Abstract

Arteriopathy on MRA was graded as 0 (none), or as increasing severity grades 1, 2 or 3, by two radiologists blinded to the other data. Results: Despite no significant differences across the degrees of arteriopathy in genotype or hemoglobin, mean overnight SpO2 was higher (p< 0.01) in those with grade 0 (97.0±1.6%) than those with grades 2 (93.9±3.7%) or 3 (93.5±3.0%) arteriopathy (abnormal MRA). Reticulocyte count was lower (p< 0.01) in those with grade 0 (187±136*109/L) than those with grades 1 (466±322*109/L), 2 (450±239*109/L) and 3 (624±293*109/L) arteriopathy. In logistic regression, lower mean overnight SpO2 (Odds ratio 0.51, 95% confidence intervals, CI, 0.32 0.81; p< 0.01) predicted abnormal MRA. Higher reticulocyte count (Odds ratio 1.011, 95%CI 1.005 1.018; p< 0.01) was negatively correlated with mean overnight SpO2 and independently predicted abnormal MRA in a multivariable model, with a trend for an effect of lower mean overnight SpO2. Conclusion: Low nocturnal SpO2 and reticulocytosis are associated with intracranial arteriopathy on MRA in children with SCD. Preventative strategies might reduce stroke risk.

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