Decreased bone mineral density in neurofibromatosis-1 patients with spinal deformities
Decreased bone mineral density in neurofibromatosis-1 patients with spinal deformities
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DOI：
10.1007/s001980170032
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发表时间：
2001-01-01
影响因子：
4
通讯作者：
Dubousset, J
中科院分区：
文献类型：
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作者：
Illés, T;Halmai, V;Dubousset, J
进行了一项横断面研究，以获得一组神经纤维瘤病-1脊柱畸形患者的骨密度状态的数据，并寻找可能伴随的骨矿物质转换的变化。神经纤维瘤病-1是一种常见的疾病，10-50%的患者伴有各种骨骼异常（主要是脊柱畸形）。术中观察到椎体骨质较差。已作出努力，以确定因素，防止曲线进展，优化操作计划，并解释病理机制。作为术前评估的一部分，双能X线骨密度测定法用于评估12例神经纤维瘤病-1患者的腰椎骨密度，并辅以实验室血液/尿液检查。测量到腰椎骨矿物质密度显著降低。脊柱侧凸的严重程度与腰椎Z评分呈负相关。在实验室测量中未发现关键性变化。在神经纤维瘤病-1患者术中观察到的骨组织异常可描述为轴向骨矿物质密度降低。生化参数不支持存在甲状旁腺功能亢进、肾脏疾病或其他影响骨矿物质转换的相关疾病。在神经纤维瘤病-1的术前计划过程中骨密度的评价，提出了确切的背景和可能的骨质疏松症在预后中的作用仍有待阐明。
A cross-sectional study was carried out to obtain data on the bone mineral density status of a group of neurofibromatosis-1 patients with spinal deformities, and to search for possible accompanying changes in the bone mineral turnover. Neurofibromatosis-1 is a here-dofamiliar disorder that is associated with a variety of skeletal anomalies (mostly spinal deformities) in 10-50% of patients. Intraoperatively, a poor vertebral bone quality has been observed. Efforts have been made to identify factors preventing curve progression, to optimize operational planning and to explain the pathomechanism. As part of the preoperative evaluation, dual-energy X-ray absorptiometry was used to assess the bone mineral density of the lumbar spine in 12 patients with neurofibromatosis- 1, supplemented by laboratory blood/urine investigations. A significant decrease in bone mineral density of the lumbar spine was measured. An inverse relation was suggested between the severity of scoliosis and the lumbar spine Z-scores. No pivotal alterations were identified in the laboratory measurements. The bony tissue abnormality observed intraoperatively in neurofibromatosis-1 patients may be described as a diminution of the axial bone mineral density. The biochemical parameters do not support the presence of hyperparathyroidism, renal disorders or other associated diseases influencing the bone mineral turnover. The evaluation of bone mineral density in the course of the preoperative planning is proposed in neurofibromatosis-1; the exact background and the role of a possible osteoporosis in the prognosis remain to be elucidated.