RESUMEN

Hemophilia refers to a group of bleeding disorders in which there is a deficiency of one of the factors necessary for coagulation of the blood. Susceptibility to joint hemorrhage in persons with Hemophilia suggests that the routine assessment of joint health is an important aspect of clinical management and outcome studies assessing the efficacy of treatment. This prospective study was conducted to study joint health status in Hemophilia patients and draw their joint disability score by using Hemophilia Joint Health Score (HJHS). Out of total 56 cases 51 (91.07 %) cases were diagnosed as hemophilia A while 5 cases (8.92 %) were diagnosed as hemophilia B. According to their factor level 44 % cases had severe 36 % had moderate and 20 % had mild disease. Knee joint was the predominant joint affected by hemarthrosis in 67.85 % cases followed by ankle joint (51.7 %) elbow joint (35.7 %), hip joint (12.5 %), shoulder joint (5.3 %) and proximal metacarpophalangeal joint (1.78 %).Out of total 37.5 % patients of hemophilia had developed target joint. Knee joint was the predominant target joint in 28.57 % cases and ankle joint was the target joint in 8.92 % cases. Maximum number of patients (40.47 %) had HJHS score of zero. The mean HJHS score was 6.78 ± 9.04. HJHS score showing significant positive correlation with age of patient (p < 0.0001). Most risky period and most aggravating development of hemophilic joint damage starts from 7 years of age. Therefore, treatment decisions, such as starting prophylaxis, should be tailored according to bleeding pattern and age of patients rather than based on the clotting factor activity levels.