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.

单词	sc disease
释义	SC disease 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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