Assessment of Factors That Affected Respiratory Parameters in Mucopolysaccharidoses Patients
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 24
- 试验地点
- 1
- 主要终点
- functional capacity
研究概览
简要总结
Respiratory system affects in mucopolysaccharidosis (MPS) disease. Respiratory system symptoms are seen in almost every MPS patients and respiratory failure is one of the most common causes of death in this population. The aim of the study was to evaluate respiratory muscle strength and endurance, cough effectiveness and functional capacity in MPS patients and to defined the factors that affected the respiratory problem in this.
详细描述
This study was participated MPS patients. Patients' pulmonary function test, functional capacity, respiratory muscle strength and endurance, peak cough flow was assessed and recorded.
Pulmonary function test was assessed with spirometry, functional capacity was evaluated with 3-minute step test and six minute walk test (MWT). Respiratory muscle strength was evaluated mouth pressure measurement (MIP- maximal inspiratory pressure and MEP- maximal expiratory pressure) and Sniff nasal inspiratory pressure (SNIP). Respiratory muscle endurance was assessed Powerbreathe. And cough effectiveness was evaluated peak cough flow.
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
- 7 Years 至 30 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •People who diagnosed Mucopolysaccharidoses disease in Hacettepe University.
- •Cooperation
排除标准
- •Patients who were not walking ability.
结局指标
主要结局
functional capacity
时间窗: 15 minutes
Six minute walk test: Distance walked in six minute was recorded
3 minute step test
时间窗: 5 minutes
number of completed step in 3 minute was recorded
Respiratory muscle strength assessment
时间窗: 15 minutes
Respiratory muscle strength was evaluated by measuring maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) and sniff nasal inspiratory pressure(SNIP). Also dynamic inspiratory muscle strength was evaluated Powerbreathe K5 device.
Respiratory muscle endurance
时间窗: 10 minutes
Pressure time unit: A constant load test was used to evaluate respiratory muscle endurance. Using a PowerBreathe device (HaB International Ltd., Southam, UK), the participants were asked to breathe maintaining a constant threshold (60% of MIP) until task failure, and the test duration was recorded. Results were obtained by multiplying the set pressure by the test duration
Pulmonary functions
时间窗: 15 minutes
PEF (peak expiratory flow) assessment via spirometry
MVV (Maximal ventilatory assesssment)
时间窗: 5 minutes
Maximize ventilation by inhaling and exhaling as quickly and deeply as possible for 15 s via spirometry
cough effectiveness
时间窗: 5 minutes
cough effectiveness assessment was evaluated peak cough flow meter.
次要结局
- heart rate(6 minutes)
- oxygen saturation(6 minutes)
研究者
Kübra Kılıç
Physiotherapist
Hacettepe University