Ladis Vassilis, Raptou Panagiota, Rigatou Efthimia, Chouliaras George, Galanos Antonis, Korres Dimitrios, Kattamis Christos
Thalassemia Unit, First Department of Pediatrics, University of Athens, Aghia Sophia Children's Hospital, Athens, Greece.
Pediatr Endocrinol Rev. 2008 Oct;6 Suppl 1:127-31.
Bone changes are a prominent symptom of beta-thalassemias, related to expansion of bone marrow and reduction of bone density. Conventional treatment ameliorates bone changes and improves survival, thus increasing the morbidity of bone diseases in adulthood. Peripheral quantitative computer tomography (pQCT) was used recently to assess the changes in volumetric bone mineral density (vBMD) in various bone compartments.
Assessment of indices of bone density and structure in patients with thalassemia major (thal-major) and intermedia (thal-interm) on conventional therapy and in healthy adults.
45 patients with thal-major, 27 with thal-interm and 32 healthy individuals aged 21-42 years were studied by pQCT analysis. The vBMD total (tot), trabecular (trab) and cortical (cort), the bone mineral content (BMC), the cross sectional area (CSA), the cortical thickness (CRTHK) and the stress strain index (SSI) were assessed at the 4% site of the distal radius.
Tot, trab, and cort vBMD, BMC, and cortical thickness showed statistically significant differences among the three groups with significant reduction in thalassemics. No significant differences were found in the three groups with CSA and SSI. Impairment of bone density and structure in Greek thalassemics on proper treatment was not as severe as expected. A significant proportion of patients had bone density indices within the normal range and above the 10th percentile of normal.
Peripheral QCT analysis is a convenient method to study the regional changes of bone density in patients with thalassemia. These changes affect mainly the cortical compartment and are more pronounced in thalassemia intermedia.
骨骼改变是β地中海贫血的一个突出症状,与骨髓扩张和骨密度降低有关。传统治疗可改善骨骼改变并提高生存率,从而增加成年期骨骼疾病的发病率。外周定量计算机断层扫描(pQCT)最近被用于评估不同骨区域的体积骨密度(vBMD)变化。
评估接受传统治疗的重型地中海贫血(thal-major)和中间型地中海贫血(thal-interm)患者以及健康成年人的骨密度和结构指标。
通过pQCT分析对45例重型地中海贫血患者、27例中间型地中海贫血患者和32名年龄在21 - 42岁的健康个体进行了研究。在桡骨远端4%部位评估vBMD总量(tot)、小梁骨(trab)和皮质骨(cort)、骨矿物质含量(BMC)、横截面积(CSA)、皮质厚度(CRTHK)和应力应变指数(SSI)。
三组之间的tot、trab和cort vBMD、BMC以及皮质厚度存在统计学显著差异。地中海贫血患者这些指标显著降低。三组在CSA和SSI方面未发现显著差异。接受适当治疗的希腊地中海贫血患者的骨密度和结构损害没有预期的那么严重。相当一部分患者的骨密度指标在正常范围内且高于正常第10百分位数。
外周QCT分析是研究地中海贫血患者骨密度区域变化的一种便捷方法。这些变化主要影响皮质骨区域,在中间型地中海贫血中更为明显。