In order to evaluate joint alteration, 17 patients with hemophilia A and B were investigated over a period of 4 years (1993-1997). Patients were subdivided into two groups, according to therapy regimens. In group 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, and ankle joints, the radiological score and the physical examination score of the Orthopedic Advisory Committee of the World Federation of Hemophilia were used. The sum of the scores of these six joints was defined 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), whereas 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 manifestation or progression of arthropathic alteration was seen in those 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 > or = 4. Elbow joints were more often affected than knee and ankle joints. In conclusion, the number of joint bleedings before prophylactic treatment was started influenced the progression of arthropathy even in patients with early onset of prophylaxis. The aim of treatment in severe hemophilia should be early prophylaxis before repeated joint bleeding occurs in order to prevent osteoarthropathic alteration.
Dear Sir, Osteoathropathic alteration is a major cause of morbidity in patients with severe haemophilia. During infancy and adolescence, prophylaxis is undoubtedly preferable to on-demand therapy, but as yet poor data exist comparing the onset of prophylactic treatment at an early age with treatment initiated at a more advanced age (1,2,3). In order to determine which therapy regime is needed to prevent haemophilic arthropathy we prospectively compared 17 patients with moderate and severe haemophilia A (age: 3-14 years; mean age: 6 2/12 years). All 17 patients were treated with factor VIII (30-40 IU/kg bw/three times per week; Humate P®, Beriate HS®, Behring, Germany). Prior to radiological and clinical examinations of the knee, elbow and ankle joints patients were subdivided into two groups according to therapy regimes. In group 1 prophylactic treatment was initiated in
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