Department of Morphology, Surgery and Experimental Medicine, Section of Radiology, University of Ferrara, Ferrara, Italy.
Department of Medical Sciences, Section of Endocrinology and Internal Medicine, University of Ferrara, Ferrara, Italy.
Endocrine. 2019 Sep;65(3):469-482. doi: 10.1007/s12020-019-02003-x. Epub 2019 Jul 12.
Low mineral mass and reduced bone strength with increased fracture risk are the main causes of morbidity in Thalassemia Major (TM). The pathogenesis is multifactorial and includes ineffective erythropoiesis with medullary expansion, multiple endocrine dysfunctions, direct iron bone deposition, deferoxamine-induced bone dysplasia, and reduced physical activity associated with disease complications. Dual-energy X-ray absorptiometry (DXA) is the "gold standard" for bone mineral density (BMD) assessment and for bone strength and quality evaluation. This method identifies patients at greater risk of fragility fractures, guiding treatment and monitoring response to therapy. In TM, DXA shows limitations concerning BMD calculation accuracy and fracture risk prediction. One of the main challenges in the assessment of bone health in patients with TM is the accurate interpretation of densitometric results.
This review investigates the major pitfalls in DXA implementation and interpretation in TM.
Available literature has been assessed.
DXA shows limitations in assessing bone mineral "status" in TM, especially in the paediatric population, due to the peculiar characteristics of bone architecture and deformities associated with the disease. A radiological technique adjustment in this population is mandatory.
低矿物质含量和骨强度降低伴骨折风险增加是重型地中海贫血（TM）患者发病和致残的主要原因。其发病机制是多因素的，包括无效的红细胞生成伴骨髓扩张、多种内分泌功能障碍、直接铁骨沉积、去铁胺诱导的骨发育不良，以及与疾病并发症相关的体力活动减少。双能 X 线吸收法（DXA）是评估骨矿物质密度（BMD）、骨强度和质量的“金标准”。这种方法可以识别出更容易发生脆性骨折的患者，指导治疗并监测治疗反应。在 TM 中，DXA 在 BMD 计算准确性和骨折风险预测方面存在局限性。评估 TM 患者骨骼健康的主要挑战之一是准确解读骨密度测量结果。
本文研究了 DXA 在 TM 中的实施和解读中的主要缺陷。
评估了现有文献。
DXA 在评估 TM 中的骨矿物质“状态”方面存在局限性，尤其是在儿科人群中，因为该病存在骨结构的特殊性和与疾病相关的畸形。因此，有必要在该人群中调整放射技术。
