Sickle Cell Disease
Sickle cell disease is one of the 'grand' diseases in the terminology of Victorian pathology, because its widespread and multitudinous manifestations may affect any organ system in the body. It has served as a model of molecular disease being one of the first genetic disorders to be explained at the molecular level. Since publication of the Second Edition, however, further advances have been made. Much has been learnt about the causes of variability in the natural history of the disease and of the reasons why there are such marked variations in the patterns of morbidity and mortality. As Director of The Jamaican Sickle Cell Unit, Graham Serjeant and his colleagues have shown the disease to be extremely variable ranging from a severe clinical course with death in early childhood to mildly affected patients living to the age of 80 years. This extreme variability within a single genotype implies that other factors, both genetic and environmental, must influence expression of the disease. This new edition contains many new references and incorporates the latest thinking on the biology of the disease and the best practice in its management.
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Distribution of sickle cell disease
Nomenclature and genetics of sickle
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AA controls abnormal acid acute chest syndrome adults African aplastic crisis Arch artery associated autopsy avascular necrosis blood bone marrow cardiac cent cerebral Chapter Charache children with sickle chronic Clin common complications crises deficiency diagnosis Diggs effect erythrocytes erythropoiesis exchange transfusion femoral head fetal haemoglobin folate frequency function gallstones gene genetic genotype globin Goldberg Haematol haematological haemoglobin haemoglobin levels haplotype HbA2 HbF levels hemoglobinopathies hepatic homozygous homozygous sickle cell increased infarction infection Jamaican Cohort Study leg ulceration lesions Nigeria normal observations occlusion occur Ophthalmol osteomyelitis painful crisis pathology patients with sickle patients with SS Pediatr plasma platelet pneumococcal population pregnancy prevalence priapism pulmonary red cell renal reported reticulocyte retinal retinopathy salmonella Saudi Serjeant serum sickle cell anemia sickle cell disease sickle cell trait sickle-cell anemia sickle-cell disease spleen splenectomy splenomegaly SS disease SS patients steady-state thalassaemia therapy vascular vaso-occlusive vessels Vichinsky