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Abnormalities of the central nervous system in very young children with sickle cell anemia
W C Wang1, J W Langston, R G Steen
1Department of Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105-2794, USA.
Insights
Central nervous system (CNS) abnormalities, including brain infarction and stenosis, are present in very young children with sickle cell anemia. These findings suggest a need for early intervention to prevent long-term neurological damage.
Area of Science:
- Neurology
- Pediatrics
- Hematology
Background:
- Sickle cell anemia is a genetic blood disorder.
- Central nervous system (CNS) complications are a major cause of morbidity in sickle cell disease.
- The prevalence of CNS abnormalities in very young, asymptomatic children is not well-defined.
Purpose of the Study:
- To investigate the presence of CNS abnormalities in children with sickle cell anemia (hemoglobin SS) aged 7 to 48 months.
- To assess for brain infarction and cerebral artery stenosis using advanced neuroimaging techniques.
Main Methods:
- Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) were performed on 39 children with sickle cell anemia.
- Children were between 7 and 48 months of age and had no history of clinical stroke.
- Developmental testing was conducted on a subset of patients.
Main Results:
- CNS abnormalities were detected in 11% of asymptomatic children.
- One child had both a silent infarct and arterial stenosis; three others had stenosis.
- Children with a history of seizures showed MRI lesions consistent with infarcts.
Conclusions:
- Very young children with sickle cell anemia exhibit brain infarction and/or stenosis of cerebral arteries.
- These findings are similar to those observed in older children with the disease.
- Further research is needed to determine the incidence of CNS lesions and the necessity of early therapeutic interventions.
Objective:
To determine whether abnormalities of the CNS are present in very young children with sickle cell anemia.
Study Design:
Thirty-nine children with hemoglobin SS between the ages of 7 and 48 months were examined with magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). No child had a history of clinical stroke, although 3 had a history of seizures (2 neonatal). Twenty-one patients underwent developmental testing with the Bayley or McCarthy Scales.
Results:
The overall prevalence of CNS abnormalities in asymptomatic children was 4 of 36 (11%, confidence interval 3, 26%). One patient had a silent infarct observed on MRI and a stenotic lesion on MRA; 3 other patients had stenotic lesions on MRA. The 3 patients who had a history of seizures all had lesions consistent with infarcts on MRI. Of the asymptomatic patients who had psychometric testing, 1 of 18 was developmentally delayed. One of 3 with a history of seizures had mild developmental delay.
Conclusions:
Very young children with sickle cell anemia (and no history of clinical stroke) have infarction in the brain and/or stenosis of major cerebral arteries, similar to those reported in older children. These findings indicate a need for larger studies to define the incidence of CNS lesions in this age group and to determine the need for early therapeutic intervention to prevent CNS sequelae of sickle cell disease.
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