Schlüter B, Buschatz D, Trowitzsch E, Aksu F, Andler W
Vestische Kinderklinik Datteln, Universität Witten-Herdecke, Germany.
Eur J Pediatr. 1997 Jan;156(1):65-8. doi: 10.1007/s004310050555.
Physiological parameters of infants and children with Prader-Willi syndrome were examined in order to clarify whether there were indicators of disturbed respiratory control mechanisms in the pre-obesity stage of the syndrome. From January 1993 to March 1995 in eight patients with Prader-Willi syndrome (five boys, three girls, aged 6 weeks-12.5 years), polysomnography was performed and compared with 28 children matched for gestational age, sex, birth weight and age at sleep study. The recordings included thoracic and abdominal breathing movements, nasal airflow, tcPO2, tcPCO2, oxygen saturation, EEG, EOG and ECG. Respiratory responses to hypercapnia during quiet sleep were obtained from five Prader-Willi patients and ten peers. The Prader-Willi group showed an increased number of apnoeas per hour of sleep, a decreased nadir of oxygen saturation, increased maximum of the instantaneous heart rate and decreased respiratory responses to hypercapnia during quiet sleep.
These findings indicate a primary disturbance of central respiratory control in patients with Prader-Willi syndrome which may be worsened by the development of obesity.
对普拉德-威利综合征患儿的生理参数进行了检查，以明确在该综合征肥胖前期是否存在呼吸控制机制紊乱的指标。1993年1月至1995年3月，对8例普拉德-威利综合征患者（5名男孩，3名女孩，年龄6周-12.5岁）进行了多导睡眠图检查，并与28名在胎龄、性别、出生体重及睡眠研究时年龄相匹配的儿童进行比较。记录包括胸腹部呼吸运动、鼻气流、经皮血氧分压（tcPO2）、经皮二氧化碳分压（tcPCO2）、血氧饱和度、脑电图（EEG）、眼电图（EOG）和心电图（ECG）。从5例普拉德-威利综合征患者和10名同龄人中获取了安静睡眠时对高碳酸血症的呼吸反应。普拉德-威利组显示每小时睡眠呼吸暂停次数增加、血氧饱和度最低点降低、瞬时心率最大值增加以及安静睡眠时对高碳酸血症的呼吸反应降低。
这些发现表明普拉德-威利综合征患者存在中枢呼吸控制的原发性紊乱，肥胖的发展可能会使其恶化。
