Pergantou H, Matsinos G, Papadopoulos A, Platokouki H, Aronis S
Haemophilia Centre, Haemostasis Unit, Aghia Sophia Children's Hospital, Athens, Greece.
Haemophilia. 2006 May;12(3):241-7. doi: 10.1111/j.1365-2516.2006.01208.x.
To evaluate joints alterations, we performed clinical examination, X-rays and magnetic resonance imaging (MRI) (Denver score) in 165 joints of 40 children with severe (n, 32) or moderate (n, 8) haemophilia A or B. All investigated joints had a history of more than three bleeds. At evaluation, 25 of 40 haemophilic patients were on prophylaxis for the last 1-8 years (mean: 3.5 years). MRI revealed chronic synovitis in 55.4% and 50% of joints, which were diagnosed, as normal by the clinical scale and plain radiography respectively. Moreover, MRI unmasked more profound alterations than those observed by plain radiography in 70% of cases. Statistical analysis showed that the clinical and Pettersson scores in contrast to the Denver score provide an underestimation of arthritic changes. Besides, Denver score did not provide resolution in differentiating stages of arthropathy, because of its inherent nature; however, a score of 6 expressing severe synovitis seemed to be the cut-off value for the distinction of severe cases. Based on MRI findings we intensified prophylaxis in nine children and initiated it in another nine children. Five children, who were already on prophylaxis complied with our recommendations and eliminated haemorrhages. Finally, three boys with severe haemophilic arthropathy in knees underwent successful chemical synovectomy with rifampicin.
为评估关节改变情况,我们对40例重度(n = 32)或中度(n = 8)甲型或乙型血友病患儿的165个关节进行了临床检查、X线检查及磁共振成像(MRI)(丹佛评分)。所有接受检查的关节都有超过三次出血的病史。在评估时,40例血友病患者中有25例在过去1至8年(平均:3.5年)接受了预防治疗。MRI显示,分别有55.4%和50%的关节存在慢性滑膜炎,而这些关节在临床检查和X线平片检查中分别被诊断为正常。此外,在70%的病例中,MRI显示的关节改变比X线平片观察到的更为严重。统计分析表明,与丹佛评分相比,临床评分和彼得松评分对关节炎改变的评估存在低估。此外,由于丹佛评分的固有性质,它在区分关节病阶段方面无法提供明确结果;然而,6分表示严重滑膜炎似乎是区分严重病例的临界值。基于MRI检查结果,我们加强了9名患儿的预防治疗,并对另外9名患儿开始进行预防治疗。5名已经接受预防治疗的患儿遵循了我们的建议,避免了出血。最后,3名患有重度膝关节血友病性关节病的男孩成功接受了利福平化学滑膜切除术。