Sickle Cell DiseaseSickle 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 references and incorporates ideas on the biology of the disease and the best practice in its management. |
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Contents
Basic concepts | 3 |
Distribution of sickle cell disease | 16 |
Nomenclature and genetics of sickle | 31 |
Copyright | |
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Common terms and phrases
abnormal acid activity acute adults African appears Arch areas artery associated blood bone bone marrow cause cent changes Chapter Child chronic Clin clinical common compared complications contribute controls count crises death decreased deficiency diagnosis Diggs early effect erythrocytes et al evidence factors fall frequency function further gene genotype greater Haematol haematological haemoglobin haemoglobin levels homozygous improvement increased indices infarction infection iron Jamaican leg ulceration less levels lower major manifestations mean mechanism months necrosis normal noted observations occur oxygen painful crisis patients with sickle Pediatr population pregnancy prevalence priapism pulmonary red cell reduced relatively renal reported response risk Serjeant severe showed sickle cell anemia sickle cell disease sickle cell trait sickle-cell significant similar splenic SS disease studies subjects suggested syndrome thalassaemia therapy transfusion treatment trial vascular vessels