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Joint scores in hemophilic arthropathy in children: Developing country perspectives
Aritra Guha1, Akash Rai1, Arnab Nandy1
1Pediatric Rheumatology Clinic, Department of Pediatric Medicine, North Bengal Medical College, Darjeeling, India.
Insights
Clinician-assessed joint scores in children with hemophilia correlate with imaging findings and quality of life. The Hemophilia Early Arthropathy Detection with Ultra Sound (HEAD-US) score shows strong association, aiding management.
Area of Science:
- Pediatric Rheumatology
- Hematology
- Radiology
Background:
- Hemophilia, an X-linked recessive coagulopathy, frequently causes recurrent joint bleeding (hemophilic arthropathy).
- Articular and periarticular abnormalities are common sequelae of hemophilic arthropathy.
- Assessing hemophilic arthropathy in children from developing countries requires validated clinico-radiological tools.
Purpose of the Study:
- To comprehensively evaluate clinico-radiological joint scores in pediatric hemophilic arthropathy.
- To assess the impact of these scores on the quality of life in affected children.
- To validate the utility of specific scoring systems in a developing country context.
Main Methods:
- Prospective, descriptive study of children in a pediatric rheumatology clinic.
- Joint physical examination using the Hemophilia Joint Health Score 2.1 (HJHS 2.1).
- Imaging via ultrasonography (USG) using the HEAD-US score and MRI using the MRI DENVER score.
- Quality of life assessment using the Functional Independence Score in Hemophilia (FISH).
- Statistical analysis of clinical, radiological, and functional scores.
Main Results:
- Mean age at presentation was 7.4 years; the knee was the most commonly affected joint.
- Significant correlations (p<0.05) were observed between HJHS 2.1 and USG (0.7086), MRI (-0.8916), and FISH (-0.8607) scores.
- USG and MRI scores showed significant negative correlations with the FISH score (-0.7145 and -0.8326, respectively).
Conclusions:
- The Hemophilia Early Arthropathy Detection with Ultra Sound (HEAD-US) score demonstrated the strongest association with the HJHS 2.1.
- Negative correlations between imaging scores (HEAD-US, MRI DENVER) and the FISH score indicate impaired quality of life with increased arthropathy.
- Consistent joint assessment using scores like HEAD-US can optimize the management of destructive changes in hemophilic arthropathy.
Objective:
Hemophilia is a common X-linked recessive coagulopathy causing recurrent bleeding into the synovial joints and results in articular and periarticular abnormalities. To our knowledge, this is the first comprehensive study aimed at studying the clinico-radiological joint score evaluation in hemophilic arthropathy in children from a developing country and its possible impact on the quality of life.
Methods:
In this hospital-based, prospective, descriptive study, all children presenting to the pediatric rheumatology clinic were studied. The joint physical examination was scored using the Hemophilia Joint Health Score 2.1 (HJHS 2.1). The patients were then subjected to imaging of the most affected joint using ultrasonography (USG) and magnetic resonance imaging (MRI). Detailed USG and MRI radiological evaluation was recorded in the predesigned proforma using the Hemophilia Early Arthropathy Detection with Ultra Sound (HEAD-US) score and MRI DENVER score. The physical quality of life as per Functional Independence Score in Hemophilia (FISH) was noted. The clinical, radiological, and functional scores were analyzed with an appropriate statistical measure.
Results:
The mean age at presentation was 7.4 years (interquartile range 4.9-10), with the knee being the most common joint involved. All of the USG score, MRI score, and FISH score have a significant correlation (p<0.05), with the HJHS 2.1 score with correlation coefficients of 0.7086, -0.8916, and 0.8607, respectively. USG and MRI had a correlation coefficient of -0.7145 and -0.8326 with FISH, respectively.
Conclusion:
The degree of association between HJHS 2.1 score was found to be maximum with HEAD-US score, whereas a negative correlation was seen evaluating FISH score with both HEAD-US and MRI DENVER scores. Use of these scores, specifically HEAD-US score, will result in consistent assessment of hemophilic joints, optimizing the management of the destructive changes.
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