Hemophilia
Conditions
Keywords
Hemophilia, Arthropathy, Range of Motion, Strength, Proprioception, Gait, Quality of Life
Brief summary
Analyze and evaluate the skeletal muscle of patients affected with hemophilia in the Region of Murcia. * Describe the periarticular muscular deficit of the lower limbs, depending on the degree of injury: acute and chronic, or subacute. * Identify the range of motion of joints with higher prevalence of hemophilic arthropathy. * Detect the biomechanical changes of the lower limbs and their impact on gait in patients with hemophilia. * Assess the factors that influence the perception of illness and quality of life of patients with hemophilia.
Detailed description
Descriptive study of joint involvement in hemophilia patients with and without a history of hemarthrosis in lower limbs. Descriptive study of alterations in the strength of the periarticular muscles of lower limbs as a result of acute conditions or the development of chronic articular sequelae. Descriptive study of the biomechanical alterations in weight-bearing joints of lower limbs and their impact on gait in patients with hemophilia. Descriptive study of the relationship between skeletal muscle pathology in patients with hemophilia and their perceptions of it and their quality of life.
Interventions
None listed
Sponsors
Universidad Católica San Antonio de Murcia
Study design
Observational model
COHORT
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
MALE
Age
6 Years to 70 Years
Healthy volunteers
Yes
Inclusion criteria
* Patients with hemophilia A and B * Patients of all ages (from 6 to 70 years). * With or without joint damage clinically diagnosed, and with or without a previous history of hemarthrosis joint load legs. * Residents in the Autonomous Community of the Region of Murcia.
Exclusion criteria
* Patients without ambulation * Patients diagnosed with other congenital coagulopathy (von Willebrand disease, etc..). * Patients with neurological or cognitive impairments that prevent understanding the questionnaires and physical tests.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Assess the quality of life | Screening visit | Quality of life (using the SF-36 and Haemo-QoL-36 questionnaire). |
| Assess the list of activities | Screening visit | List of activities in hemophilia (completing the autocuestionario Hemophilia Activities List). |
| Assess the physical performance | Screening visit | Perception of physical performance (with the HEP-Test-Q questionnaire) |
| Assess the health Profile | Screening visit | Health Profile (using the scale Nottingham health profile) |
| Assess the range of motion | Screening visit | Assess the range of motion of elbow, knee and ankle (using a goniometric evaluation). |
| Assess the muscular strength | Screening visit | Assess the muscular strength of arm, thigh and leg (through measurement of muscle balance). |
| Assess the joint pain | Screening visit | Joint pain (VAS scale with the use of a pressure algometer). |
| Assess the variables of strength | Screening visit | Assessment of variables of vertical strength, medio-lateral and anterior-posterior in jump and incorporation from the sitting position. |
| Assess the peak force | Screening visit | Functional assessment of peak force, force production per unit time and subsequent stabilization in standing (in jump and in the passage from the sitting position to standing position, respectively, using a force platform). |
| Assess the contractile activity | Screening visit | Assessment of miotendinous contractile activity in the patellar tendon and Achilles, in jump (CMJ-stiffness test). |
| Assess the state joint | Screening visit | State joint (applying Hemophilia Joint Health Score questionnaire). |
Secondary
| Measure | Time frame |
|---|---|
| Diagnosis, severity of hemophilia, type of treatment (prophylactic or on-demand) and inhibitors | Screening visit |
| Frequency of joint bleeds in the previous month and bleeding during the assessment | Screening visit |
| Age, sex, weight of the patients with hemophilia | Screening visit |
Countries
Spain
Contacts
Primary ContactRUBÉN CUESTA-BARRIUSO, PhD
Outcome results
None listed