Abstract Objective Patients with sickle cell disease (SCD) are at risk for neuropsychological deficits. Studies have documented that peripheral oxygen saturation is associated with neurocognitive performance, but the results are inconsistent. Our objective was to examine the associations between oxygen saturation and neurocognitive performance in SCD using several different measurement techniques. Methods A lifetime cohort study of 413 patients ranging from 8–25 years of age (Mean = 14.18, Standard Deviation = 4.81) diagnosed with SCD (60% HbSS) received a neuropsychological evaluation including measures of intelligence, processing speed, and working memory. Analyses were conducted separately based on SCD genotype (HbSS/Sβ0 vs. HbSC/SB+). Oxygen saturation values were examined as predictors of neurocognitive performance based on 1) mean lifetime value, 2) lifetime standard deviation, 3) most recent value (within 3 months from neuropsychological assessment), and 4) change over time. Measurement types were entered into separate multivariable models covarying for age, sex, socioeconomic status and hydroxyurea usage. Results Among patients diagnosed with HbSS/Sβ0genotypes, the lifetime standard deviation in oxygen saturation was associated with working memory (pFDR = 0.004) and the change over time in oxygen saturation was associated with processing speed (pFDR <0.001). There were no associations between oxygen saturation and neurocognitive performance among patients with the SC/SB+ genotypes. Conclusions Associations between oxygen saturation and neurocognitive performance in SCD differ based on when the values are collected and how they are analyzed. Our analyses suggest that the change over time and variability in oxygen saturation is most predictive of neurocognitive performance.
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