Hemophilia, Reliability and Validity, Step
Conditions
Keywords
Hemophilia A, Hemophilia B, Two minute step test
Brief summary
The aim of our study was to evaluate the psychometric properties of the 2-minute step test in hemophilia patients, assess its intra-rater and inter-rater reliability, and evaluate its convergent validity and construct validity supported by measurements of gait, balance, and functionality.
Detailed description
Hemophilia is a rare inherited bleeding disorder caused by a deficiency of factor (F) VIII or IX. It is a chronic group of diseases that primarily manifests as joint (hemarthrosis) and muscle (hematoma) bleeding, affecting quality of life. Easy bruising in early childhood, especially spontaneous joint and muscle bleeding, and a history of prolonged bleeding after procedures and trauma should raise suspicion of hemophilia. The severity of bleeding symptoms is directly related to the degree of FVIII or FIX deficiency. Although the clinical picture varies depending on the duration and frequency of bleeding, the severity of the disease is classified as severe (\<1 IU/dL), moderate (1-5 IU/dL), and mild (5-40 IU/dL). Hemophilia A occurs in 85% of the population (1 in 5,000 male births), while the rate for Hemophilia B is 15% (1 in 30,000 male births). Recurrent and resistant intra-articular bleeding in hemophilia patients leads to the development of hemophilic arthropathy as a result of progressive changes in the joint and bone. Physical performance is significantly affected, especially after recurrent bleeding in the lower extremities. Physical performance is a fundamental physiological indicator of health and functional fitness.Maintaining adequate physical performance is among the most important parameters for preventing social isolation in hemophilia patients, as it improves walking ability and ensures continued independence in daily living activities, participation in school and social activities. Physical performance in children is generally assessed using tests such as the 6-minute walk test, 10-meter walk test, 2-minute walk test, and Timed Up and Go test. However, these assessments may not be practical in routine clinical settings due to space limitations or the need for specialized equipment. The 2-Minute Step Test (2DAT) is a useful alternative that requires minimal equipment and space. This test measures the number of knee lifts (steps) completed during two minutes of steady walking. In addition to simulating walking, the 2DAT also mimics stair climbing, a fundamental activity of daily living. To ensure the test is suitable for both clinical and research settings, its psychometric properties must be evaluated in the relevant population. However, the validity and reliability of the 2DAT have not yet been sufficiently studied in pediatric hemophilia patients. Given the need for accessible and reliable tools to assess physical performance in hemophilia patients, further research is required on the relationship between the 2DAT and this population. In this context, the aim of our study was to evaluate the psychometric properties of the 2-minute walk test in patients with hemophilia, its intra-rater and inter-rater reliability, as well as its convergent validity and construct validity supported by measurements of walking, balance, and functionality.
Interventions
None listed
Sponsors
Hasan Kalyoncu University
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
MALE
Age
6 Years to 18 Years
Healthy volunteers
Yes
Inclusion criteria
* Diagnosed with Hemophilia (A-B) by a physician, * Aged 6-18 years, * Receiving regular prophylactic treatment, * Inhibitor positive or negative, * No history of lower extremity joint bleeding within the last month, * Individuals who are willing to participate in the study,
Exclusion criteria
* Patients who have undergone surgery on the lower extremities for any reason within the last 6 months, * Patients with neurological sequelae following a history of intracranial hemorrhage, * Patients experiencing cognitive impairment to the extent that they cannot understand the tests, * Patients who indicate they will be unable to participate in follow-up measurements.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 2-Minute Step Test | through of the study, average 4 months | The 2MST is widely used to assess aerobic endurance and physical fitness. In this study, the 2MST protocol developed by Rikli and Jones will be used. Before the test, the midpoint between the anterior superior iliac spine and the top of the patella will be measured for each participant, and the target height for knee elevation will be marked on the wall with adhesive tape for standardization. Upon hearing the verbal cue "Go," participants will begin walking (not running) in place and will lift each knee to reach the target height. The test will be timed with a stopwatch, and the number of steps will be recorded directly by observation by a physical therapist. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| 2-Minute Walk Test (2MWT): | through of the study, average 4 months | The 2MWT is a measure of functional capacity and assesses the total distance an individual can walk within a two-minute period. Participants are asked to walk as safely as possible between two cones placed 15 m apart. The dista |
| Timed Up and Go Test (TUG): | through of the study, average 4 months | The TUG is used to determine functional level and mobility. For the test, the patient sits on a standard chair, stands up from the chair upon the start command, walks as quickly and safely as possible to a marked point 3 meter |
| One-Legged Standing Test: | through of the study, average 4 months | The One-Legged Standing Test will be administered to assess patients' static balance. In the one-legged standing test, while standing with arms hanging down at the sides, each lower extremity will be lifted one at a time, and th |
Countries
Turkey (Türkiye)
Contacts
CONTACTTuğba GÖNEN, Asisst. Prof. Dr.
PRINCIPAL_INVESTIGATORTuğba GÖNEN, Asisst. Prof. Dr.
Hasan Kalyoncu University
Outcome results
None listed