RADIOLOGICAL AND ORTHOPEDIC SCORE IN PEDIATRIC HEMOPHILIC PATIENTS WITH EARLY AND LATE PROPHYLAXIS

In order to evaluate joint alteration, 17 patients with hemophilia A a nd B were investigated over a period of 4 years (1993-1997). Patients were subdivided into two groups, according to therapy regimens. In gro up 1 (n=10) prophylactic treatment was initiated until the third year of life. In group 2 (n=7) patients received prophylactic treatment at the age of 5 years and above. To assess alterations in knee, elbow, an d ankle joints, the radiological score and the physical examination sc ore of the Orthopedic Advisory Committee of the World Federation of He mophilia were used. The sum of the scores of these six joints was defi ned as the patient-dependent score. Patients of group 1 (median age at the end of observation: 10 years) reached a median radiological score of 1.0 (range: 0-13) and an orthopedic score of 0 (range: 0-4), where as patients of group 2 (median age: 14 years) had a radiological score of 20 (range: 2-47) and an orthopedic score of 8 (range: 0-12), which shows a significant difference (p<0.01). In both treatment groups a m anifestation or progression of arthropathic alteration was seen in tho se children who had repeated joint bleeding (>5) prior to the onset of prophylactic treatment (r=0.90, p>0.01). Altogether, two of 60 joints in group 1 and 12 of 42 joints in group 2 had a radiological score gr eater than or equal to 4. Elbow joints were more often affected than k nee and ankle joints. In conclusion, the number of joint bleedings bef ore prophylactic treatment was started influenced the progression of a rthropathy even in patients with early onset of prophylaxis. The aim o f treatment in severe hemophilia should be early prophylaxis before re peated joint bleeding occurs in order to prevent osteoarthropathic alt eration.