Abstract
Children with sickle cell disease (SCD) are at a high risk for neurocognitive impairment. We aim to quantitatively measure cerebral tissue R2* to investigate the brain iron deposition in children and young adults with SCD in comparison to beta thalassemia major (BTM) and healthy controls and evaluate its impact on neurocognitive functions in patients with SCD. Thirty-two SCD, fifteen BTM, and eleven controls were recruited. Multi-echo fast-gradient echo sequence brain MRI was performed, and brain R2* values of both caudate and thalamic regions were calculated. SCD patients were examined for the neurocognitive functions. SCD had high iron overload 0.30 ± 0.12mg/kg/day. 68.9% of SCD had under-threshold IQ, 12.5% had moderate to severe anxiety, and 60.8% had depression. There were no differences between SCD, BTM, and controls in brain MRI except that left thalamus R2* higher in BTM than both SCD and controls (p = 0.032). Mean right caudate R2* was higher in female than male (p = 0.044). No significant association between brain R2* and LIC or heart R2* values in SCD. Left caudate R2* directly correlate with age and HbS%, and negatively correlate with HbA% while right thalamus R2* negatively correlate with transfusion index and among SCD patients.Conclusion: Neurocognitive dysfunction in SCD could not be explained solely by brain iron overload. What is Known: • Children with sickle cell disease are at great risk of brain damage due to their irregularly shaped red blood cells that can interrupt blood flow to the brain. • There are a number of factors that have negative brain effects that result in learning difficulties, and this not only due to increase brain iron content. What is New: • Assessment of quantitative brain iron content using MRI R2* in children and young adults with SCD in comparison to beta thalassemia major and healthy controls. • Impact of brain iron content on neurocognitive functions of children and young adults with SCD.
Highlights
Anatomical networks are mostly studied using a model-driven seed-based approach with a priori hypotheses of manually selected regions of interest and their connected networks. [24,25] The basal ganglia and thalamus were chosen to be the region of interest (ROIs) as they are known to be the main sources of brain iron accumulation. [26,27]
A 2D T2* multiecho fast gradient echo pulse sequences was used in the current study for assessment of brain iron content, same technique used by Akhlaghpoor and his team [5] while other researchers used a 3D gradient-echo sequence
(31) A decade later, another study confirmed that beta thalassemia major (BTM) patients had significantly lower T2* values in basal ganglia, thalamus and adenohypophysis compared to controls and no correlation between liver T2* values and T2* values of basal ganglia and the thalamus
Summary
Owing to these observations, most recent evaluations of clinical trials to treat children with SCD have included neurocognitive function as a primary or secondary outcome. [6] It is clearly a priority of the pediatric SCD community to address concerns and better understand how neurocognitive function is affected and how to prevent this devastating impact from happening. [7] Primary objective of this work was to measure quantitative brain brain iron content, using MRI R2* values in the caudate and thalamic regions in Egyptian children and young adults with SCD in comparison to beta thalassemia major (BTM) and age and sex-matched healthy controls. While the secondary objectives were to evaluate the impact of brain iron content on neurocognitive functions assessed by neurocognitive examinations and to evaluate its association with the liver and heart iron concentrations
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