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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Hemophilia Group | The sample population consists of hemophilia patients aged 6-18 years who are receiving outpatient treatment, who voluntarily participate in the study and who meet the inclusion criteria. An appropriate sampling method will be used in the selection of the sample. |
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| Measure | Description | Time Frame |
|---|---|---|
| 2-Minute Step Test | 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. | through of the study, average 4 months |
| Measure | Description | Time Frame |
|---|---|---|
| 2-Minute Walk Test (2MWT): | 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 | through of the study, average 4 months |
| Timed Up and Go Test (TUG): |
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Inclusion Criteria:
Exclusion Criteria:
Hemophilia X is a genetically inherited disease, so women are carriers. Symptoms are observed in males.
The sample population consists of hemophilia patients aged 6-18 who are receiving outpatient treatment, who voluntarily participate in the study and meet the inclusion criteria. An appropriate sampling method will be used in the sample selection.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Tuğba GÖNEN, Asisst. Prof. Dr. | Contact | 505 090 58 46 | tugba.badat@hku.edu.tr |
| Name | Affiliation | Role |
|---|---|---|
| Tuğba GÖNEN, Asisst. Prof. Dr. | Hasan Kalyoncu University | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hasan Kalyoncu University | Gaziantep | Şahinbey | (505) 090-5846 | Turkey (Türkiye) |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 33431278 | Result | Nogueira MA, Almeida TDN, Andrade GS, Ribeiro AS, Rego AS, Dias RDS, Ferreira PR, Penha LRLN, Pires FO, Dibai-Filho AV, Bassi-Dibai D. Reliability and Accuracy of 2-Minute Step Test in Active and Sedentary Lean Adults. J Manipulative Physiol Ther. 2021 Feb;44(2):120-127. doi: 10.1016/j.jmpt.2020.07.013. Epub 2021 Jan 9. | |
| 31326830 |
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| ID | Term |
|---|---|
| D006467 | Hemophilia A |
| D002836 | Hemophilia B |
| ID | Term |
|---|---|
| D025861 | Blood Coagulation Disorders, Inherited |
| D001778 | Blood Coagulation Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
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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 |
| through of the study, average 4 months |
| One-Legged Standing Test: | 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 | through of the study, average 4 months |
| Cederberg KLJ, Sikes EM, Bartolucci AA, Motl RW. Walking endurance in multiple sclerosis: Meta-analysis of six-minute walk test performance. Gait Posture. 2019 Sep;73:147-153. doi: 10.1016/j.gaitpost.2019.07.125. Epub 2019 Jul 10. |
| 10960937 | Result | Shumway-Cook A, Brauer S, Woollacott M. Predicting the probability for falls in community-dwelling older adults using the Timed Up & Go Test. Phys Ther. 2000 Sep;80(9):896-903. |
| 24099878 | Result | Eriksen L, Curtis T, Gronbaek M, Helge JW, Tolstrup JS. The association between physical activity, cardiorespiratory fitness and self-rated health. Prev Med. 2013 Dec;57(6):900-2. doi: 10.1016/j.ypmed.2013.09.024. Epub 2013 Oct 5. |
| 32744769 | Result | Srivastava A, Santagostino E, Dougall A, Kitchen S, Sutherland M, Pipe SW, Carcao M, Mahlangu J, Ragni MV, Windyga J, Llinas A, Goddard NJ, Mohan R, Poonnoose PM, Feldman BM, Lewis SZ, van den Berg HM, Pierce GF; WFH Guidelines for the Management of Hemophilia panelists and co-authors. WFH Guidelines for the Management of Hemophilia, 3rd edition. Haemophilia. 2020 Aug;26 Suppl 6:1-158. doi: 10.1111/hae.14046. Epub 2020 Aug 3. No abstract available. |
| D020147 | Coagulation Protein Disorders |
| D006474 | Hemorrhagic Disorders |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D040181 | Genetic Diseases, X-Linked |