Sickle cell anaemia explanation for Medical Student OSCE
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Explain condition – abnormal ? peptide chains results in HbS production. Common in black Africans. Homozygous = sickle cell anaemia; heterozygotes have sickle cell trait – no disability, except in hypoxia, e.g. in anaesthesia – sickling test for all Africans pre-op.
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Pathogenesis – sickle cells haemolyse, and also block small blood vessels
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Early symptoms – anaemia, jaundice, painful swelling of hands and feet. Splenomegaly (but rare at >10yrs). Young: alternating good health and acute crises
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Later symptoms – chronic ill health due to previous crises, with renal failure, bone necrosis, osteomyelitis, leg ulcers, iron overload due to infusions. Lungs: hypoventilations, atelectasis, lung infiltrations
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Sickle cell crises – causes:
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Thrombosis (‘painful crisis’) – common, precipitated by cold, dehydration, infection, ischaemia – e.g. muscle exertion. May mimic acute abdomen or pneumonia, and CNS signs: fits / focal signs.
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Haemolysis (rare)
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Marrow Aplasia – due to parvoviruses – sudden lethargy, pallor, few reticulocytes. Urgent transfusion needed
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Sequestration / hepatic crises – spleen and liver enlarge rapidly from trapped RBCs. Hb very down, LFTs and INR raised. Serious; exchange transfusion may be needed
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Management – analgesia, rehydration, O2, transfusions e.g. when CNS / lung complications
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Chronic disease management
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Chronic blood transfusion programmes, to keep HbS <30% - but high risk of antibody development to red cell antigens
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Marrow transplant may be curative, but controversial
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Children: if febrile, have risk of septicaemia
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Prevention
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Genetic counselling + prenatal tests
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Parental education – prevent deaths from sequestration crises
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by: Admin
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Date: Fri, 22 Jan 2010 Time: 4:20 PM
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