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.
References in periodicals archive ?
Chronic granulocytic leukemia in a patient with hemoglobin SC disease.
The above cases represent four different scenarios of SC disease in children with malignancies.
SC disease is usually diagnosed late in the evolution of the pathological process.
The clinical features of SC disease with which most clinicians are familiar include back pain, localised spinal tenderness, motor deficits (weakness/spasticity at or below the level of the lesion), sensory abnormalities and sphincter disturbances.
Some people with this genotype develop Hb SC disease, a variant of sickle cell disease.
Hb SC disease is the least severe of the hemoglobinopathies, and patients generally have less morbidity and mortality than do patients with homozygous Hb SS disease (sickle cell anemia).
Many of the effects of Hb SC disease are similar to those of Hb SS disease.
We encountered a 62-year-old man with hemoglobin (Hb) SC disease.
This method accurately detects glycated HbA, HbS, and HbC (1) and might be predicted to provide accurate values in this patient with SC disease.
Patients with Hb SC disease typically have erythrocyte life spans of -29 days (5).