Sickle Cell DiseaseSickle cell disease was one of the first diseases to be explained at the molecular level. In the years since the first edition of this book was published, further advances have been made, and much has been learned about the causes of variation in the natural history of the disease and in the patterns of morbidity and mortality. The role of genetic modifiers such as alpha-thalassemia and persisting high levels of fetal hemoglobin, and of the beta-globin haplotype, have been recognized and explored. Studies of the disease which compare geographically separated populations have contributed to understanding this variability. These studies have led to more rigorous thinking about the nature of the disease and a clearer appreciation of how to manage it. This new edition contains many new references and incorporates the latest thinking on the biology and management of the disease. |
Contents
Basic concepts | 3 |
Distribution of sickle cell disease | 18 |
Nomenclature and genetics of sickle | 29 |
Copyright | |
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AA controls abnormal acute adults aplastic crisis artery associated autopsy avascular necrosis blood bone marrow cardiac cent cerebral changes Chapter children with SS chronic Clin common complications courtesy of Dr crises decreased diagnosis Diggs effect episodes erythrocytes erythropoiesis factors femoral head folate frequently function g/dl gallstones gene genotype Haematol haematological haemoglobin haemoglobin levels haemorrhage HbF levels hemoglobinopathies hepatic homozygous homozygous sickle cell hypersplenism increased infarction infection Jamaican Cohort Study leg ulceration lesions normal observations occlusion occur osteomyelitis oxygen painful crisis pathology patients with sickle patients with SS Pediatr photo courtesy plasma platelet pneumococcal population Powars pregnancy prevalence priapism pulmonary red cell renal reported reticulocyte retinal salmonella Serjeant serum sickle cell anemia sickle cell disease sickle cell trait sickle-cell sickle-cell anemia sickle-cell disease significantly spleen splenectomy splenomegaly SS disease SS patients steady-state syndrome thalassaemia therapy total haemoglobin transfusion vaccine vascular vaso-occlusive vessels