Abstract:
Brain injury is a common complication of sickle cell anaemia (SCA). White matter
(WM) and cortical and subcortical grey matter (GM), structures may have reduced
volume in patients with SCA. This study focuses on whether silent cerebral infarction (SCI), vasculopathy or anaemia affects WM and regional GM volumes in children living in Africa. Children with SCA (n = 144; aged 5–20 years; 74 male) and
sibling controls (n = 53; aged 5–17 years; 29 male) underwent magnetic resonance
imaging. Effects of SCI (n = 37), vasculopathy (n = 15), and haemoglobin were assessed. Compared with controls, after adjusting for age, sex and intracranial volume,
patients with SCA had smaller volumes for WM and cortical, subcortical and total
GM, as well as bilateral cerebellar cortex, globus pallidus, amygdala and right thalamus. Left globus pallidus volume was further reduced in patients with vasculopathy.
Putamen and hippocampus volumes were larger in patients with SCA without SCI
or vasculopathy than in controls. Significant positive effects of haemoglobin on regional GM volumes were confined to the controls. Patients with SCA generally have
reduced GM volumes compared with controls, although some subcortical regions
may be spared. SCI and vasculopathy may affect the trajectory of change in subcortical GM and WM volume. Brain volume in non-SCA children may be vulnerable to
contemporaneous anaemia
