Pneumococcal and other infections in children with sickle-cell hemoglobin C (SC) disease.

The pattern of infection was compared in 139 children with sickle cell-hemoglobin C (SC) disease and in 250 control children with a normal hemoglobin (AA) genotype ascertained at birth and followed prospectively for periods of zero to six years. Both infection incidence rates and survival curve analysis indicated highly significant increases in serious infection among children with SC disease. Respiratory infection and gastroenteritis were the most common infections, but only respiratory infections were significantly more frequent in SC disease. Pneumococcal bacteremia was confined to the SC group. No hematologic differences were apparent between SC patients with and without a history of serious infection, but infection was significantly more common in patients manifesting early splenomegaly.