Background and Purpose-Sickle cell anemia is associated with compromised oxygen-carrying capability of hemoglobin and a high incidence of overt and silent stroke. However, in children with no evidence of cerebral infarction, there are changes in brain morphometry relative to healthy controls, which may be related to chronic anemia and oxygen desaturation. Methods-A whole-brain tract-based spatial statistics analysis was carried out in 25 children with sickle cell anemia with no evidence of abnormality on T2-weighted magnetic resonance imaging (13 male, age range: 8-18 years) and 14 age-and race-matched controls (7 male, age range: 10-19 years) to determine the extent of white matter injury. The hypotheses that white matter damage is related to daytime peripheral oxygen saturation and steady-state hemoglobin were tested. Results-Fractional anisotropy was found to be significantly lower in patients in the subcortical white matter (corticospinal tract and cerebellum), whereas mean diffusivity and radial diffusivity were higher in patients in widespread areas. There was a significant negative relationship between radial diffusivity and oxygen saturation (P<0.05) in the anterior corpus callosum and a trend-level negative relationship between radial diffusivity and hemoglobin (P<0.1) in the midbody of the corpus callosum. Conclusions-These data show widespread white matter abnormalities in a sample of asymptomatic children with sickle cell anemia, and provides for the first time direct evidence of a relationship between brain microstructure and markers of disease severity (eg, peripheral oxygen saturation and steady-state hemoglobin). This study suggests that diffusion tensor imaging metrics may serve as a biomarker for future trials of reducing hypoxic exposure. (Stroke. 2015;46:1793-1799.
Aims Research in populations with sickle cell anaemia (SCA) has consistently found differences from healthy controls on tests of executive function (EF), attributed to the elevated stroke risk. In older adults without SCA there is an association between pain and mental flexibility. The aims of this study were to investigate the executive function performance of a group of SCA children across a single test battery and the possibility that chronic pain impacts upon these skills. Methods Patients with SCA were recruited via their consultants at outpatients clinics. Sibling controls and other ethnically matched children were recruited via the community. The Weschler Abbreviated Scales of Intelligence (WASI): two-sub-test version was administered together with five of the nine available sub-tests of the Delis Kaplan Executive Function System (D-KEFS), representing a range of different skills: the trail-making, the design fluency, colour-word interference, sorting and tower tests. Composite scores were created for each of the DKEFS sub-tests. Current pain was measured using the Faces Pain Scale-Revised (FPS-R). Frequency of pain was measured via self-report of the number of pain episodes over the past year. A number of t-tests, followed by correlations, were then employed to compare performance across the two groups. Results Eight controls (3 male), mean age 11 y 3 m (9 y 8 m-14 y 8 m) and 14 (11 male) children with SCA, mean age 13 y 4 m (10 y 7 m-17 y 6 m) were recruited. There was a significant difference between the frequency of pain episodes in the SCA and control groups, t (20)=−2.56, p=0.019. T-tests showed no significant differences at the 95% confidence interval in FPS-R scores, any of the DKEFS sub-tests, or FSIQ. No significant correlations were found between either frequency of pain episodes or current pain, and any of the composite EF sub-test scores. Discussion We found no evidence of impaired performance by the SCA group on any of the EF sub-tests. The SCA group reported significantly more frequent pain episodes, and a wider range of pain levels on the day of testing but there was no association between current pain, or pain frequency, and EF test performance, perhaps explained by the subjective nature of pain, and differences in pain perception. Abstract G151 Table 1 Transfusion Strategy Mean DE rate (95% CI) Number of babies exposed to >1 donor No. of babies exposed to >2 donors SP wastage (Rounded) 4-SP 2.47 (2.03,2.88) 20 (86.9%) 8 (34.7%) 5 6-SP 2.44 (1.98,2.88) 18 (78.2%) 8 (34.7%) 10 8-SP 2.44 (1.98,2.88) 18 (78.2%) 8 (34.7%) 15
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