Sleep medicine · 2025
Sleep problems in youth with WAGR syndrome.
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Kalish-Schur Gavriela B, Somayaji Mahalakshmi, Meltzer Lisa J, Cielo Christopher M
Аннотация
OBJECTIVES: Children with WAGR syndrome, an ultra-rare genetic condition associated with deletion of the brain-derived neurotrophic factor (BDNF), may be at increased risk for sleep problems, but there are limited data assessing sleep in this population. This study aimed to compare sleep disturbances and sleep-disordered breathing in youth with WAGR syndrome compared to healthy youth.
METHODS: Parents of youth with WAGR syndrome and healthy controls completed an online survey including demographics, the PROMIS Pediatric Sleep Disturbance item bank, and the Pediatric Sleep Questionnaire Sleep-Related Breathing Disorders scale (PSQ-SRBD).
RESULTS: Fifteen youth with WAGR syndrome, median (interquartile range) age 10 (7,11) years and 27 controls 9 (6, 10) years were included. PROMIS Sleep Disturbance T-scores were higher in youth with WAGR syndrome (58.1 [53.3, 66.1]) versus controls (52.5 [44.3, 58.3], p = 0.03) indicating more sleep disturbance. Melatonin was used for sleep in 7 (47 %) youth with WAGR syndrome versus 3 (11 %) controls (p = 0.009). PSQ-SRBD scores were greater in youth with WAGR syndrome (0.52 [0.45, 0.65]) compared to controls (0.09 [0.05, 0.18], p < 0.001); 93 % of youth with WAGR syndrome had elevated PSQ-SRBD scores. One-third of youth with WAGR syndrome reported having a diagnosis of obstructive sleep apnea. No differences in sleep outcomes were identified based on BDNF deletion status in those with WAGR syndrome.
CONCLUSIONS: Sleep disturbances and sleep-disordered breathing are prevalent in youth with WAGR syndrome. Diverse cohorts with clinical data including polysomnography are needed to confirm these findings.
METHODS: Parents of youth with WAGR syndrome and healthy controls completed an online survey including demographics, the PROMIS Pediatric Sleep Disturbance item bank, and the Pediatric Sleep Questionnaire Sleep-Related Breathing Disorders scale (PSQ-SRBD).
RESULTS: Fifteen youth with WAGR syndrome, median (interquartile range) age 10 (7,11) years and 27 controls 9 (6, 10) years were included. PROMIS Sleep Disturbance T-scores were higher in youth with WAGR syndrome (58.1 [53.3, 66.1]) versus controls (52.5 [44.3, 58.3], p = 0.03) indicating more sleep disturbance. Melatonin was used for sleep in 7 (47 %) youth with WAGR syndrome versus 3 (11 %) controls (p = 0.009). PSQ-SRBD scores were greater in youth with WAGR syndrome (0.52 [0.45, 0.65]) compared to controls (0.09 [0.05, 0.18], p < 0.001); 93 % of youth with WAGR syndrome had elevated PSQ-SRBD scores. One-third of youth with WAGR syndrome reported having a diagnosis of obstructive sleep apnea. No differences in sleep outcomes were identified based on BDNF deletion status in those with WAGR syndrome.
CONCLUSIONS: Sleep disturbances and sleep-disordered breathing are prevalent in youth with WAGR syndrome. Diverse cohorts with clinical data including polysomnography are needed to confirm these findings.