Wong Shi-Bing, Yang Mei-Chen, Tzeng I-Shiang, Tsai Wen-Hsin, Lan Chou-Chin, Tsai Li-Ping
Department of Pediatrics, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City 23142, Taiwan.
School of Medicine, Tzu Chi University, Hualien 97004, Taiwan.
Children (Basel). 2022 Jun 17;9(6):912. doi: 10.3390/children9060912.
Obstructive sleep apnea syndrome (OSAS) is one of the most common comorbidities in patients with Prader-Willi syndrome (PWS) and causes significant consequences. This observational study was conducted to investigate the progression of OSAS in pediatric patients with PWS, who had not undergone upper airway surgery, through a longitudinal follow-up of their annual polysomnography results. Annual body mass index (BMI), BMI z-score, sleep efficiency and stages, central apnea index (CAI), obstructive apnea-hypopnea index (OAHI), and oxygen saturation nadir values were longitudinally analyzed. At enrollment, of 22 patients (10 boys and 12 girls) aged 11.7 ± 3.9 years, 20 had OSAS. During the 4-year follow-up, only two patients had a spontaneous resolution of OSAS. The average BMI and BMI z-score increased gradually, but CAI and OAHI showed no significant differences. After statistical adjustment for sex, age, genotype, growth hormone use, and BMI z-score, OAHI was associated with the BMI z-score and deletion genotype. In conclusion, OSAS is common in patients with PWS, and rarely resolved spontaneously. Watchful waiting may not be the best OSAS management strategy. Weight maintenance and careful selection of surgical candidates are important for OSAS treatment in patients with PWS.
阻塞性睡眠呼吸暂停综合征（OSAS）是普拉德-威利综合征（PWS）患者中最常见的合并症之一，并会导致严重后果。本观察性研究旨在通过对未接受过上气道手术的PWS患儿每年多导睡眠图结果进行纵向随访，来调查OSAS的进展情况。对每年的体重指数（BMI）、BMI z评分、睡眠效率和阶段、中枢性呼吸暂停指数（CAI）、阻塞性呼吸暂停低通气指数（OAHI）以及最低血氧饱和度值进行纵向分析。入组时，22名年龄为11.7±3.9岁的患者（10名男孩和12名女孩）中，20名患有OSAS。在4年的随访期间，只有两名患者的OSAS自行缓解。平均BMI和BMI z评分逐渐升高，但CAI和OAHI无显著差异。在对性别、年龄、基因型、生长激素使用情况和BMI z评分进行统计调整后，OAHI与BMI z评分和缺失基因型相关。总之，OSAS在PWS患者中很常见，很少自行缓解。密切观察等待可能不是最佳的OSAS管理策略。维持体重和谨慎选择手术候选人对PWS患者的OSAS治疗很重要。
