SEVERITY OF JOINT INVOLVEMENT USING HEMOPHILIA JOINT HEALTH SCORE 2.1 (HJHS) IN CHILDREN SUFFERING FROM HEMOPHILIA A

Abstract

Introduction: Hemophilia A is a rare, recessive X-linked, congenital disorder caused by deficient or defective coagulation factor VIII (FVIII) and the deficiency of this factor leads to excessive bleeding following trauma or injury and spontaneous bleeding episodes, primarily in the joints, muscles, and soft tissues. Worldwide the diagnosis and treatment provisions vary widely. In developing countries, access to health care is very limited and very few people can be tested for hemophilia. Developed countries, on the other hand, use personalized prophylactic therapy to optimize outcomes and work towards zero bleeds. Numerous measuring methods for the evaluation of hemophilic have been developed. One of the most used systems is the Hemophilia Joint Health Score (HJHS) 2.1 given its sensitivity to clinical changes appearing in the joints because of recurrent hemarthrosis. Material and Methods: Severity of joint involvement using Hemophilia joint Health score 2.1 was measured by various parameters. Nine items are assessed to evaluate the status of a joint which include swelling, duration of swelling, muscle atrophy, crepitus of motion, range of motion (extension and flexion loss), joint pain, strength, and gait. The summation of the 9 HJHS items in 6 index joints provides users with a relative indicator of joint health. Lower HJHS score represents superior joint health. Results: A total of 51 children were included in the study. Mean HJHS was highest in 10-14 years group while it was lowest (2.81) in 6-9 years group. It was shown that score increases with age indicating that disability increases with the age. Swelling and flexion loss was contributing highest among the all disability items taken into account for calculation of HJHS. It was also observed that almost half of the score was due to contribution of swelling and flexion loss. It was observed that man HJHS was higher in cases diagnosed after the age of 12 months (2.68) as compared to cases that were diagnosed earlier (7.96). This difference was statistically significant (P=0.049). Conclusion: Comprehensive musculoskeletal assessment for monitoring joint health in hemophilia can be done by HJHS. It showed moderate-to-strong correlation across the age group and cases diagnosed after the age of 12 months. keywords: Hemophilia, HJHS 2.1,musculoskeletal assessment.