Disorders of Hemoglobin: Genetics, Pathophysiology, and Clinical Management

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Martin H. Steinberg, Bernard G. Forget, Douglas R. Higgs, Ronald L. Nagel
Cambridge University Press, 2001 - Medical - 1268 pages
Disorders of Hemoglobin is the first comprehensive reference on the genetic and acquired disorders of hemoglobin in over a decade. It stands as the definitive work on the genetics, pathophysiology, and clinical management of this wide range of disorders. Drs. Steinberg, Forget, Higgs, and Nagel have gathered the absolute world authorities on the science and clinical management of thalassemias, sickle cell disease, and other inherited and acquired hemoglobinopathies to create this authoritative textbook for researchers and clinicians alike. This text is divided into eight distinct sections, and includes coverage of the molecular and genetic basis of hemoglobinopathies and thalassemias, their epidemiology and genetic selection, and the diagnosis and special treatments of ^D*b and ^D*a thalassemias, sickle cell disease, Hb E, unstable hemoglobins, Hb M disorders, and acquired and secondary disorders of hemoglobin. Clinical features of all disorders are anchored to the scientific and pathophysiological events that precede them; providing clinicians with a clear scientific background of the disorders they treat, and scientists with an essential link between their research and its clinical manifestation. Disorders of Hemoglobin is the only single-source reference of its kind for hematologists, internists, pediatricians, clinical investigators, and geneticists worldwide.
 

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Contents

THE MOLECULAR CELLULAR AND GENETIC BASIS
25
Erythropoiesis
52
Nuclear Factors that Regulate Erythropoiesis
72
Molecular Genetics of the Human Globin Genes
117
Molecular and Cellular Basis of Hemoglobin Switching
131
Posttranscriptional Factors Influencing the Hemoglobin Content
146
Molecular Anatomy and Physiology of Hemoglobin
174
Hemoglobins of the Embryo and Fetus and Minor Hemoglobins of Adults
197
Nature and Treatment of the Acute Painful Episode in Sickle Cell Disease
671
Origins Epidemiology and Epistasis
711
Hemoglobin SC Disease and HbC Disorders 756
761
Compound Heterozygous and Other Sickle Hemoglobinopathies
786
Sickle Cell Trait
811
EPIDEMIOLOGY AND GENETIC SELECTION
831
Worldwide Distribution of 3 Thalassemia
861
Geographic Distribution of O Thalassemia
878

THE B THALASSEMIAS
231
Molecular Mechanisms of 3 Thalassemia
252
Clinical Aspects of 3 Thalassemia
277
Strucutural Variants with a 3 Thalassemia Phenotype
342
Hereditary Persistence and Fetal Hemoglobin and 63 Thalassemia
356
OM THALASSEMIA
389
Molecular Mechanisms of 0 Thalassemia
405
Clinical and Laboratory Features of oThalassemia Syndromes
431
The Alpha ThalassemiaMental Retardation Syndrome
470
SICKLE CELL DISEASE
489
Cell Adhesion and Microheology in Sickle Cell Disease
527
Red Cell Membrane in Sickle Cell Disease
550
Polymer Structure and Polymerization of Deoxyhemoglobins
577
Clinical Aspects of Sickle Cell Anemia in Adults and Children
611
Geographic Heterogeneity of Sickle Cell Disease
895
DIAGNOSIS AND SPECIAL TREATMENTS
907
DNABased Diagnosis of Hemoglobin Disorders
941
Prenatal Diagnosis and Screening for Thalassemia and Sickle Cell Disease
958
Transfusion and Iron Chelation Therapy in Thalassemia
979
Bone Marrow Transplantation in 3 Thalassemia
1052
Prospects for Gene Therapy of Sickle Cell Disease and Thalassemia
1084
Experimental Therapies for Sickle Cell Anemia and 3 Thalassemia
1119
OTHER INHERITED DISORDERS OF HEMOGLOBIN 1137
1139
Disorders of Hemoglobin Function and Stability
1155
Native and Recombinant Mutant Hemoglobins of Biological Interest
1195
The Dyshemoglobins
1214
Index
1235
Copyright

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