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Assessment of the progression of haemophilic arthropathy in children
- Source :
- Haemophilia : the official journal of the World Federation of Hemophilia. 16(1)
- Publication Year :
- 2009
-
Abstract
- Arthropathy is considered as an irreversible and progressive complication in patients with haemophilia, even in children on prophylaxis. To estimate the progression of haemophilic arthropathy, 85 joints of 24 boys with severe (n = 18) and moderate (n = 6) haemophilia (A: 22, B: 2) were investigated with clinical examination, X-rays and magnetic resonance imaging (MRI) at two time periods (time 0 and 1). Patients' age at time 0 was 10.5 +/- 3.6 years and time elapsed to time 1 was 3.8 +/- 1.4 years. At time 0: all investigated joints had more than three bleeds. Sixteen boys were on secondary prophylaxis for 5.4 +/- 2.8 years. Clinical score (a modification of World Federation of Haemophilia's scale): 2.0 +/- 3.6, X-ray score (Pettersson): 2.1 +/- 2.8, MRI score (Denver): 4.5 +/- 3.8. After the first evaluation, prophylaxis was intensified in 11 children and initiated in four. At time 1: clinical score: 1.5 +/- 3.1, X-ray: 1.7 +/- 2.7, MRI score: 5.1 +/- 4.1. On average, the clinical and X-ray scores showed a significant improvement (26% and 40% of the joints respectively, P < 0.01) and the number of haemarthroses evidenced a threefold reduction from time 0 to 1 (P < 0.01), findings that could be associated with the modification of prophylaxis after time 0. MRI findings showed deterioration in 34% of the joints. Conversely, 14 joints (16.5%) with mild or moderate synovitis without cartilage degradation at time 0 showed an improvement at time 1. The information carried by the three scales could be divided into information shared by the three scores and information specific to each score, thus giving a more complete picture of joint damage caused by bleedings.
- Subjects :
- Male
medicine.medical_specialty
Adolescent
Knee Joint
Radiography
Physical examination
Haemophilia
Hemophilia A
Hemophilia B
Internal medicine
Synovitis
Arthropathy
Elbow Joint
Hemarthrosis
medicine
Humans
Child
Genetics (clinical)
Haemophilic arthropathy
medicine.diagnostic_test
business.industry
Magnetic resonance imaging
Hematology
General Medicine
medicine.disease
Magnetic Resonance Imaging
Surgery
Disease Progression
Joint Diseases
business
Complication
Ankle Joint
Subjects
Details
- ISSN :
- 13652516
- Volume :
- 16
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Haemophilia : the official journal of the World Federation of Hemophilia
- Accession number :
- edsair.doi.dedup.....4c4238931bf934bed1e275f5487487fc