脊髓性肌萎缩症中的低骨矿物质密度

Low bone mineral density in spinal muscular atrophy.

作者信息

Khatri Ismail A, Chaudhry Umar S, Seikaly Mouin G, Browne Richard H, Iannaccone Susan T

机构信息

Shifa College of Medicine and Shifa International Hospitals, Islamabad, Pakistan.

出版信息

J Clin Neuromuscul Dis. 2008 Sep;10(1):11-7. doi: 10.1097/CND.0b013e318183e0fa.

DOI:10.1097/CND.0b013e318183e0fa

PMID:18772695

Abstract

BACKGROUND AND PURPOSE

Pathological fractures are common in pediatric neuromuscular disorders. Dual-energy x-ray absorptiometry has become the most accepted technique for the measurement of bone mineral density (BMD) in adults and children. Limited data are available on BMD in pediatric neuromuscular diseases except Duchenne muscular dystrophy.

METHODS

We retrospectively analyzed the results of all dual-energy x-ray absorptiometry scans done in a period of 23 months at a tertiary care pediatric neuromuscular center. BMD was performed on spine region L1-4. Osteopenia was classified as mild if the Z scores were between 0 and -1.5, moderate if Z scores were between -1.5 and -2.5, and severe if Z scores were > -2.5 standard deviation scores.

RESULTS

Eighty-four dual-energy x-ray absorptiometry scans were performed on 79 patients between the ages of 4 months and 18 years with the mean age of 8 years. Z scores were used to compare their BMDs. BMD was lowest in patients with spinal muscular atrophy (SMA) with Z score of -2.25 +/- 0.31 standard deviation scores. The Z score for patients with Duchenne muscular dystrophy was -1.72 +/- 0.1. The BMD in nonambulatory patients with SMA was significantly decreased compared with ambulatory patients with SMA (P < 0.05).

CONCLUSIONS

We conclude that osteopenia is common in children with neuromuscular disorders. Patients with SMA have the lowest BMD.

摘要

背景与目的

病理性骨折在儿童神经肌肉疾病中很常见。双能X线吸收法已成为测量成人和儿童骨密度（BMD）最常用的技术。除了杜氏肌营养不良症外，关于儿童神经肌肉疾病骨密度的可用数据有限。

方法

我们回顾性分析了一家三级儿科神经肌肉中心在23个月内进行的所有双能X线吸收法扫描结果。在脊柱L1 - 4区域进行骨密度检测。如果Z值在0至 - 1.5之间，骨质疏松被分类为轻度；如果Z值在 - 1.5至 - 2.5之间，为中度；如果Z值> - 2.5标准差分数，则为重度。

结果

对79例年龄在4个月至18岁（平均年龄8岁）的患者进行了84次双能X线吸收法扫描。使用Z值比较他们的骨密度。脊髓性肌萎缩症（SMA）患者的骨密度最低，Z值为 - 2.25±0.31标准差分数。杜氏肌营养不良症患者的Z值为 - 1.72±0.1。与能行走的SMA患者相比，不能行走的SMA患者骨密度显著降低（P < 0.05）。