Radiological and orthopedic score in pediatric hemophilic patients with early and late prophylaxis

Abstract

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.

Hemophilic arthropathy. A scoring system for magnetic resonance imaging

“…Although a larger series should be evaluated and a longer follow-up period is needed, these findings suggest that a return to normality or improvement is possible when slight synovial hypertrophy, such as in MR grade I, occurs. A possible explanation for the discrepancy between our findings and the previous studies about possible improvement in early phases of HA may be due to the fact that previous reports used radiological scores [18,19,20], which are less sensitive to earlier phases of arthropathy. In fact, in our series we had been unable to establish the presence of HA and the follow-up of abnormalities detected on MR in 6 joints if only conventional radiography had been evaluated.…”

Prevention of bleeds in hemophilia patients with inhibitors: Emerging data and clinical direction

“…Usually, small amounts of FVIII are given 3 times weekly, or often enough to maintain the trough FVIII level above 1-2%. Long-term follow-up studies of patients maintained on prophylactic FVIII since early childhood have demonstrated a significantly reduced prevalence of clinical and radiographic joint abnormalities compared with patients treated with FVIII on demand [37][38][39]. Many clinicians throughout the world now consider prophylaxis an important therapeutic option for the prevention of end-stage joint disease and recommend initiating it in all children with severe hemophilia.…”

Risks and challenges of orthopaedic invasive interventions in haemo -philia in a low-resource country

“…In low income countries, the shortage of specific replacement therapy (lack of prophylaxis, delayed-and under dosed on demand therapy) increases and enhances the need of invasive orthopaedic procedures. Intractable and chronic pain and severely restricted joint function underlie the indication of such measures (8). In contrast to developed countries, common indications are: corrective osteotomy, arthrodesis, synovectomy, removal of pseudo-tumours, articular debridement, alignment osteotomy and osteosyntesis (12,13).…”

“…Several parameters were monitored: bleeding rate, pain score, range of motion (ROM), Petrini score for children, Orthopaedic Joint Score (OJS) modified (Gilbert) for adults (7,8). Factor concentrates consumption in relation to the type of treatment and invasiveness of the intervention was also calculated.…”