Factors Associated With Hypoventilation in the Myotonic Dystrophy, Progressive Profile Over 5 Years
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 发起方
- 入组人数
- 113
- 试验地点
- 6
- 主要终点
- Change of measurement of spirometry
研究概览
简要总结
The aim of this study is to determine the factors associated with alveolar hypoventilation in terms of cognitive impairment, daytime sleepiness, respiratory function, nocturnal respiratory events This evaluation will clarify the clinical phenotypes of respiratory disease in myotonic steinert dystrophy.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Myotonic dystrophy of Steinert genetically confirmed (number of known CTG repeats), in its classical form of adult
排除标准
- •Inability to perform EFR assessment or polysomnography
- •Moderate to severe asthma according to the GINA classification
- •moderate to severe COPD (> stage 1 of the GOLD recommendations)
- •Other significant respiratory pathology
- •Patient already on NIV, CPAP or under oxygen therapy
- •Impossibility of receiving enlightened information
- •Inability to participate in the entire study
结局指标
主要结局
Change of measurement of spirometry
时间窗: every year during 5 years
composite predictive factors of alveolar hypoventilation
Change respiratory force
时间窗: every year during 5 years
composite predictive factors of alveolar hypoventilation to change Inspiratory and expiratory force
Change CO2 response (Read test)
时间窗: every year during 5 years
composite predictive factors of alveolar hypoventilation
Change subjective sleepiness (Epworth)
时间窗: every year during 5 years
composite predictive factors of alveolar hypoventilation
change Krupp's Fatigue Severity Scale (KFSS)
时间窗: every year during 5 years
composite predictive factors of alveolar hypoventilation This is a short questionnaire of 9 questions to which the patient responds on a Likert scale ranging from 1 to 7
change cognitive impairment by Trail Making Test A & B
时间窗: every year during 5 years
composite predictive factors of alveolar hypoventilation Trail Making Test is a neuropsychological test of visual attention and task switching. It consists of two parts in which the subject is instructed to connect a set of 25 dots as quickly as possible while still maintaining accuracy.
change Oximetry
时间窗: every year during 5 years
composite predictive factors of alveolar hypoventilation
次要结局
未报告次要终点