Respiratory involvement in ambulant and non-ambulant patients with facioscapulohumeral muscular dystrophy
Respiratory involvement in ambulant and non-ambulant patients with facioscapulohumeral muscular dystrophy
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DOI：
10.1007/s00415-017-8525-9
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发表时间：
2017-06-01
影响因子：
6
通讯作者：
Evangelista, Teresinha
中科院分区：
文献类型：
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作者：
Moreira, Sandra;Wood, Libby;Evangelista, Teresinha
了解FSHD呼吸损害的发生和预测因素。收集了100例FSHD患者的数据，包括人口统计学、遗传学、呼吸状态和肺功能检查、临床表现和临床严重程度量表（CSS）评分。使用CSS将患者分为两个严重程度组：轻度（评分= 3.5）。如果用力肺活量（FVC）低于预测值的50%，则将其归类为重度受损。使用IBM SPSS Statistics 23进行统计分析，检验是双尾的，显著性水平设定为5%。94例患者可进行肺功能测定; 41.5%的患者结果异常，38.3%的患者存在限制性模式。FVC、CSS评分和D4 Z4片段长度之间存在相关性，早发组、中/重度疾病和D4 Z4片段中重度呼吸系统受累的概率较高
Understand the occurrence and predictors of respiratory impairment in FSHD. Data from 100 FSHD patients was collected regarding demographics, genetics, respiratory status and pulmonary function tests, clinical manifestations and Clinical Severity Scale (CSS) scores. Patients were assigned to two severity groups using CSS: mild (scores = 3.5). Forced Vital Capacity (FVC) was classified as severely impaired if less than 50% of the predicted. Statistical analysis was performed using IBM SPSS Statistics 23, tests were two-tailed and the level of significance set at 5%. Spirometry was available for 94 patients; 41.5% had abnormal results with a restrictive pattern in 38.3% patients. There was a correlation between FVC; CSS score and D4Z4 fragment length with a higher probability of severe respiratory involvement in the early onset group, moderate/severe disease and D4Z4 fragments