Correlation between musculoskeletal function and radiological joint scores in haemophilia A adolescents

Abstract

The functional independence score in haemophilia (FISH) is a performance-based instrument used to assess musculoskeletal function in patients with haemophilia. We aimed to evaluate the functional independence of haemophilia A adolescents and its correlation to radiological joint scores. A cross-sectional study was carried out on 50 adolescent haemophilia A patients. Musculoskeletal function was assessed using the FISH and individual joints were assessed radiologically using the Pettersson score and MRI scale. The mean age of our patients was 16 ± 1.1 with a mean FISH of 23.32 ± 4.69 (range 13-28) and a mean Pettersson score of 2.32 ± 3.09 (range 0-13) for the knees, 1.86 ± 2.67 (range 0-11) for ankles and 1.42 ± 2.17 (range 0-10) for elbows. The mean MRI score for the knees was 3.92 ± 2.74 (range 0-10) while that for ankles was 3.16 ± 2.64 (range 0-10) and for elbows was 2.34 ± 2.63 (range 0-10). There was highly significant correlation between both radiological joint scores and FISH and between degree of factor VIII deficiency and each of FISH, Pettersson score and MRI score. MRI was superior to conventional radiography in detection of subchondral cyst formation and erosions at joint margins. Given the highly significant correlation with both radiological joint scores, FISH appears to be a reliable tool for assessment of functional independence in adolescents with haemophilia A. MRI is more sensitive than conventional radiography in detection of early joint abnormalities.