Assessment of bone mineral density by dual energy x-ray absorptiometry in patients with mucopolysaccharidoses
Assessment of bone mineral density by dual energy x-ray absorptiometry in patients with mucopolysaccharidoses
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DOI：
10.1186/1750-1172-8-71
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发表时间：
2013-05-11
影响因子：
3.7
通讯作者：
Lin, Shuan-Pei
中科院分区：
文献类型：
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作者：
Lin, Hsiang-Yu;Shih, Shou-Chuan;Lin, Shuan-Pei
背景资料：粘多糖沉积症（MPS）患者骨生长和矿化能力差，但关于MPS患者骨密度（BMD）与年龄和治疗的关系的资料有限（21例男性和9例女性; 2例MPS I，12例MPS II，2例MPS IIIB，9例MPS IVA和5例MPS VI;中位年龄为10.8岁;年龄范围为5.0岁至23.7岁; 26例患者年龄小于19岁，4例年龄大于19岁），使用Hologic QDR 4500系统评估腰椎（L1-L4）的BMD（贝德福德，马萨诸塞州，美国）。对于26名19岁以下的患者，标准差评分身高、体重、体重指数（BMI）和BMD的z评分分别为-4.53 +/- 2.66、-1.15 +/- 1.55、0.74 +/- 1.23和-3.03 +/- 1.62，与年龄呈负相关（P < 0.05）。然而，校正身高年龄z评分（HAZ）后，HAZ校正的BMD z评分为-0.7 +/-1.24。8名患者（31%）有骨质减少（HAZ校正的BMD z评分< -1 and >= -2），4名患者（15%）有骨质疏松症（HAZ校正的BMD z评分< -2）。8例MPS I，II或VI谁接受后续DXA后，接受酶替代疗法为1.0至7.4年，所有显示绝对BMD value.Conclusions增加：这些研究结果和后续数据可用于制定高质量的护理策略与MPS患者。
Background: Patients with mucopolysaccharidoses (MPS) are associated with poor bone growth and mineralization, however, information regarding the assessment of bone mineral density (BMD) in relation to age and treatment in this disorder is limited.Methods: Dual energy x-ray absorptiometry (DXA) was performed in 30 patients with MPS (21 males and 9 females; 2 with MPS I, 12 with MPS II, 2 with MPS IIIB, 9 with MPS IVA, and 5 with MPS VI; median age, 10.8 years; age range, 5.0 years to 23.7 years; 26 patients were under 19 and 4 were above 19 years of age) to assess BMD of the lumbar spine (L1-L4), using the Hologic QDR 4500 system (Bedford, MA, USA).Results: For 26 patients under 19 years of age, standard deviation scores (z scores) for height, weight, body mass index (BMI), and BMD were -4.53 +/- 2.66, -1.15 +/- 1.55, 0.74 +/- 1.23, and -3.03 +/- 1.62, respectively, and they were all negatively correlated with age (p < 0.05). However, after correction for height-for-age z score (HAZ), HAZ adjusted BMD z score was -0.7 +/- 1.24. Eight patients (31%) had osteopenia (HAZ adjusted BMD z score < -1 and >= -2), and 4 patients (15%) had osteoporosis (HAZ adjusted BMD z score < -2). Of 8 patients with MPS I, II or VI who underwent follow-up DXA after receiving enzyme replacement therapy for 1.0 to 7.4 years, all showed increase in absolute BMD values.Conclusions: These findings and the follow-up data can be used to develop quality of care strategies for patients with MPS.