Sleep-related symptoms and sleep-disordered breathing in adult Pompe disease
Sleep-related symptoms and sleep-disordered breathing in adult Pompe disease
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DOI：
10.1111/ene.12582
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发表时间：
2015-02-01
影响因子：
5.1
通讯作者：
Young, P.
中科院分区：
文献类型：
--
作者：
Boentert, M.;Karabul, N.;Young, P.
背景与目的呼吸肌无力是成人庞贝病患者早期死亡的主要原因。它首先表现为夜间高碳酸血症，最终导致睡眠中断。对 65 名成年庞贝病患者的睡眠相关症状以及运动表现、用力肺活量 (FVC) 和呼吸道症状进行了调查。方法患者回答了匹兹堡睡眠质量指数 (PSQI)、Epworth 嗜睡量表、疲劳严重程度量表、鹿特丹九项障碍量表、SF-36 健康相关生活质量问卷和呼吸道症状问卷。对所有患者进行6分钟步行测试并获得FVC。 31 名患者获得了多导睡眠图和血氧饱和度测定结果。结果 60 名患者接受了酶替代治疗，32 名患者接受家庭通气支持。睡眠质量下降非常普遍（PSQI>5；43.1%），并且与白天过度嗜睡（Epworth 嗜睡量表>10；24.6%）和疲劳（疲劳严重程度量表>4；72.3%）相关。 SF-36健康相关生活质量问卷在身体领域有所减少，并且与睡眠质量、FVC和运动表现呈负相关。在有多导睡眠图记录的 17 名非通气患者中，有 11 名存在睡眠呼吸障碍，并且存在夜间氧饱和度下降的持续时间 (SaO(2)
Background and purposeRespiratory muscle weakness is the major cause of early death in patients with adult Pompe disease. It first manifests as nocturnal hypercapnia, eventually leading to sleep disruption. Sleep-related symptoms along with motor performance, forced vital capacity (FVC) and respiratory symptoms were investigated in 65 adult patients with Pompe disease.MethodsPatients answered the Pittsburgh Sleep Quality Index (PSQI), the Epworth Sleepiness Scale, the Fatigue Severity Scale, the Rotterdam Nine-item Handicap Scale, the SF-36 health-related quality of life questionnaire, and a respiratory symptom questionnaire. In all patients, the 6-min walk test was performed and FVC was obtained. Polysomnography and oxycapnometry results were available in 31 patients.ResultsSixty patients received enzyme replacement therapy, and 32 individuals were on home ventilatory support. Reduced sleep quality was highly prevalent (PSQI>5; 43.1%) and correlated with both excessive daytime sleepiness (Epworth Sleepiness Scale>10; 24.6%) and fatigue (Fatigue Severity Scale>4; 72.3%). The SF-36 health-related quality of life questionnaire was reduced in the physical domains, and was inversely correlated with sleep quality, FVC and motor performance. In 11 out of 17 non-ventilated patients with polysomnography records, sleep-disordered breathing was present, and duration of nocturnal oxygen desaturation (SaO(2)