Validation of Ultrasound as a Diagnostic Tool for Assessment of Hemophilic Arthropathy of Knees and Ankles
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The purpose of this study is to test the external validity of the systemic ultrasound protocol for data acquisition and interpretation, in order to diagnose soft tissue and osteochondral abnormalities in hemophilic children.
Condition or disease
Ultrasound has advantages over MRI as it is less expensive, does not require sedation and is more readily available in centres around the world. Given these characteristics of ultrasound it is an ideal imaging tool for early assessment of hemophilic joints in countries whose access to MRI is limited. Early evaluation of soft tissue changes in young hemophilic patients may lead to earlier interventions and better mid-term and long-term clinical outcomes. By this way the joint function of hemophilic patients can be preserved, saving their joints from severe arthropathy and offering them the possibility of a normal professional life in the society.
Ultrasound findings according to the International Prophylaxis Study Group (IPSG) scale [ Time Frame: Day 1 ]
The readers will mark all the positive findings on a standardized spreadsheet and providing a final score for each component of the scale. Color Doppler findings will be rated as normal (grade 0), mild/moderate (grade 1) or severe (grade 2) synovial hyperemia according to an atlas that shows the intensity of hyperemia, which has been prepared by our group.
Secondary Outcome Measures
MRI findings according to the International Prophylaxis Study Group (IPSG) scale [ Time Frame: Day 1 ]
The readers will interpret the MRI findings in an additive way, i.e., marking all positive findings on a standardized spreadsheet and providing a final score for the soft tissue and osteochondral components of the MRI scale.
X-ray findings according to the Pettersson radiographic system [ Time Frame: Day 1 ]
Each joint will be rated on a 13-point score after the Pettersson system.
Physical Assessment according to the HJHS and FISH scores [ Time Frame: Day 1 ]
Each knee, elbow and ankle joint receives a numeric score, which can be compared to itself over time to determine whether it is showing degeneration due to bleeding. FISH score is a seven item measure of disability used to evaluate change in functional independence over time.
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Ages Eligible for Study:
7 Years to 18 Years (Child, Adult)
Sexes Eligible for Study:
Accepts Healthy Volunteers:
Children attending the Hemophilia clinics at the participating institutions (Universidade de São Paulo, Universidade Federal de São Paulo and Universidade Estadual de Campinas), who have a history of ankle or knee bleed(s), will be recruited to take part in this study.
Diagnosis of hemophilia A (factor VIII deficiency ≤1%) or B (factor IX deficiency ≤1.0%)
Cooperative patients (age of 7 to 18 years old)
Patients under 7 years old who have clinical evidence of arthropathy and imaging studies are required for clinical reasons. In these cases, the procedures may be done under sedation.
Male sex (higher prevalence in males)
History of previous ankle or knee bleed
Co-morbid illness such as juvenile idiopathic arthritis, muscular dystrophy, neuropathic arthropathy that cause osteoarticular findings that may obscure or confound the hemophilia-based joint findings
Active bleed (defined as evidence of a recent bleed with one week of the proposing imaging studies and/or musculoskeletal changes on physical examination suggestive of a bleed within one week of the proposed imaging studies).