RESUMO
STUDY OBJECTIVES: Sleep-disordered breathing (SDB) is prevalent in children with sickle cell disease (SCD) and is associated with worse outcomes. This study aims to compare the outcomes of polysomnography (PSG) performed for pediatric patients with SCD at three United States centers. METHODS: We included children with SCD aged 0-21 who underwent PSG at three American Academy of Sleep Medicine accredited centers: the University of Alabama at Birmingham (UAB), the University of Florida (UF), and Duke University Hospital (DUH), between 2012 and 2022. Descriptive statistics were used as appropriate to compare the baseline characters and PSG outcomes among the different centers. RESULTS: A total of 210 children with SCD from the three centers were included, with comparable sex, SCD genotypes, hemoglobin, hematocrit levels, and chronic transfusion. Children from the different centers exhibited variations in age (P < .001), BMI (P < .05), mean corpuscular volume (P < .05), and hydroxyurea use (P < .05) at the time of the PSG. Overall, the three centers showed significantly different PSG outcomes. Patients from UF had worse obstructive sleep apnea, oxygenation, and periodic leg movement events, together with lower hydroxyurea usage. While those from DUH showed higher hypoventilation and arousal indices. CONCLUSIONS: This multicenter study underscores variations in PSG outcomes among pediatric SCD patients at different centers in the Southeast United States. These findings emphasize the need for standardized approaches to screen for SDB, refer to PSG, and interpret the results in children with SCD. These conclusions may apply to other genetic disorders associated with an increased risk of sleep-disordered breathing.
RESUMO
STUDY OBJECTIVES: Nocturnal hypoxemia is associated with increased risk of sickle cell disease (SCD) complications. The association of nighttime hypoxemia and acute chest syndrome (ACS) in children with SCD has yet to be determined. METHODS: This is a retrospective study of children with SCD who underwent polysomnography at a SCD center. Univariate logistic regression was used to assess the association between nocturnal hypoxemia and ACS admissions. Multivariate logistic regression was performed to verify the effects of different clinical covariates on ACS. Secondary analysis comparing patients with one vs multiple ACS admissions was performed. RESULTS: One hundred ten individuals with SCD who completed their polysomnogram (mean age of 9.4 years) were identified. Fifty-nine (54%) had a history of at least one episode of ACS admission (mean age of 4.1 years), including 40 with multiple episodes. The percentage of total sleep time with O2 saturation < 90% was greater in the ACS group (P < .05). Similarly, mean nocturnal O2 saturation was lower in the ACS group (P < .0005). Mean nocturnal O2 saturation of < 97.3% and the percentage of total sleep time with O2 saturation < 90% higher than 2.7% were associated with ACS. There was no difference in nocturnal hypoxemia between patients with single and multiple ACS admissions. CONCLUSIONS: Nocturnal hypoxemia later in life is associated with previous ACS admissions in children with SCD. This can increase the yield of interpreting polysomnograms in this vulnerable population. Prospective studies are needed to determine the temporal relations of nocturnal hypoxemia and ACS, which may identify a modifiable risk for ACS.