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Elise Turner

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Objective: Impairment in executive and attentional function is common in youth with sickle cell disease (SCD) without history of stroke, indicating that mechanisms other than infarction contribute to cognitive deficits. Sleep-disordered breathing (SDB) may be a unique contributor due to increased prevalence rates in SCD and well-established associations with executive and attentional impairments in healthy peers. The current study examines associations between SDB and executive and attentional function in a heterogenous pediatric SCD sample. Methods: Participants included 60 youth with SCD ages 8-18 years (M=13.05, SD=3.10) and a caregiver. The Pediatric Sleep Questionnaire (PSQ) was used as a proxy for SDB. Cognition was measured by the Delis Kaplan Executive Function System (DKEFS) Trail Making Test, Psychomotor Vigilance Test (PVT), and the Parent Report Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2). Results: Half of youth (50%) met the clinical cut-off for SDB based on caregiver ratings on the PSQ. Performance based measures of cognition (e.g., DKEFS, PVT) were consistent with impaired performance regardless of stroke status, whereas caregiver ratings on the BRIEF-2 were not consistent with impairment in everyday executive dysfunction. The PSQ significantly predicted the BRIEF-2, F(1, 58)=37.95, p<0.001, R2=0.39, f2=0.64, but not the PVT or DKEFS, p>0.05. Conclusions: Sleep-disordered breathing may contribute to parent report of everyday executive dysfunction in pediatric SCD. Future research is needed to delineate the impact of SDB on cognition across youth with varying stroke status and SDB severity.