
Disorders of Hemoglobin: Genetics, Pathophysiology, and Clinical Management
by Martin H. Steinberg , Bernard G. Forget , Douglas R. Higgs , David J. Weatherall-
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Summary
Table of Contents
Foreword H. Franklin Bunn | |
Preface | |
Introduction David Weatherall | |
The Cellular, Molecular and Genetic Basis of Hemoglobin Disorders: Introduction | |
The development of hematopoiesis | |
Erythropoiesis | |
The normal structure regulation of human globin gene clusters | |
The normal regulation of globin gene expression | |
The molecular and cellular basis of developmental globin gene switching | |
Structure and function of hemoglobin and its dysfunction in sickle cell disease | |
Hemoglobins of the embryo, fetus and adult | |
Pathophysiology of Hemoglobin and its Disorders: Introduction | |
Vascular biology | |
The erythrocyte membrane | |
The biology of vascular nitric oxide | |
Mechanisms and complications of hemolysis in sickle cell disease and thalassemia | |
The importance of animal models in understanding pathophysiology | |
+ Thalassemia: Introduction | |
The molecular basis of alpha thalassemia | |
The pathophysiology and clinical features of alpha thalassemia | |
Alpha thalassemia with mental retardation and myelodysplasia | |
Beta Thalassemia: Introduction | |
The molecular basis of beta thalassemia | |
Clinical aspects of beta thalassemia and related disorders | |
HbE disorders | |
Sickle Cell Disease: Introduction | |
Clinical and pathophysiological aspects of sickle cell anemia | |
Biology of pain and treatment of the sickle cell painful episode | |
HbSC disease and HbC | |
Sickle cell trait | |
Other sickle hemoglobinopathies | |
Other Clinically Important Disorders of Hemoglobin: Introduction | |
Unstable hemoglobins; hemoglobins with altered oxygen affinity; hemoglobin M; other variants of clinical and biological interest | |
Dyshemoglobinemias | |
Special Topics in Hemoglobinopathies: Introduction | |
Population genetics and global health burden | |
Genetic modulation of sickle cell disease and thalassemia | |
Developments in laboratory methods to detect hemoglobinopathies | |
New Approaches to the Treatment of Hemoglobinopathies and Thalassemia: Introduction | |
Transfusion and iron chelation | |
Induction of HbF | |
Novel approaches to treatment - (anitoxidants, statins, anti-inflammatory agents, K-Cl and Gardos inhibitors, other transport inhibitors, NO) Carlo Brugnara, Kirkwood A. Pritchard, | |
Stem cell transplantation Emanuele Angelucci and Mark Walters | |
Gene therapy | |
Table of Contents provided by Publisher. All Rights Reserved. |
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