Sickle cell anemia : from basic science to clinical practice /: from basic science to clinical practice. ([2016])

• Record Type: Book
• Further Information: Note: Fernando Ferreira Costa, Nicola Conran, editors.
• Editors: Costa, Fernando Ferreira; Conran, Nicola
• 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. 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. 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). 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. 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.
• Publisher Details: Cham : Springer
• Publication Date: 2016
• Extent: 1 online resource
• Subjects: 616.1/527; Medicine; Sickle cell anemia; HEALTH & FITNESS -- Diseases -- General; MEDICAL -- Clinical Medicine; MEDICAL -- Diseases; MEDICAL -- Evidence-Based Medicine; MEDICAL -- Internal Medicine; Sickle cell anemia; Anemia, Sickle Cell; Medicine & Public Health; Hematology; Internal Medicine; Medical Biochemistry; Medicine/Public Health, general; Medical -- Biochemistry; Medical -- General; Clinical & internal medicine; Biochemistry; Medicine: general issues; Hematology; Internal medicine; Biochemistry; Medical -- Hematology; Haematology; Electronic books; Electronic books
• Languages: English
• ISBNs: 9783319067131; 3319067133; 3319067125; 9783319067124
• Related ISBNs: 9783319067124
• Notes: Note: Includes bibliographical references.; Note: Vendor-supplied metadata.
• Access Rights: Legal Deposit; Only available on premises controlled by the deposit library and to one user at any one time; The Legal Deposit Libraries (Non-Print Works) Regulations (UK).
• Access Usage: Restricted: Printing from this resource is governed by The Legal Deposit Libraries (Non-Print Works) Regulations (UK) and UK copyright law currently in force.