Preserved Sleep for the Same Level of Respiratory Disturbance in Children with Prader-Willi Syndrome

Abstract

Debate remains as to how to balance the use of recombinant human growth hormone (rhGH) as an important treatment in Prader-Willi syndrome (PWS) with its potential role in obstructive sleep apnea. This single-center, retrospective study assessed differences in overnight polysomnography results between children with and without PWS and changes in respiratory parameters before and after the initiation of rhGH treatment in those with PWS. Compared with age-, sex-, and body-mass-index-matched controls (n = 87), children with PWS (n = 29) had longer total sleep time (434 ± 72 vs. 365 ± 116 min; p < 0.01), higher sleep efficiency (86 ± 7 vs. 78 ± 15%; p < 0.05), and lower arousal events (8.1 ± 4.5 vs. 13.0 ± 8.9 events/h; p < 0.05). Mean oxygen saturation was lower in PWS children (94.3 ± 6.0 vs. 96.0 ± 2.0%; p < 0.05), with no other differences in respiratory parameters between groups. Eleven children with PWS (38%) met the criteria for further analyses of the impact of rhGH; polysomnography parameters did not change with treatment. Compared with other children undergoing polysomnography, children with PWS had more favorable markers of sleep continuity and lower oxygen saturation for the same level of respiratory disturbance. rhGH administration was not associated with changes in respiratory parameters in PWS.

The Impact of Growth Hormone Therapy on Sleep-Related Health Outcomes in Children with Prader–Willi Syndrome: A Review and Clinical Analysis

“…Schaefer et al [32] found a reduction in the severity of respiratory events in children with PWS during GH treatment. Tan et al [33] stated that respiratory parameters did not significantly change with rhGH treatment in children with PWS.…”

“…They went on to conclude that GH therapy improved body composition and resting energy expenditure (REE), thus potentially enhancing sleep quality and lung function. In contrast, Tan et al [33] found that PWS was not associated with an altered risk of OSA and suggested that the risk and severity of OSA were similar for the patients with PWS and the controls. Interestingly, uninterrupted sleep and sleep efficiency (percentage of time spent sleeping compared to total time in bed) were better in the children with PWS than the controls.…”

“…Out of a total of 23 studies, 22 conducted PSG, 2 of which conducted overnight respiratory polygraphy [8,38], while 1 study did not state whether PSG was performed [39], and 13 studies performed follow-up PSG [8,[25][26][27][31][32][33][34]36,37,41,43,45]. A total of 1242 patients with PWS were involved in 22 studies, with a minimum age of 2 months [29] and a maximum age of 63 years [35] in 23 studies.…”

“…Differences in patient age can influence study results in various ways, such as treatment response and PWS characteristics (patient age may reflect different stages of PWS). Age categories vary across studies and are not always explicitly defined: neonates/ infants [29,37], prepubertal [23,31,39], children [33,42,43,45], patients [24,27,30,36] or non-severely obese [28]. In addition, one study administered rhGH therapy or placebo for 6 months, then an alternative intervention for 6 months [25], one study administered rhGH at baseline and after 6 weeks [29], four studies administered rhGH and PSG [8,29,35], six studies administered rhGH [26,27,32,41,42,44], and one study administered rhGH, PSG, and the Multiple Sleep Latency Test (MSLT) [30].…”

“…In addition, one study administered rhGH therapy or placebo for 6 months, then an alternative intervention for 6 months [25], one study administered rhGH at baseline and after 6 weeks [29], four studies administered rhGH and PSG [8,29,35], six studies administered rhGH [26,27,32,41,42,44], and one study administered rhGH, PSG, and the Multiple Sleep Latency Test (MSLT) [30]. Some studies reported specific dosages, such as 0.043 mg/kg/day [25], 0.24 mg/kg/wk [29], 1 mg/m 2 /day [31], 0.23-0.22 mg/kg/wk [41], 0.22 mg/kg/wk [30], 1 mg/m 2 /day [29], 0.25 mg/kg/wk [24], 0.03 mg/kg/day [28], 0.010-0.030 mg/kg/day [43], and 0.028 mg/kg/day [8], while others gave no information [23,27,32,33,35,36,38,42,44].…”