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SC disease

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SC disease
Sickle-hemoglobin C disease A hemoglobinopathy affecting circa 1:800 US blacks, characterized by ↑ infections–eg, bacterial meningitis, Salmonella osteomyelitis due to a defect in the alternate–properdin complement pathway; other effects of SC disease include osteoporosis–resulting in formation of 'fishmouth vertebrae', nephropathy with ↓ renal concentration, acidification, ↑ glomerular filtration rate, retinopathy–in ± 75% of SCD vs 15% of Pts with sickle cell anemia Fundoscopy 'Black sunburst' pattern, due to ↑ glycolysis in end-arterioles; 'seafoam' pattern of proliferative retinopathy Lab ↑ 2,3 DPG, ↑ factor VIII; ↓ osmotic fragility; reticulocytes comprise 5-25% of peripheral RBCs, which have a 'holly-leaf' or navicular shape. See Sickle cell anemia.


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Case Report A 36-year-old black male with a history of hemoglobin SC disease presented with severe left hip pain.
This rare neoplasm has been associated with sickle cell trait in all reported cases but one in which the patient had hemoglobin SC disease.
 
 
 
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