Multicenter comparative study of polysomnography outcomes in children with the monogenic disorder sickle cell disease.

STUDY OBJECTIVES

Sleep-disordered breathing is prevalent in children with sickle cell disease (SCD) and is associated with worse outcomes. This study aimed to compare the outcomes of polysomnography (PSG) performed for pediatric patients with SCD at 3 US centers.

METHODS

We included patients with SCD aged 0-21 years who underwent PSG at 3 American Academy of Sleep Medicine-accredited centers, the University of Alabama at Birmingham, the University of Florida, and Duke University Hospital, 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 3 centers were included, with comparable sex, SCD genotypes, hemoglobin, hematocrit levels, and chronic transfusion. Children from the different centers exhibited variations in age ( < .001), body mass index ( < .05), mean corpuscular volume ( < .05), and hydroxyurea usage ( < .05) at the time of the PSG. Overall, the 3 centers showed significantly different PSG outcomes. Patients from the University of Florida had worse obstructive sleep apnea, oxygenation, and periodic leg movement events, together with lower hydroxyurea usage, and those from Duke University Hospital showed higher hypoventilation and arousal indices.

CONCLUSIONS

This multicenter study underscores variations in PSG outcomes among pediatric SCD patients at different centers in the southeastern United States. These findings emphasize the need for standardized approaches to screen for sleep-disordered breathing, 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.

CITATION

Alishlash AS, Nourani AR, Lebensburger J, Rothman JA, Ali-Dinar T, Ezmigna D. Multicenter comparative study of polysomnography outcomes in children with the monogenic disorder sickle cell disease. 2025;21(2):297-304.