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黏多糖贮积症IV型(Morquio综合征)患儿步态模式及下肢运动学评估。

Evaluation of Gait Pattern and Lower Extremity Kinematics of Children with Morquio Syndrome (MPS IV).

作者信息

Salazar-Torres Jose J, Church Chris, Shields Thomas, Shrader M Wade, Fisher Lydia, Mackenzie William G, Mackenzie W G Stuart

机构信息

Nemours A.I. duPont Hospital for Children, 1600 Rockland Rd., Wilmington, DE 19803, USA.

出版信息

Diagnostics (Basel). 2021 Jul 27;11(8):1350. doi: 10.3390/diagnostics11081350.

Abstract

Morquio syndrome (mucopolysaccharidosis IV/MPS IV) is a genetic disorder leading to skeletal abnormalities and gait deviations. Research on the gait patterns and lower extremity physical characteristics associated with skeletal dysplasia in children with MPS IV is currently limited. This research aimed to provide baseline gait patterns and lower limb skeletal alignment of children with MPS IV utilizing three-dimensional instrumented gait analysis. This Institutional Review Board-approved retrospective study evaluates the kinematics of the lower extremities of children with MPS IV during gait, comparing them with an age-matched group of typically developing children. Thirty-three children with MPS IV were included (8.6 ± 4.0 years old). Children with MPS IV walk with increased anterior pelvic tilt, knee valgus, knee flexion, external tibial torsion, and reduced walking speed and stride length ( < 0.001). Multiplanar abnormal alignment results in abnormal knee moments ( < 0.001). Limited correlations exist ( = 0.69-0.28) between dynamic three-dimensional measurements of knee varus/valgus and rotational alignment and traditional static two-dimensional measures (physical examination or radiographs) suggesting the possibility of knee instability during gait and the benefits of dynamic assessment.

摘要

黏多糖贮积症IV型(莫尔基奥综合征/MPS IV)是一种导致骨骼异常和步态偏差的遗传性疾病。目前,关于MPS IV型患儿骨骼发育不良相关步态模式和下肢身体特征的研究有限。本研究旨在利用三维仪器化步态分析,提供MPS IV型患儿的基线步态模式和下肢骨骼对线情况。这项经机构审查委员会批准的回顾性研究评估了MPS IV型患儿在步态过程中下肢的运动学,将他们与年龄匹配的典型发育儿童组进行比较。纳入了33名MPS IV型患儿(8.6±4.0岁)。MPS IV型患儿行走时骨盆前倾增加、膝外翻、膝关节屈曲、胫骨外旋,且步行速度和步长降低(<0.001)。多平面异常对线导致膝关节力矩异常(<0.001)。膝关节内翻/外翻和旋转对线的动态三维测量与传统静态二维测量(体格检查或X线片)之间存在有限的相关性(=0.69 - 0.28),这表明步态期间膝关节不稳定的可能性以及动态评估的益处。

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