Descriptive analysis on sleep-disordered breathing in children with Prader-Willi syndrome

Abstract

Purpose: Sleep-disordered breathing is one of the complicating characteristics in patients with Prader-Willi syndrome (PWS). No de-tailed description and risk factors are suggested on breathing problems during sleep in Korean children with PWS. Methods: We reviewed clinical and sleep-study data in patients with PWS who underwent polysomnography before they took the growth hormone therapy. Results: Of the 27 patients with PWS, 25 (92.6%) had sleep-disordered breathing, of whom 14 showed moderate to severe sleep ap-nea. Obstructive dominance was prevalent (64%), followed by central dominance (24%). The apnea-hypopnea index (AHI) increased with increasing weight-for-height z-score (WHZ) (r=0.50, P=0.009), but did not differ by age. Apnea duration of over 12 months was longer in the patient group than in the infant group (15.1±4.3 seconds vs. 9.4±1.7 seconds, P=0.001) and in the obese than non- obese groups (16.8±4.3 seconds vs. 10.0±2.0 seconds, P=0.003). Desaturation below 70% was more common in the obese than nonobese subjects (3/9 vs. 0/18, P=0.029). Age was not different between the central and obstructive apnea groups, but patients with central apnea tended to be younger than patients with obstructive apnea (median [range]: 8.0 months [6.0-12.0 months] vs. 16.5 months [8.5-79.5 months], P=0.092). In addition, patients with obstructive apnea showed higher AHI (12.8 [5.9-19.2] vs. 3.9 [3.4-4.5], P=0.045). Conclusion: Sleep-disordered breathing is common in PWS children with different intensity and patterns according to age and BMI. Close monitoring of breathing problems during sleep is required in PWS patients. (Allergy Asthma Respir Dis 2021;9:216-224)