| Description |
1 online resource |
| Contents |
Dedication; Preface; Acknowledgments; Contents; Contributors; Chapter 1: Hemoglobin: Structure, Synthesis and Oxygen Transport; 1.1 Human Hemoglobins; 1.1.1 Heme Synthesis; 1.1.2 Globin Synthesis; 1.1.3 Globin Chain Genes; 1.1.4 Regulation of Globin Gene Expression; 1.2 Oxygen Transport; 1.2.1 Functional Aspects of Hemoglobin; 1.2.2 Homotropic Interactions: Heme-Heme Cooperativity; Allosteric Regulation: Symmetry Model-MWC; Allosteric Regulation: Sequential Model-The Adair and KNF Model; Stereochemical Model of Perutz, SK, SKL and TTS; 1.2.3 Heterotropic Interactions: Allosteric Effectors |
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2,3-Bisphosphoglycerate (2,3-BPG) The Bohr Effect; Chloride Anions -- Cl-; Transport of CO2; Effect of Temperature; 1.3 Hemoglobin Variants; 1.3.1 Methemoglobin; References; Chapter 2: Sickle Cell Anemia: History and Epidemiology; 2.1 Natural History; 2.1.1 Introduction; 2.1.2 The Malaria Hypothesis; 2.1.3 Mechanisms of Malaria Protection; 2.1.4 Historical Distribution of the Sickle Mutation; 2.1.5 Origin and Genetic Diversity; 2.1.6 Epistatic Interactions; 2.2 Epidemiology; 2.2.1 Contemporary Geographic Distribution; 2.2.2 Mortality; 2.2.3 Newborn and Population Estimates |
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2.2.4 Screening Programs2.2.5 Future Burden; 2.2.6 Public Health Interventions; References; Chapter 3: Overview of Sickle Cell Anemia Pathophysiology; 3.1 Introduction; 3.2 The Phenotype of Sickle Hemoglobinopathies; 3.3 HbS and the HbS Polymer; 3.3.1 HbF, HbS Polymer, and the Phenotype of Sickle Cell Anemia; 3.4 The Sickle Erythrocyte Membrane; 3.5 Cellular Interactions; 3.6 Hemolytic Anemia and the Nitric Oxide Paradigm; 3.6.1 Subphenotypes of Disease; 3.7 Summary; References; Chapter 4: Red Blood Cells and the Vaso-Occlusive Process; 4.1 The Sickle Red Blood Cell (RBC) |
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4.2 Hemoglobin S Polymerization4.3 Cation Loss and Dehydration; 4.4 Oxidant Injury of the Sickle RBC Membrane; 4.5 Clusters of Band 3; 4.6 Increased Phosphatidylserine (PS) Exposure; 4.7 Membrane Deformability and Irreversibly Sickled Cells (ISC); 4.8 Adhesive Properties of Sickle RBCs; 4.9 Increased Fragility and Microvesiculation; 4.9.1 Intravascular Hemolysis; 4.9.2 Microvesiculation; References; Chapter 5: Leukocytes in the Vaso-Occlusive Process; 5.1 Inflammation in Sickle Cell Disease; 5.1.1 Sickle RBCs Promote Inflammation; 5.1.2 Hypoxia-Reoxygenation Promotes Inflammation |
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5.1.3 Monocytes and iNKT Cells Promote Inflammation5.1.4 Cytokine Profile in SCD; 5.1.5 A "Second Hit" for Vaso-Occlusive Crisis; 5.2 Leukocytes in Sickle Cell Disease; 5.2.1 Leukocyte Count Is a Major Risk Factor for VOC; 5.2.2 Myeloid Growth Factors Are Contraindications in SCD; 5.2.3 Reduction in Neutrophil Count Benefits SCD; 5.3 Adherent Leukocytes in the Vaso-Occlusive Process; 5.3.1 Adherent Leukocytes Interact with Sickle RBCs; 5.3.2 Neutrophil Microdomains Mediate Heterotypic Interactions; 5.3.3 A Multistep and Multicellular Model of Sickle Cell VOC; 5.4 Targeting Vaso-Occlusion |
| 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 |
| Bibliography |
Includes bibliographical references |
| Notes |
Vendor-supplied metadata |
| In |
Springer eBooks |
| Subject |
Sickle cell anemia.
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Biochemistry.
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Anemia, Sickle Cell
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Biochemistry
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biochemistry.
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medicines (material)
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Clinical & internal medicine.
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Biochemistry.
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Medicine: general issues.
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Haematology.
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HEALTH & FITNESS -- Diseases -- General.
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MEDICAL -- Clinical Medicine.
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MEDICAL -- Diseases.
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MEDICAL -- Evidence-Based Medicine.
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MEDICAL -- Internal Medicine.
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Sickle cell anemia
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| Genre/Form |
dissertations.
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Academic theses
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Academic theses.
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Thèses et écrits académiques.
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| Form |
Electronic book
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| Author |
Costa, Fernando Ferreira, editor
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Conran, Nicola, editor
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| ISBN |
9783319067131 |
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3319067133 |
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3319067125 |
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9783319067124 |
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