Sickle cell disease : a new vision for an old problem / Darío Acuña-Castroviejo and Iryna Rusanova, editors.

Includes bibliographical references and index.

Print version record.

SICKLE CELL DISEASE ; Library of Congress Cataloging-in-Publication Data ; Contents; Preface; Chapter I Epidemiology of Sickle Cell Disease (SCD); Abstract; Introduction; Genetics and Prevalence of SCD in American and European Continents; Neonatal Screening of SCD; General Review of Pathophysiology of SCD; Cation Homeostasis and Sickle Red Blood Cell (SS-RBCS) Dehydration; An Enhanced Adhesion of the Sickle Red Blood Cell (SS-RBCS) to the Endothelial Cells; The Participation of Inflammatory Phenomenon.

A Global Activation of All the Present Cells in the Vessel, through Signaling Pathways, Susceptible to Oxidative Stress, Hypoxia and InflammationA Complex Endothelial Dysfunction Involving Abnormalities of the Metabolism of Nitric Oxide (NO(); Oxidative Stress in SCD; The Cyclic Nature of SCD; Conclusion; References; Chapter II The Phenotype Diversity and Hematological Status in Sickle Cell Anemia; Abstract; Introduction; Clinical Features of SCD; Spleen; Central Nervous System; Bones and Joints; Eye; Genitourinary System; Pulmonary System; Asthma in SCD; Liver.

Major Clinical Complications of SCDAcute Painful Episodes; Cerebrovascular Accident; Acute Chest Syndrome; Pulmonar Hypertension; Infection; Aplastic Crisis; Laboratory Findings; Treatment; Hydroxyurea; Transfusion and Chelation; Hematopoietic Stem Cell Transplant; Conclusion; References; Chapter III Pathophysiological Consequences of Hemolysis in Sickle Cell Disease; Abstract; Hemolysis; Hemolysis and Pulmonary Hypertention; Hemolysis and Vascular Occlusion; Hemolysis and Priapism; Hemolysis and Thrombosis; References; Chapter IV Genotypes of Sickle Cell Disease; Abstract; Introduction.

Sickle Cell TraitSickle Cell Anemia; Sickle Cell -Ý-Thalassemia; Genotype S- thal(+); Genotype S- Thal(0); Sickle Cell -- Abnormal Hemoglobin Variant; Genotype Sickle cell-Hb_C; Genotype Sickle cell-Hb_E; Genotype Sickle cell-Hb_D-Los Angeles; The Current Trend toward Genomic Analysis; Haplotypes of the Globin Gene-Like Cluster in SCD; Relationships between Haplotypes of the Ý-Globin Gene Cluster and SCD Phenotype; Genetic Modulation of Fetal Hemoglobin; Cis-Acting Factors at the Beta-Globin Gene Cluster; Factors Located on Different Chromosomes with a Trans Effect; BCL11A.

HBS1L-MYB Intergenic RegionKLF1; Effect of -Thalassemia on Clinical Complications of SCD; Conclusion; References; Chapter V Vasculopathy, Chronic Oxidative Stress and Endothelial Dysfunction in SCD; Abstract; Introduction; Increased Oxidative Stress; Decreased Antioxidant Capacity; Oxidant Cell Damage; Markers of Oxidative Stress and Cell Damage; Targeted Therapies; Conclusion; References; Chapter VI Biology of Nitric Oxide Relevant to Sickle Cell Disease; Abstract; Introduction; The Biology of Nitric Oxide; Functions of NO; Vasodilation; Anti-Adhesion; Immune System; Neurotransmission.

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