Assessment of Musculoskeletal Function and its Correlation with Radiological Joint Score in Children with Hemophilia A.

Abstract

To evaluate the functional independence of children with hemophilia A and its correlation to radiological joint score. The present cross sectional study was conducted at SPMCHI, SMS Medical College, Jaipur, India. Children in the age group of 4-18 y affected with severe, moderate and mild hemophilia A and with a history of hemarthrosis who attended the OPD, emergency or got admitted in wards of SPMCHI, SMS Medical College were examined. Musculoskeletal function was measured in 98 patients using Functional Independence Score in Hemophilia (FISH) and index joints (joints most commonly affected with repeated bleeding) were assessed radiologically with plain X rays using Pettersson score. The mean FISH score was 28.07 ± 3.90 (range 17-32) with squatting, running and step climbing as most affected tasks. The mean Pettersson score was 3.8 ± 3.2. A significant correlation was found between mean Pettersson score and FISH (r = -0.875, P < 0.001) with knee and elbow having r = -0.810 and -0.861 respectively, but not in case of ankle with r = -0.420 (P 0.174). The FISH and radiological joint (Pettersson's) scores may be extremely useful in the clinical practice in the absence of magnetic resonance imaging (MRI), which is considered very sensitive to detect early joint damage, but at a cost that makes it relatively inaccessible. FISH seems to be a reliable tool for assessment of functional independence in patients with hemophilia A.