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…
Chapter Outline INTRODUCTION BILIRUBIN METABOLISM Bilirubin Production and Transport Hepatic Uptake of Bilirubin Bilirubin Conjugation Bilirubin Excretion Enterohepatic Circulation of Bilirubin DIAGNOSIS OF HYPERBILIRUBINEMIA Total Serum Bilirubin Measurements Transcutaneous Bilirubinometry…
Chapter Outline DEFINITIONS HISTORY FOLATE Chemistry of Folates Folate Sources, Requirements, and Stores Physiology of Folates Biochemistry of Folates COBALAMIN (VITAMIN B 12 ) Chemistry of Cobalamins Cobalamin Sources, Requirements,…
