Comparison of Functional Exercise Capacity, Muscle Oxygenation, Respiratory Muscle Strength, and Physical Activity Level in Pediatric Cystic Fibrosis and Primary Ciliary Dyskinesia
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 88
- 试验地点
- 1
- 主要终点
- Muscle oxygenation (THbrecovery)
研究概览
简要总结
Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are genetic diseases characterized by chronic respiratory tract infections. In both diseases, impaired mucociliary clearance, recurrent respiratory infections, and persistent inflammation lead to progressive deterioration in respiratory function. This condition limits patients' activities of daily living, leading to physical inactivity and exercise intolerance. Functional exercise capacity in patients with CF and PCD is reduced due to increased respiratory load, musculoskeletal involvement, and nutritional deficiencies. In exercise tests involving the upper and lower extremities, both patient groups exhibited significantly lower performance compared to healthy individuals. Muscle oxygenation is particularly reduced in patients with cystic fibrosis and is associated with inadequate oxygen delivery to peripheral muscles, mitochondrial dysfunction, and increased muscle fatigue. Although studies on muscle oxygenation in PCD patients are limited, it is thought to be affected by similar pathophysiological mechanisms. Respiratory muscle strength is weakened in both patient groups due to chronic cough, hyperinflation, and increased respiratory effort. This is particularly evident in a significant decrease in inspiratory and expiratory muscle strength. The number of studies in the literature evaluating muscle oxygenation, respiratory muscle strength, and physical activity levels in patients with CF and PCD is limited. There are no studies comparing muscle oxygenation between patients with CF and PCD.
详细描述
In patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD), lower extremity exercise capacity, skeletal muscle function, respiratory muscle strength, and physical activity levels are limited by various pathophysiological mechanisms. In CF patients, lower extremity exercise capacity is significantly reduced due to ventilation limitation, respiratory muscle fatigue, and mitochondrial dysfunction. Early fatigue findings such as delayed oxygen uptake and lactate accumulation have been reported in lower extremity-specific exercise tests. In PCD patients, respiratory workload increases due to ventilation-perfusion mismatch and impaired mucociliary clearance, which can limit muscle oxygen utilization during exercise. Recent studies have shown that PCD patients have lower resting muscle oxygen saturation compared to healthy individuals, but these values are relatively maintained during exercise. In CF, respiratory muscle strength is weakened, particularly at the diaphragm and intercostal muscles, leading to a decrease in ventilatory reserve during exercise. Similarly, submaximal respiratory muscle fatigue and decreased inspiratory muscle strength have been reported in patients with PCD. Regarding physical activity levels, daily activity levels in both patient groups are significantly lower than in healthy peers, and this has been associated with disease progression, muscle dysfunction, and exercise intolerance. Objectively measured studies in children and adolescents with CF have reported that they fall below the recommended daily activity level, and this inadequacy negatively impacts muscle function over time. A similar tendency toward physical inactivity is also found in PCD patients, and this is considered directly related to exercise capacity. The number of studies in the literature evaluating muscle oxygenation, respiratory muscle strength, and physical activity levels in patients with CF and PCD is limited. There are no studies comparing muscle oxygenation in patients with CF and PCD. The aim of our study was to compare functional exercise capacity, muscle oxygenation, respiratory muscle strength, and physical activity in children with CF, PCD, and healthy children.
研究设计
- 研究类型
- Observational
- 观察模型
- Other
- 时间视角
- Retrospective
入排标准
- 年龄范围
- 6 Years 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •Cystic fibrosis patients;
- •Patients diagnosed with cystic fibrosis according to the American Cystic Fibrosis Association consensus report
- •Between the ages of 6 and 18
- •Clinically stable conditions
- •Primary ciliary dyskinesia patients;
- •Patients diagnosed with primary ciliary dyskinesia according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) guidelines
- •Between the ages of 6 and 18
- •Clinically stable conditions
- •Healthy controls;
- •Agreeing to participate voluntarily in the study
- •Between the ages of 6 and 18
排除标准
- •Uncooperative
- •Orthopedic or neurological disorders that will affect functional capacity
- •Pneumonia or any acute infection
- •Healthy controls;
- •Chronic disease
- •Uncooperative
- •Orthopedic or neurological disorders that will affect functional capacity
研究组 & 干预措施
Patient with Primary Ciliary Dyskinesia
Demographic information (age, gender, education level), physical characteristics (weight, height, BMI (Body Mass Index)), medical history (past and present history, family history, diagnosis, disease duration, smoking and environmental exposure, exacerbation status within the last year, number of hospital admissions and hospitalizations, medications used, parental consanguinity, socioeconomic status, number of siblings diagnosed with primary ciliary dyskinesia (PCD) or, cystic fibrosis (CF) body weight, height, and BMI Z scores were recorded. Pulmonary function, respiratory muscle strength and endurance, functional exercise capacity, muscle oxygenation, and physical activity level were assessed for all individuals.
