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Foreword H. Franklin Bunn | |
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Preface | |
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Introduction David Weatherall | |
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The Cellular, Molecular and Genetic Basis of Hemoglobin Disorders: Introduction | |
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The development of hematopoiesis | |
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Erythropoiesis | |
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The normal structure regulation of human globin gene clusters | |
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The normal regulation of globin gene expression | |
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The molecular and cellular basis of developmental globin gene switching | |
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Structure and function of hemoglobin and its dysfunction in sickle cell disease | |
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Hemoglobins of the embryo, fetus and adult | |
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Pathophysiology of Hemoglobin and its Disorders: Introduction | |
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Vascular biology | |
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The erythrocyte membrane | |
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The biology of vascular nitric oxide | |
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Mechanisms and complications of hemolysis in sickle cell disease and thalassemia | |
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The importance of animal models in understanding pathophysiology | |
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+� Thalassemia: Introduction | |
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The molecular basis of alpha thalassemia | |
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The pathophysiology and clinical features of alpha thalassemia | |
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Alpha thalassemia with mental retardation and myelodysplasia | |
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Beta Thalassemia: Introduction | |
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The molecular basis of beta thalassemia | |
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Clinical aspects of beta thalassemia and related disorders | |
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HbE disorders | |
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Sickle Cell Disease: Introduction | |
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Clinical and pathophysiological aspects of sickle cell anemia | |
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Biology of pain and treatment of the sickle cell painful episode | |
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HbSC disease and HbC | |
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Sickle cell trait | |
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Other sickle hemoglobinopathies | |
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Other Clinically Important Disorders of Hemoglobin: Introduction | |
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Unstable hemoglobins; hemoglobins with altered oxygen affinity; hemoglobin M; other variants of clinical and biological interest | |
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Dyshemoglobinemias | |
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Special Topics in Hemoglobinopathies: Introduction | |
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Population genetics and global health burden | |
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Genetic modulation of sickle cell disease and thalassemia | |
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Developments in laboratory methods to detect hemoglobinopathies | |
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New Approaches to the Treatment of Hemoglobinopathies and Thalassemia: Introduction | |
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Transfusion and iron chelation | |
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Induction of HbF | |
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Novel approaches to treatment - (anitoxidants, statins, anti-inflammatory agents, K-Cl and Gardos inhibitors, other transport inhibitors, NO) Carlo Brugnara, Kirkwood A. Pritchard, | |
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Stem cell transplantation Emanuele Angelucci and Mark Walters | |
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Gene therapy | |
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