Sleep-Related Breathing Disorders in Prepubertal Children with Prader-Willi Syndrome and Effects of Growth Hormone Treatment

Abstract

Abstract Context: Recently, several cases of sudden death in GH-treated and non-GH-treated, mainly young Prader-Willi syndrome (PWS), patients were reported. GH treatment in PWS results in a remarkable growth response and an improvement of body composition and muscle strength. Data concerning effects on respiratory parameters, are however, limited. Objective: The objective of the study was to evaluate effects of GH on respiratory parameters in prepubertal PWS children. Design: Polysomnography was performed before GH in 53 children and repeated after 6 months of GH treatment in 35 of them. Patients: Fifty-three prepubertal PWS children (30 boys), with median (interquartile range) age of 5.4 (2.1–7.2) yr and body mass index of +1.0 sd score (−0.1–1.7). Intervention: Intervention included treatment with GH 1 mg/m2·d. Results: Apnea hypopnea index (AHI) was 5.1 per hour (2.8–8.7) (normal 0–1 per hour). Of these, 2.8 per hour (1.5–5.4) were central apneas and the rest mainly hypopneas. Duration of apneas was 15.0 sec (13.0–28.0). AHI did not correlate with age and body mass index, but central apneas decreased with age (r = −0.34, P = 0.01). During 6 months of GH treatment, AHI did not significantly change from 4.8 (2.6–7.9) at baseline to 4.0 (2.7–6.2; P = 0.36). One patient died unexpectedly during a mild upper respiratory tract infection, although he had a nearly normal polysomnography. Conclusions: PWS children have a high AHI, mainly due to central apneas. Six months of GH treatment does not aggravate the sleep-related breathing disorders in young PWS children. Our study also shows that monitoring during upper respiratory tract infection in PWS children should be considered.