Hemophilia, Hemophilia A, Hemophilia B
Conditions
Keywords
Hemophilia, Muscle tone, Stiffness, Elasticity
Brief summary
The aim of our study is to evaluate the viscoelastic properties of lower extremity muscles in patients with hemophilia A and hemophilia B secondarily to compare them with their healthy peers.
Detailed description
Hemophilia is a rare inherited coagulation disorder that develops as a result of factor VIII (hemophilia A) or factor IX (hemophilia B) deficiency, and is a chronic group of diseases that mainly manifests with intra-articular (hemarthrosis) and intramuscular (hematoma) bleeding, affecting quality of life. The presence of easy ecchymosis formation in early childhood, especially intra-articular and intramuscular spontaneous bleeding, interventions and a history of bleeding that lasted longer than expected after trauma should suggest hemophilia. The severity of bleeding findings is directly related to the degree of deficiency of hemophilia A and B. Hemarthroses due to bleeding in the joint (80%) and hematomas due to intramuscular bleeding (20%) cause joint degeneration and muscle atrophy. The joint in which hemarthrosis is most common is the knee joint, however, bleeding into the lower extremity muscles seriously affects the activities of daily living and lower extremity functionality. For this reason, the researchers aimed to evaluate the viscoelastic properties of lower extremity muscles in hemophilia patients and, secondarily, to compare them with their healthy peers.
Interventions
None listed
Sponsors
Hasan Kalyoncu University
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
MALE
Age
4 Years to 17 Years
Healthy volunteers
Yes
Inclusion criteria
* Children aged 4-17 years * Those who have been diagnosed with Hemophilia A and Hemophilia B by a physician * No history of acute bleeding * Children who have family consent and volunteer to participate in the study
Exclusion criteria
* Those with a history of lower extremity surgery * Those with neurological disease * Those with a history of lower extremity hemarthrosis or hematoma in the last 1 month * Patients with inhibitor positive
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Lower extremity moscle tone (Hz) | through of the study, average 6 months | The tone (Hz) of lower extremity muscles (Vastus Medialis Obliquus, Rectus Femoris, Vastus Lateralis, Biceps Femoris, Tibialis Anterior, Gastrocnemius) will be evaluated with MyotonPro device (Myoton Ltd). Vastus Medialis Obliquus, Rectus Femoris, Vastus Lateralis and Tibialis Anterior muscle measurements will be made from the reference points determined in the supine relaxed position, while Biceps Femoris and Gastrocnemius measurements will be made in the prone position with the ankle in the neutral position. Participants will be allowed to rest on the table for 10 minutes before the measurements are taken. The viscoelastic measurement will be repeated three times by determining the reference points (cm) of the relevant muscles and the averages will be recorded. Only measurements with a coefficient of variation less than 3% will be taken into account, otherwise the measurements will be repeated. |
| Lower extremity stiffness (N/m) | through of the study, average 6 months | The stiffness (N/m) of lower extremity muscles (Vastus Medialis Obliquus, Rectus Femoris, Vastus Lateralis, Biceps Femoris, Tibialis Anterior, Gastrocnemius) will be evaluated with MyotonPro device (Myoton Ltd). Vastus Medialis Obliquus, Rectus Femoris, Vastus Lateralis and Tibialis Anterior muscle measurements will be made from the reference points determined in the supine relaxed position, while Biceps Femoris and Gastrocnemius measurements will be made in the prone position with the ankle in the neutral position. Participants will be allowed to rest on the table for 10 minutes before the measurements are taken. The viscoelastic measurement will be repeated three times by determining the reference points (cm) of the respective muscles and the averages will be recorded. Only measurements with a coefficient of variation less than 3% will be taken into account, otherwise the measurements will be repeated. |
| Lower extremity elasticity (log) | through of the study, average 6 months | The elasticity (log) of lower extremity muscles (Vastus Medialis Obliquus, Rectus Femoris, Vastus Lateralis, Biceps Femoris, Tibialis Anterior, Gastrocnemius) will be evaluated with MyotonPro device (Myoton Ltd). Vastus Medialis Obliquus, Rectus Femoris, Vastus Lateralis and Tibialis Anterior muscle measurements will be made from the reference points determined in the supine relaxed position, while Biceps Femoris and Gastrocnemius measurements will be made in the prone position with the ankle in the neutral position. Participants will be allowed to rest on the table for 10 minutes before the measurements are taken. The viscoelastic measurement will be repeated three times by determining the reference points (cm) of the respective muscles and the averages will be recorded. Only measurements with a coefficient of variation less than 3% will be taken into account, otherwise the measurements will be repeated. |
Countries
Turkey (Türkiye)
Contacts
Primary ContactTuğba GÖNEN, Asst. Prof.
Outcome results
None listed