has already published 260 papers in peer-reviewed journals and supervised 29 doctoral theses. He received a decoration from the Government of Brazil (2008) and has been awarded a great number of scientific prizes, including one from the Government of the State of São Paulo (2000). At the University of Campinas, Professor Costa served as Dean of the School of Medical Sciences (1994-1998), Director of the Hematology and Hemotherapy Center (1998-2002), Vice-President for Research (2002-2005) and General Coordinator of the University (2005-2009). He was the president of the University of Campinas from April 2009 to April 2013.
Nicola Conran - Research scientist at the University of Campinas, Brazil. Graduated in Biochemistry (University of Birmingham, UK); PhD, University of Nottingham, UK.
Summary
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.
