Hemophilia, Muscle Disorder, Postural; Defect
Conditions
Keywords
haemophilia, posture, anti-gravity muscles, joint health
Brief summary
Hemophilia A and B are inherited disorders characterized by deficient or missing coagulation factors VIII or IX, respectively, of which the main long-term clinical manifestation is joint damage. Patients with haemophilia (PwH) are susceptible to clinical joint bleeding that may cause irreversible joint damage. Some degree of damage may already occur after the first haemarthrosis or even in children who never experienced clinically evident joint bleeds. Joints are mechanical systems with a structure strictly related to functioning. Therefore, any alteration in structure may have an impact on function (starting from the primary level of posture and anti-gravity muscles), which might in turn stress the joints and increase the risk of bleeding.The primary aim of this study is to investigate the changes in posture and the mechanical properties of anti-gravity muscles of adolescent PwH. The secondary aim is to determine the effect of joint dysfunction on posture in adolescent PwH.
Interventions
DIAGNOSTIC_TESTPosture Analysis
Posture analysis and assessment of mechanical properties of posture muscles
Sponsors
Cukurova University
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
DIAGNOSTIC
Masking
NONE
Intervention model description
cross-sectional study for two groups
Eligibility
Sex/Gender
MALE
Age
10 Years to 19 Years
Healthy volunteers
Yes
Inclusion criteria
* Adolescent boys with haemophilia A or haemophilia B (for experimental group) and healthy adolescent boys (for control group).
Exclusion criteria
* To be with another congenital coagulopathy such as von Willebrand syndrome. * To be unable to stand without support due to hemophilic arthropathy. * To be having another musculoskeletal problem. * To be having factor VIII or IX level in the plasma above 6%
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Photogrammetry | Baseline | The photogrammetry is a single evaluation method based on the analysis of two-dimensional photographs with a software.The erect-static posture of the participants will be analyzed with photogrammetry method. Firstly, two-dimensional photographs of the participant in the static-erect position will be taken.Afterwards, spinal and extremity angles will be measured in degrees using Kinovea™ software in the two-dimensional photographs transferred to the computer. |
| Myotonometric assessment | Baseline | Myotonometric assessment is a reliable method in which the stiffness, elasticity and tone of the muscles are evaluated with a single measurement method.The mechanical properties (tone, stiffness, and elasticity) of the superficial muscles that affect posture will be evaluated with Myoton Pro™ device (Myoton AS, Tallinn, Estonia). With a single measurement made with Myoton Pro™ device, numerical values reflecting the mechanical properties of the muscle are obtained on the screen of the device. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Haemophilia Joint Health Score | Baseline | Joint health of adolescent patients with haemophilia will be assessed by Haemophilia Joint Health Score (HJHS). The HJHS, is calculated by adding the global gait score to the sum of 6 joint totals. The test could yield a minimum score of 0, which indicates healthy joint status, and a maximum score of 124 points (20 points per joint × 6+ 4 points for gait score), which indicates severe arthropathy in multiple joints |
Countries
Turkey (Türkiye)
Outcome results
None listed