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 ?
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).
A 36-year-old black male with a history of hemoglobin SC disease presented with severe left hip pain.
In one large series of children with hemoglobin SC disease, only 5% had episodes of acute splenic sequestration.
Case report: splenic infarction and acute splenic sequestration in adults with hemoglobin SC disease.
The hemoglobin SC disease had been first diagnosed during pregnancy.
Black, MD: This 41-year-old African American woman with hemoglobin SC disease had recurrent cough; dyspnea; and chest, back, and leg pain consistent with a vaso-occlusive crisis involving the lungs, vertebral bodies, and long bones of the legs.
The severity of hemoglobin SC disease is somewhere between the severity of sickle cell anemia and that of sickle cell trait.