Christoforidis Athanasios, Economou Marina, Papadopoulou Eleni, Kazantzidou Eirini, Gompakis Nikolaos, Athanassiou-Metaxa Miranda
1st Paediatric Department, Aristotle University, Thessaloniki, Greece.
J Pediatr Hematol Oncol. 2010 Oct;32(7):e259-63. doi: 10.1097/MPH.0b013e3181e8cd40.
Recent studies report reduced bone mineral density (BMD) even among young adults and children with hemophilia. Our aim was to assess bone status in children and adolescents with hemophilia with 2 methods: Quantitative UltraSonography (QUS) and Dual energy x-ray Absorptiometry (DXA), and consequently to investigate the degree of correlation between them. Twenty-seven patients (17 with severe hemophilia; residual factor activity <1% and 10 with moderate hemophilia) participated in the study. Mean age was 12.28±4.48 y (range: 4.94 to 18.00 y). All patients were evaluated with QUS at radius and at tibia and had DXA scan at lumbar spine. Anthropometric parameters were measured using standard techniques and joint evaluation was carried out using the Hemophilia Joint Health Score (HJHS). Only 2 out of 27 patients (7.5%) had BMD Z-scores <-2, whereas another 4 patients (15%) had BMD Z-scores between -1 and -2. QUS values in both radius and tibia were generally within the normal limits as only 1 patient had radius and another 1 had tibia QUS Z-score <-2. HJH scores were significantly although negatively correlated to Z-scores of tibia QUS (r=-0.455, P=0.034). No correlations were observed between lumbar BMD and radius or tibia QUS and no agreement was recorded between QUS and DXA in identifying patients at risk for osteoporosis (k=0.262). In conclusion, our study showed that only a small number of children and young adults with hemophilia have impaired bone properties as assessed both by DXA and QUS; no correlation was observed between these 2 methods.
近期研究报告称，即便在患有血友病的年轻人和儿童中，骨矿物质密度（BMD）也有所降低。我们的目的是用两种方法评估血友病患儿和青少年的骨骼状况：定量超声检查（QUS）和双能X线吸收测定法（DXA），进而研究二者之间的相关程度。27名患者（17名重度血友病患者；残余因子活性<1%，10名中度血友病患者）参与了该研究。平均年龄为12.28±4.48岁（范围：4.94至18.00岁）。所有患者均接受了桡骨和胫骨的QUS检查以及腰椎的DXA扫描。使用标准技术测量人体测量参数，并采用血友病关节健康评分（HJHS）进行关节评估。27名患者中只有2名（7.5%）的BMD Z值<-2，另有4名患者（15%）的BMD Z值在-1至-2之间。桡骨和胫骨的QUS值总体在正常范围内，只有1名患者的桡骨和另1名患者的胫骨QUS Z值<-2。HJH评分与胫骨QUS的Z值显著负相关（r=-0.455，P=0.034）。未观察到腰椎BMD与桡骨或胫骨QUS之间存在相关性，在识别骨质疏松风险患者方面，QUS和DXA之间也未达成一致（k=0.262）。总之，我们的研究表明，通过DXA和QUS评估，只有少数血友病儿童和青少年的骨骼特性受损；这两种方法之间未观察到相关性。
