Hemolytic Anemia in Disorders of Red Cell Metabolism

ISBN-13: 9781468424591 / Angielski / Miękka / 2012 / 266 str.

Ernest Lindbergh

Opis

I am prepared to predict that this monograph by Dr. Ernest Beutler will long serve as a model for monographs dealing with topics in medical science. I make this bold statement because we encounter in this work a degree of accuracy and authoritativeness well beyond that found in much of the medical literature. Too often, a monograph is simply a review of past reviews. The preparation of an exhaustive and completely accurate study such as the present one is a very laborious task; consequently, many authors make extensive use of the reviews of earlier writers assum- ing that the latter have checked and evaluated each previously published report. Unfortunately, however, this assumption of validity has not al- ways been correct. Dr. Beutler, who is a world authority on the subject about which he writes, was determined to make this book as correct and complete as possible, and, to this end, has checked all the original sources. Nowhere else will such an exhaustive bibliography be found. Moreover, he has also undertaken to reevaluate in the light of current knowledge material pub- lished in earlier days. This he is eminently able to do, and in some in- stances his investigations have resulted in new interpretations. The result is a volume that will be recognized as truly the last word on this important subject.

Spis treści

1 The Red Cell.- 1.1. Red Cell Structure.- 1.2. Red Cell Metabolism.- 1.2.1. Embden-Meyerhoff Pathway.- 1.2.2. Hexose Monophosphate Pathway.- 1.2.3. Other Pathways.- 1.2.4. ATPase Activity and Ion Movements.- 1.2.5. Methemoglobin Reduction.- References.- 2 Glucose-6-Phosphate Dehydrogenase Deficiency.- 2.1. History.- 2.2. Genetics and Population Distribution.- 2.3. Glucose-6-phosphate Dehydrogenase.- 2.3.1. Purification.- 2.3.2. Subunit Structure.- 2.3.3. Kinetic Properties.- 2.3.4. Biochemical Properties of Variants.- 2.4. Physiology and Pathophysiology.- 2.4.1. The Steady State.- 2.4.1.1. Red Cells.- 2.4.1.2. White Cells.- 2.4.1.3. Platelets.- 2.4.1.4. Other Tissues.- 2.4.2. Association of Various Disease States with G-6-PD Deficiency.- 2.4.2.1. Nonhematologic Changes.- 2.4.2.2. Drug-Induced Hemolytic Anemia.- 2.4.2.3. Hemolytic Anemia Induced by Infection.- 2.4.2.4. Hemolytic Anemia in Diabetic Acidosis.- 2.4.2.5. Neonatal Icterus.- 2.4.2.6. Favism.- 2.4.2.6a. The Predisposition to Favism.- 2.4.2.6b. The Active Factor.- 2.4.2.7. Hereditary Nonspherocytic Hemolytic Anemia.- 2.5. Diagnosis.- 2.6. Treatment.- References.- 3 Pyruvate Kinase Deficiency.- 3.1. History.- 3.2. Genetics.- 3.3. Acquired Pyruvate Kinase Deficiency.- 3.4. Pyruvate Kinase.- 3.4.1. The Normal Enzyme.- 3.4.2. Mutant Pyruvate Kinase.- 3.5. The Pyruvate Kinase-Deficient Red Cell.- 3.6. Clinical Manifestations.- 3.7. Diagnosis.- 3.8. Treatment.- References.- 4 Hemolytic Anemia Due to Other Enzyme Deficiencies.- 4.1. Hexokinase Deficiency.- 4.2. Glucose Phosphate Isomerase Deficiency.- 4.3. Phosphofructokinase Deficiency.- 4.4. Aldolase Deficiency.- 4.5. Triose Phosphate Isomerase Deficiency.- 4.6. Phosphoglycerate Kinase Deficiency.- 4.7. Glyceraldehyde Phosphate Dehydrogenase Deficiency.- 4.8. Diphosphoglycerate Mutase Deficiency.- 4.9. Diphosphoglycerate Phosphatase Deficiency.- 4.10. Enolase Deficiency.- 4.11. Defects in Glutathione Synthesis.- 4.12. Glutathione Reductase Deficiency.- 4.13. Glutathione Peroxidase Deficiency.- 4.14. Phosphogluconate Dehydrogenase Deficiency.- 4.15. Disorders of Nucleotide Metabolism.- 4.15.1. ATPase Deficiency.- 4.15.2. Adenylate Kinase Deficiency.- 4.15.3. “High ATP” Syndromes.- 4.15.4. Pyrimidine 5?-Nucleotidase Deficiency.- 4.15.5. Increased Adenosine Deaminase Activity.- References.