Chapter Outline HEREDITARY THROMBOTIC DISORDERS Antithrombin III Deficiency Protein C Deficiency Protein S Deficiency Resistance to Activated Protein C and the Factor V Leiden Mutation Prothrombin G20210A Mutation HYPERHOMOCYSTEINEMIA DYSFIBRINOGENEMIAS…
Chapter Outline DEFINITION AND CLASSIFICATION OF PHAGOCYTES PHAGOCYTE DISTRIBUTION AND STRUCTURE Regulation of Myelopoiesis Granulocytes Mononuclear Phagocytes FUNCTION OF PHAGOCYTES Humoral Mediators of the Inflammatory Response Adhesion and Migration into…
Chapter Outline HEMOGLOBIN STRUCTURE AND FUNCTION Hemoglobin Primary Structure Hemoglobin Secondary Structure Hemoglobin Tertiary Structure The Prosthetic Heme Group Hemoglobin Quaternary Structure Dioxygen Binding Redox Reactions of Hemoglobin Ineffective Erythropoiesis…
Chapter Outline PATHOPHYSIOLOGY Hemoglobin S Mutation Hemoglobin S Polymer Effects on the Erythrocyte Membrane External Membrane Interactions Hemolysis and Nitric Oxide Depletion Acute and Chronic Inflammation Genetic Modifiers DIAGNOSIS Fetus…
Chapter Outline FORMATION OF THE PYRROLE The δ-Aminolevulinate Synthases δ-Aminolevulinate Dehydratase ASSEMBLY OF THE TETRAPYRROLE Porphobilinogen Deaminase Uroporphyrinogen III Synthase MODIFICATION OF THE TETRAPYRROLE SIDE CHAINS Uroporphyrinogen Decarboxylase Coproporphyrinogen Oxidase…
Chapter Outline G6PD IN RED BLOOD CELL METABOLISM Structure and Biochemistry of G6PD Genetics of G6PD Features of G6PD in Red Blood Cells Molecular Basis of G6PD Deficiency Epidemiology of…
Chapter Outline HISTORY EPIDEMIOLOGY MOLECULAR PATHOGENESIS Paroxysmal Nocturnal Hemoglobinuria and Complement Decay Accelerating Factor Membrane Inhibitor of Reactive Lysis Inherited Mutations of CD55 and CD59 Congenital Abnormalities of Genes Involved…
Chapter Outline PHYSIOLOGIC CHEMISTRY OF IRON Iron and Oxidation Iron-Protein Complexes Iron Toxicity ACQUISITION AND DISTRIBUTION OF IRON Iron Absorption Intercellular Iron Transport Intracellular Iron Metabolism ROLE OF IRON IN…
Chapter Outline HEXOKINASE DEFICIENCY Clinical Manifestations Biochemistry Genetics Therapy GLUCOSE PHOSPHATE ISOMERASE DEFICIENCY Clinical Manifestations Biochemistry Genetics and Inheritance Therapy PHOSPHOFRUCTOKINASE DEFICIENCY Clinical Manifestations Biochemistry Genetics and Inheritance Therapy ALDOLASE…
Chapter Outline PRIMARY AUTOIMMUNE HEMOLYTIC ANEMIA Incidence Natural History Clinical Manifestations Routine Laboratory Evaluation Serologic Laboratory Evaluation Differential Diagnosis Characteristics of Erythrocyte Autoantibodies Therapy Pathogenesis of Autoantibody Formation SECONDARY AUTOIMMUNE…
