An observational study in Haemophilia A, Synovitis and Hemophilia Arthropathy, sponsored by University Hospital, Bonn. Recruiting at 1 site in Germany. Open to male participants aged 18 Years and older. Per ClinicalTrials.gov, last updated 2024-04-16.
Sponsored by University Hospital, Bonn · Observational
The aim of this study is to evaluate the prevalence of synovitis in adult patients with haemophilia A.
The bleeding disorder haemophilia causes recurrent spontaneous bleedings primarily into the joints and soft tissues. The reason is a congenital lack of factor VIII (haemophilia A) or factor IX (haemophilia B). In Germany, the most affected joints are the ankle, knee, and elbow. The joint bleedings are accompanied by pain and an inflammatory process that leads to synovitis and ultimately to degenerative joint changes resulting in haemophilic arthropathy: The synovial tissue is responsible for removing blood residuals from the joint cavity. However, blood components, especially iron, induce several changes in the synovial tissue. The resulting synovitis leads to damage of cartilage, bone cysts, osteophytes, and joint effusions. Synovitis can even become a bleeding-independent process, especially after repeated bleeding. According to current knowledge, there is no option to prevent synovitis except for preventing bleedings.
In the aftermath of recurrent joint bleedings, various changes in the synovial tissue, the articular cartilage, and the subchondral bone and blood vessels are observed. These changes are accompanied by restricted joint mobility and musculoskeletal dysfunction. In addition to these peripheral structural changes, various studies show that the entire musculoskeletal system is also affected by recurrent joint bleedings, as it adapts to pain.
Improved therapy over the past decades has led to a reduction in major joint bleeding rates. For this reason, it is important to examine whether this has improved the average joint status and how often patients with haemophilia (PwH) are affected by synovitis at all. Sonography is the radiation-free method of choice for the initial determination of synovitis.
In addition to the structural consequences, joint bleedings also lead to pain and reduction in physical performance. When considering the overall process of degenerative joint changes of PwH, it becomes apparent that this also reduces daily mobility and muscle strength. To the best of our knowledge, the effects of the degree of synovitis on pain perception and physical performance have not been evaluated in previous studies.
For this reason, the prevalence of synovitis in PwH and the consequences in relation to the pain situation and physical performance will be investigated.
844 studies on the registry are indexed under Hemophilia A; 138 are open to participants now.
This study's planned enrollment of 300 is above the median of 81 across 310 observational studies indexed under Hemophilia A.
Browse Hemophilia A studies →University Hospital, Bonn is the lead sponsor of 154 studies on the registry; 28 are open to participants now.
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Adult patients (≥ 18 years of age) suffering from moderate (FVIII 1-5 %) or severe (FVIII \<1 %) haemophilia A.
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
Exclusion Criteria:
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
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Investigational drug or device
A drug or device being studied in a clinical trial.
It may be new, or already approved and being studied for another use, dose or form. Criteria often leave out people who took part in another study of one within a recent period, commonly 30 days or five half-lives.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Patients with severe and moderate Haemophilia A (FVIII \< 5 %, ≥ 18 years' old)
Diagnostic Test: Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US); Haemophilia joint health score (HJHS)
Each subject is examined for the presence of synovitis using the aforementioned diagnostics.
Haemophilia early arthropathy detection with ultrasound (HEAD-US)
HEAD-US protocol examines both elbow, knee and ankle joints sonographically and assesses the presence of synovitis or joint damage from 0-8 points per joint according to Martinoli et al. 2013. A "total score" of 0 to 48 points is achievable. A higher score means a worse joint status.
Time frame: 20 minutes
The Hemophilia Joint Health Score version 2.1 (HJHS)
Clinical joint examination according to De la Corte-Rodriguez et al. 2020. HJHS protocol examines both elbow, knee and ankle joints using a goniometer for range of motion and axial alignment (0-3 points), pain numerical rating scale (0-10 points), swelling (0-3 points), crepitation (0-2 points) and gait abnormalities (0-4 points). A "total score" of 0 to 124 points is achievable. A higher score means a worse joint status.
Time frame: 45 minutes
PROMIS-29 Questionaire
Patient-reported outcomes are surveyed using short questionnaires with a stable number of items from seven PROMIS sub scales ("depression", "anxiety", "physical functioning", "impairment due to pain", "fatigue", "sleep impairment", "participation in social roles" and "activities"). The measures use a T-score metric in which 50 is the mean of a relevant reference population and 10 is the standard deviation (SD) of that population. For the sub scales "participation in social roles and activities" and "physical functioning", a high score is interpreted as a good result and for the sub scales "anxiety", "depression", "fatigue", "impairment due to pain", and "sleep impairment" a low score is a good result.
Time frame: 10 minutes
Haemophilia Activities List (HAL)
to evaluate to subjective physical performance. A higher scores represent a better functional status. Scores can be calculated for each of ten domains of the HAL and a sum score ("Lying / sitting / kneeling / standing" (8-48 Points), "Functions of the legs" (9-54 Points), "Functions of the arms" (4-24 Points), "Use of transportation" (3-18 Points), "Self care" (5-30 Points), "Household tasks" (6-36 Points), "Leisure activities and sports" (7-42 Points), "Upper Extremity Activities" (9-54 Points), "Basic Lower Extremity Activities" (6-36 Points), "Complex Lower Extremity Activities" (9-54 Points), and "Sum score" (42-252 Points).
Time frame: 10 minutes
Baseline Characteristics
1. General anamnesis questionnaire (diseases, pain medication, surgeries, haemophilia related clinical data, date of birth, weight in kilograms, hight in centimeters. 2. Haemophilia treatment past twelve months (on demand/prophylaxis/non-factor therapy/EHL or SHL therapy) 3. Bleeding events past twelve months (traumatic or spontaneous)
Time frame: 15 minutes
Plan to share: No — The publication of anonymous patient data is not allowed by informed consent.
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