Patient with Cystic Fibrosis
Demographic information (age, gender, education level), physical characteristics (weight, height, BMI (Body Mass Index)), medical history (past and present history, family history, diagnosis, disease duration, smoking and environmental exposure, exacerbation status within the last year, number of hospital admissions and hospitalizations, medications used, parental consanguinity, socioeconomic status, number of siblings diagnosed with primary ciliary dyskinesia (PCD) or, cystic fibrosis (CF) body weight, height, and BMI Z scores were recorded. Pulmonary function, respiratory muscle strength and endurance, functional exercise capacity, muscle oxygenation, and physical activity level were assessed for all individuals.
Health Controls
Demographic information (age, gender, education level), physical characteristics (weight, height, BMI (Body Mass Index)), body weight, height, and BMI Z scores were recorded. Pulmonary function, respiratory muscle strength and endurance, functional exercise capacity, muscle oxygenation, and physical activity level were assessed for all individuals.
结局指标
主要结局
Muscle oxygenation (THbrecovery)
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Functional Exercise Capacity
时间窗: First Day
The six minute walk test (six-MWT) was used to assess functional exercise capacity. The six-MWT was administered according to the criteria of the American Thoracic Society and the European Respiratory Society. Heart rate at rest, after the test, and at the first minute of recovery were assessed using a heart rate monitor (Polar FTI00, China), blood pressure using a sphygmomanometer (Erka Perfect Aneroid, Germany), oxygen saturation using a portable pulse oximeter (Nonin Onyx Vantage 9590, Minnesota, USA), and respiratory frequency (counting the number of breaths taken per minute). The severity of dyspnea and body and leg fatigue was determined using the modified Borg Scale. The six-MWT was repeated twice. Walking distance was expressed in meters and as a percentage of the predicted value. The best walking distance result was selected for analysis. The percentage of the predicted walking distance values was calculated using the reference equation of Gibbons et al.
Muscle oxygenation (SmO2averaged -max)
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (ΔSmO2averaged)
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (SmO2recovery)
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (Resting muscle oxygen saturation (SmO2rest))
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the six-MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (ΔSmO2)
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (SmO2averaged-min)
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (SmO2recovery-averaged)
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (Minimum muscle oxygen saturation (SmO2min))
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (Maximum muscle oxygen saturation (SmO2max))
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (Resting total hemoglobin level (THbrest))
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded
Muscle oxygenation (Minumum total hemoglobin level (THbmin))
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (Maximum total hemoglobin level (Thbmax))
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (ΔTHb)
时间窗: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
次要结局
- Heart rate(First day)
- Blood pressure(First day)
- Oxygen saturation(First day)
- Breathing frequency(First day)
- Body and leg fatigue(First day)
- Dyspnea(First day)
- Pulmonary function (Forced vital capacity (FVC))(First Day)
- Pulmonary function (Forced expiratory volume in the first second (FEV1))(First Day)
- Pulmonary function (FEV1 / FVC)(First Day)
- Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))(First Day)
- Pulmonary function (Peak flow rate (PEF))(First Day)
- Respiratory Muscle Strength(Second Day)
- Respiratory Muscle Endurance(Second Day)
- Physical Activity Level (Total energy expenditure)(Second Day)
- Physical activity (Active energy expenditure (joule / day))(Second Day)
- Physical activity (Physical activity time (min / day))(Second day)
- Physical activity (Average metabolic equivalent (MET / day))(Second Day)
- Physical activity (Number of steps (steps / day))(Second Day)
- Physical activity (Time spent lying down (min / day) days))(Second Day)
- Physical activity (Sleep time (min / day))(Second Day)
研究者
Meral Boşnak Güçlü
Prof. Dr.
Gazi University