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Pneumococcal and other infections in children with sickle-cell hemoglobin C (SC) disease
Insights
Children with sickle cell-hemoglobin C (SC) disease face significantly higher risks of serious infections, particularly respiratory infections, compared to those with normal hemoglobin (AA) genotype. Early splenomegaly is linked to increased infection frequency in SC disease patients.
Area of Science:
- Pediatrics
- Hematology
- Infectious Diseases
Background:
- Sickle cell-hemoglobin C (SC) disease is a hemoglobinopathy that may affect immune function.
- Understanding infection patterns in pediatric SC disease is crucial for clinical management.
Purpose of the Study:
- To compare the incidence and patterns of infection in children with SC disease versus those with normal hemoglobin (AA) genotype.
- To identify risk factors for serious infections in children with SC disease.
Main Methods:
- Prospective cohort study comparing 139 children with SC disease and 250 children with AA genotype from birth to six years.
- Analysis included infection incidence rates and survival curve analysis.
Main Results:
- Children with SC disease had significantly higher rates of serious infections compared to the AA genotype group.
- Respiratory infections and gastroenteritis were most common; only respiratory infections were significantly more frequent in SC disease.
- Pneumococcal bacteremia occurred exclusively in the SC disease group; early splenomegaly was associated with higher infection rates.
Conclusions:
- Children with SC disease are at substantially increased risk for serious infections, especially respiratory infections.
- Early splenomegaly is a clinical indicator of heightened infection risk in pediatric SC disease.
- Pneumococcal infections represent a significant threat to children with SC disease.
Abstract:
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.