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Cerebral Vasculopathy in Children With Sickle Cell Disease in an Amazonian Population
Felipe Lima Alcolumbre Tobelem1, Guilherme Augusto Lira Ribeiro de Andrade1, Joelma Karin Sagica Fernandes Paschoal1,2
1Amazônia Neurovascular Research Group, Federal University of Pará, Belém, Brazil.
Insights
Transcranial Doppler effectively screens children with sickle cell disease for cerebral vasculopathy. Many identified patients face a high risk of stroke, necessitating further investigation.
Area of Science:
- Neurology
- Pediatrics
- Hematology
Background:
- Sickle cell disease is a prevalent hereditary condition.
- Cerebral vasculopathy is a serious complication in children with sickle cell disease.
- Early detection of cerebral vasculopathy is crucial for stroke prevention.
Purpose of the Study:
- To evaluate transcranial Doppler as a screening tool for cerebral vasculopathy in pediatric sickle cell disease patients.
- To assess the prevalence of cerebral vasculopathy and associated stroke risk in this population.
Main Methods:
- An epidemiologic, descriptive, cross-sectional study was conducted.
- Patients aged 2-16 years with sickle cell disease underwent transcranial Doppler and complementary examinations.
- Data were collected between January 2014 and March 2020.
Main Results:
- Cerebral vasculopathy was diagnosed in 8.5% (14 of 164) of screened patients.
- Of those diagnosed, 85.7% (12 of 14) were classified as high risk for stroke based on cerebral blood flow velocity.
- Conditional stroke risk was identified in 14.2% (2 of 14) of patients with cerebral vasculopathy.
Conclusions:
- Transcranial Doppler is a valuable screening method for identifying cerebral vasculopathy in children with sickle cell disease.
- Complementary examinations are essential to confirm diagnoses suggested by transcranial Doppler.
- Further research, including genetic studies, is needed to elucidate the link between sickle cell disease and cerebral vasculopathy.
Abstract:
Introduction: Sickle cell disease is the most prevalent hereditary disease in the country. The aim of this study was to use transcranial Doppler as a screening method for identifying cerebral vasculopathy in children with sickle cell disease. Methods: An epidemiologic, descriptive, and cross-sectional study was conducted. Patients aged 2-16 years with sickle cell disease and followed at a neurology referral service between January 2014 and March 2020 underwent transcranial Doppler and complementary examinations to screen for cerebral vasculopathy. Results: Screening and confirmatory examinations diagnosed 14 of 164 patients (8.5%) with cerebral vasculopathy. Regarding stroke risk, as measured by cerebral blood flow velocity, 2 of 14 patients (14.2%) were classified as conditional risk (170-199 cm/s) and 12 of 14 (85.7%) as high risk of stroke. Conclusion: Complementary examinations should be performed in all patients with changes on transcranial Doppler to confirm cerebral vasculopathy. Further studies, particularly genetic, are needed to better understand the relationship between sickle cell disease and cerebral vasculopathy.
