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普拉德-威利综合征与脊柱侧弯。

Prader-Willi syndrome and scoliosis.

作者信息

Holm V A, Laurnen E L

出版信息

Dev Med Child Neurol. 1981 Apr;23(2):192-201. doi: 10.1111/j.1469-8749.1981.tb02441.x.

DOI:10.1111/j.1469-8749.1981.tb02441.x
PMID:7215706
Abstract

Of 37 patients with the Prader-Willi syndrome for whom spinal x-rays were available, 32 had a structural scoliosis of 10 degrees or greater. Kyphosis was also found to be more common in older persons with this syndrome, occurring in only one of 14 adolescents but in five of 10 adults. The scoliosis was analyzed in infantile, juvenile, adolescent and adult subgroups. The results suggest that the scoliosis is present from an early age and remains stable during childhood, but progresses in 15 to 20 per cent of cases during adolescence. Scoliosis in patients with Prader-Willi syndrome has many of the clinical characteristics encountered in idiopathic scoliosis. Because it is difficult to detect in these individuals, the authors recommended that baseline spinal X-rays be obtained in all such patients who have suspicious asymmetry of the spine, followed by regular clinical examination, especially during adolescence.

摘要

在37例有脊柱X光片的普拉德-威利综合征患者中,32例有10度或以上的结构性脊柱侧弯。还发现脊柱后凸在患有该综合征的老年人中更常见,14名青少年中只有1例出现,而10名成年人中有5例出现。对婴儿、青少年、青年和成人亚组的脊柱侧弯情况进行了分析。结果表明,脊柱侧弯在早年就已存在,在儿童期保持稳定,但在15%至20%的病例中在青春期会进展。普拉德-威利综合征患者的脊柱侧弯具有特发性脊柱侧弯的许多临床特征。由于在这些个体中很难检测到,作者建议对所有有可疑脊柱不对称的此类患者进行脊柱X光基线检查,随后进行定期临床检查,尤其是在青春期。

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