Comparison of Pulmonary Function, Exercise Capacity, and Physical Activity Level Between Children with Hemophilia and Healthy Controls

Recruiting

• Conditions: Hemophilia; Healthy Children
• Interventions: Other: No intervention

• Registration Number: NCT06636565
• Lead Sponsor: Hacettepe University

Brief Summary

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.

Detailed Description

Not available

Study Timeline

• Study Start: 2024-07-25
• Status Verified: 2024-10
• Study First Submitted: 2024-10-07
• Study First Submitted QC: 2024-10-09
• Last Update Submitted: 2024-10-09
• Study First Posted: 2024-10-10
• Last Update Posted: 2024-10-10
• Primary Completion: 2026-07-25
• Study Completion: 2026-07-25

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 30

Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Hemophilia	No intervention	Children with hemophilia
Healthy children	No intervention	Healthy children

Primary Outcome Measures

Name	Time	Method
Forced vital capacity	1st day	Pulmonary function test using a spirometer will be performed. Forced vital capacity will be recorded.
Forced expiratory volume in one second	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	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	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)	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	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	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	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	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.

Trial Locations

Locations (1)

Hacettepe University Faculty of Physical Therapy and Rehabilitation; 🇹🇷 Ankara, Turkey