Prospective brain imaging evaluation of children with sickle cell trait: initial observations

Area of Science:

• Neurology
• Hematology
• Pediatrics

Background:

• Sickle cell trait (hemoglobin AS) is common but its long-term effects on the brain in children are not fully understood.
• Previous research has primarily focused on individuals with sickle cell disease, leaving a gap in knowledge regarding asymptomatic carriers.

Purpose of the Study:

• To investigate the association between sickle cell trait and brain abnormalities in asymptomatic children.
• To identify specific neuroimaging findings and vascular changes in children with hemoglobin AS.

Main Methods:

• Prospective magnetic resonance (MR) imaging and MR angiography were conducted on 26 siblings of patients with sickle cell disease.
• Images were reviewed by blinded neuroradiologists to identify abnormalities such as lacunae, white matter volume loss, encephalomalacia, leukoencephalopathy, and arterial changes.
• A quantitative scale was used to assess arterial tortuosity.

Main Results:

• Among 21 children with sickle cell trait, 10% showed mild MR imaging abnormalities and 19% exhibited arterial tortuosity.
• Arterial tortuosity was significantly more prevalent in children with sickle cell trait compared to controls (P =.014).
• A higher percentage of hemoglobin S was significantly associated with the presence of tortuosity (P <.03).

Conclusions:

• The findings suggest a link between sickle cell trait and mild vasculopathy in children.
• Increased hemoglobin S levels appear to be associated with these vascular changes.
• This vasculopathy may contribute to the elevated stroke risk observed in certain populations, such as African Americans.