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[Screening of cerebral vasculopathy in sickle cell anemia children using transcranial Doppler]
Insights
Systematic transcranial doppler (TCD) screening identifies 18% of children with sickle cell disease (SCD) having abnormal or conditional cerebral vasculopathy, highlighting the need for regular blood transfusions to prevent stroke.
Area of Science:
- Neurology
- Hematology
- Pediatrics
Background:
- Cerebral vasculopathy in sickle cell disease (SCD) significantly increases stroke risk, leading to death and disability.
- Transcranial doppler (TCD) ultrasonography is a key tool for identifying at-risk SCD patients, enabling timely treatment to reduce morbidity and mortality.
Purpose of the Study:
- To assess the prevalence of cerebral vasculopathy using TCD screening in pediatric SCD patients.
- To evaluate the predictive value of hemoglobin levels and TCD findings for stroke risk and disease progression.
- To emphasize the importance of regular blood transfusion programs in managing SCD-related neurological complications.
Main Methods:
- Systematic TCD screening was performed on 572 pediatric SCD patients (aged 1-17 years) in Bamako, Mali.
- Patients included various SCD genotypes: SS, SC, S/β(0), and S/β(+) thalassemia.
- Abnormal or conditional TCD findings were analyzed in relation to hemoglobin levels and clinical outcomes, with follow-up assessments.
Main Results:
- Abnormal or conditional TCD was detected in 18% of 541 steady-state pediatric SCD patients.
- Homozygous SS patients had the highest prevalence (8.1%) of abnormal TCD; S/β(+) thalassemia patients showed no abnormal findings.
- Lower hemoglobin concentrations correlated significantly with abnormal or conditional TCD; hemoglobin changes predicted TCD status at follow-up.
Conclusions:
- Systematic TCD screening is crucial for monitoring cerebral vasculopathy in pediatric SCD patients.
- Regular blood transfusion programs are essential for managing stroke risk, especially in resource-limited settings.
- Early detection and intervention via TCD and transfusions can significantly improve outcomes for SCD patients.
Abstract:
Cerebral vasculopathy exposes patients to a high risk of stroke, a major complication of sickle cell disease (SCD) associated with a high risk of death and disability. Transcranial doppler (TCD) ultrasonography used to identify SCD patients at risk of stroke may contribute to significantly reducing morbidity and mortality in these patients by indicating appropriate treatment. From March 2008 to February 2013, we conducted systematic screening for cerebral vasculopathy using TCD in 572 SCD patients (including 375 SS, 144 SC, 26 S/β(0), and 27 S/β(+) thalassemia patients) aged 1-17 years in a comprehensive center for follow-up and research on sickle cell disease in Bamako, Mali. After exclusion of 30 inadequate results and one case of abnormal TCD observed in a multiple organ failure patient, we found an abnormal or conditional TCD in 18% of 541 children examined in a steady state. The highest prevalence of abnormal cases concerned homozygous SS patients (8.1%). No case of abnormal or conditional TCD was observed in children with S/β(+) thalassemia. Hemoglobin concentrations were significantly lower in patients with conditional or abnormal TCD (P<0.01). In a subgroup of 68 patients with conditional TCD, nine (13%) converted to abnormal TCD over 1 year. In this subgroup of 68 conditional TCD patients, a decrease or increase in baseline hemoglobin concentration was predictive of conditional or abnormal TCD at the follow-up visit. Progression towards conditional TCD was observed in four patients (0.9%) who initially had normal TCD. Children with abnormal TCD had, whenever possible, a monthly exchange transfusion program. One case of transient stroke in the context of P. falciparum malaria with low hemoglobin concentration and one death were observed. These findings highlight the need for systematic TCD in sickle cell disease monitoring and implementing regular blood transfusion programs in the context of limited access to regular and secure blood transfusions.
