普拉德-威利综合征（PWS）中脊柱侧弯的特征：对58例PWS脊柱侧弯患者的分析。

The characteristics of scoliosis in Prader-Willi syndrome (PWS): analysis of 58 scoliosis patients with PWS.

作者信息

Nakamura Yutaka, Murakami Nobuyuki, Iida Takahiro, Ozeki Satoru, Asano Satoshi, Nohara Yutaka, Nagai Toshiro

机构信息

Department of Orthopaedic Surgery, Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minami-Koshigaya, Koshigaya, Saitama, 343-8555, Japan,

出版信息

J Orthop Sci. 2015 Jan;20(1):17-22. doi: 10.1007/s00776-014-0651-y. Epub 2014 Sep 25.

DOI:10.1007/s00776-014-0651-y

PMID:25253244

Abstract

BACKGROUND

The purpose of this study was to compare the characteristics of scoliosis in Prader-Willi syndrome (PWS) patients versus idiopathic scoliosis (IS).

METHODS

We identified 193 PWS patients. Scoliosis was found in 58 PWS patients, 39 of whom were treated with GH. Fifty-five IS patients were consecutively selected from an outpatient clinic. We investigated 113 patients (58 PWS group, 55 IS group) followed for a minimum of 2 years. The mean age was 17.9 and 16.1 years, respectively. Deformity was measured using Lenke classification, Cobb angle, thoracic kyphosis at T2-5 and T5-12, lumbar lordosis at T12-S1, and sagittal alignment at the C7 plumb line. BMI was also recorded.

RESULTS

According to the Lenke system, patients were classified as (PWS group/IS group): Type 1 (8/26), Type 2 (1/4), Type 3 (3/15), Type 4 (1/0), Type 5 (32/8), and Type 6 (13/2). The average Cobb angles were 32.6° in the PWS and 35.4° in the IS. No significant differences were found for the thoracic kyphosis (T2-5, T5-12), lumbar lordosis (T12-S1) or C7 plumb line between the two groups. BMI was increased in the PWS group not treated previously with GH as compared with the IS group and the PWS group with GH.

CONCLUSIONS

Most PWS patients presented with lumbar or thoracolumbar curves (Type 5, 6), whereas IS patients typically had thoracic scoliosis (Type 1, 2, 3).

摘要

背景

本研究旨在比较普拉德-威利综合征（PWS）患者与特发性脊柱侧凸（IS）患者脊柱侧凸的特征。

方法

我们确定了193例PWS患者。58例PWS患者发现有脊柱侧凸，其中39例接受生长激素（GH）治疗。从门诊连续选取55例IS患者。我们对113例患者（58例PWS组，55例IS组）进行了至少2年的随访。平均年龄分别为17.9岁和16.1岁。使用Lenke分类、Cobb角、T2 - 5和T5 - 12节段的胸椎后凸、T12 - S1节段的腰椎前凸以及C7铅垂线测量畸形情况。还记录了体重指数（BMI）。

结果

根据Lenke系统，患者分类如下（PWS组/IS组）：1型（8/26），2型（1/4），3型（3/15），4型（1/0），5型（32/8）和6型（13/2）。PWS组平均Cobb角为32.6°，IS组为35.4°。两组之间的胸椎后凸（T2 - 5，T5 - 12）、腰椎前凸（T12 - S1）或C7铅垂线未发现显著差异。与IS组和接受GH治疗的PWS组相比，未接受过GH治疗的PWS组BMI升高。