Comparison of Pulmonary Function, Exercise Capacity, and Physical Activity Level Between Children With Hemophilia and Healthy Controls
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 34
- 试验地点
- 1
- 主要终点
- Forced vital capacity
研究概览
简要总结
Hemophilia is a rare X-linked congenital bleeding disorder characterized by deficiency of clotting factor VIII (hemophilia A) or deficiency of factor IX (hemophilia B) with complex diagnosis and management. Participation in physical activity is still limited in children with hemophilia, probably due to protective attitudes of families/patients and avoidance of activity, and possibly also as a result of chronic pain. Exercise capacity has been identified as a protective factor against joint problems in hemophilia. Aerobic fitness is associated with better pulmonary function in children. Chronic pain and decreased range of motion due to recurrent bleeding in joints and muscles in hemophilia may indirectly affect posture and respiratory mechanics, leading to impaired pulmonary function. This study aims to compare pulmonary function, exercise capacity, posture, and physical activity level between children with hemophilia and healthy controls and to investigate the relationship between these parameters.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Control
- 时间视角
- Cross Sectional
入排标准
- 年龄范围
- 8 Years 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- 未提供
排除标准
- 未提供
结局指标
主要结局
Forced vital capacity
时间窗: 1st day
Pulmonary function test using a spirometer will be performed. Forced vital capacity will be recorded.
Forced expiratory volume in one second
时间窗: 1st day
Pulmonary function test using a spirometer will be performed. Forced expiratory volume in one second will be recorded.
Forced expiratory volume in one second/forced vital capacity ratio
时间窗: 1st day
Pulmonary function test using a spirometer will be performed. Forced expiratory volume in one second/forced vital capacity ratio will be recorded.
Peak expiratory flow
时间窗: 1st day
Pulmonary function test using a spirometer will be performed. Peak expiratory flow will be recorded. will be recorded.
Forced mid-expiratory flow (FEF25-75)
时间窗: 1st day
Pulmonary function test using a spirometer will be performed. Forced mid-expiratory flow (FEF25-75) will be recorded. will be recorded.
Hemophilia Joint Health Score
时间窗: 1st day
Hemophilia Joint Health Score is a scoring used by physiotherapists in hemophilia treatment centers to detect early hemophilic arthropathy symptoms and to monitor joint health. HJHS consists of edema (0-3), duration of edema (0-1), muscle atrophy (0-2), crepitus during movement (0-2), loss of flexion (0-3), loss of extension (0-3), joint pain (0-2) and evaluation of muscle strength of knees, ankles and elbows (0-4) and global gait score (0-4). The maximum score is 124, with higher scores indicating worse joint health.
Corbin Posture Rating Scale
时间窗: 1st day
Corbin Posture Rating Scale and observational posture analysis. It is an assessment consisting of lateral and posterior posture analysis. '0' is excellent, 12 and above indicates poor posture.
Exercise capacity
时间窗: 1st day
To assess exercise capacity, a symptom-limited cardiopulmonary exercise test will be performed on a bicycle ergometer using Godfrey protocol. Cardiopulmonary responses and effort perceptions will be recorded. In Godfrey protocol, workload increases are adjusted according to height: workload is increased by 10 W/min for \< 125 cm, by 15 W/min for 125-150 cm and by 20 W/min for \>150 cm.
Physical activity level
时间窗: 1st day
The Physical Activity Questionnaire for Children (PAQ-C) and the Physical Activity Questionnaire for Adolescents (PAQ-A) will be used to assess physical activity levels. The Turkish versions of these assessment tools have been shown to be valid and reliable.
次要结局
未报告次要终点
研究者
Aslihan Cakmak-Onal
Principal Investigator
Hacettepe University