Metabolic Diseases Clinic, The Children's Memorial Health Institute, Warsaw, Poland.
Acta Paediatr. 2012 Apr;101(4):e183-8. doi: 10.1111/j.1651-2227.2011.02522.x. Epub 2011 Dec 1.
The aims of the study were to assess shoulder range of motion (ROM) in patients with mucopolysaccharidosis type II (MPS II) and to correlate joint mobility with patients' height, age and functional status.
Passive ROM and Z-score of height were followed in 29 patients with MPS II (mean age 11.5 years, range 2-29 years) between the years 2005 and 2010. Passive ROM was measured by a goniometer, and height, by a stadiometer. Functional status was assessed by an age-appropriate health assessment questionnaire (HAQ).
(i) A strong correlation was observed between patients' age and Z-score of patients' height (R = 0.78, p < 0.001). (ii) A medium correlation was observed between Z-score of patients' height and passive shoulder flexion and abduction (R = 0.697, p < 0.001 and R = 0.63, p < 0.001, respectively). The progression of restriction was slower in attenuated patients. (iii) Restrictions in shoulder flexion and abduction were already observed before the second year of life. (iv) ROM limitations intensified and became more severe with age. (v) Activities of daily living depended on cognitive impairment of patients with MPS II.
Range of motion limitations in patients with MPS II correlate with patients' height, increase with patients' age and are more pronounced in a severe form of MPS II.
本研究旨在评估黏多糖贮积症 II 型（MPS II）患者的肩部活动度（ROM），并将关节活动度与患者的身高、年龄和功能状态相关联。
在 2005 年至 2010 年间，对 29 例 MPS II 患者（平均年龄 11.5 岁，范围 2-29 岁）进行了被动 ROM 和身高 Z 评分的随访。通过量角器测量被动 ROM，通过身高计测量身高。功能状态通过适合年龄的健康评估问卷（HAQ）进行评估。
（i）患者年龄与患者身高 Z 评分之间存在很强的相关性（R = 0.78，p < 0.001）。（ii）患者身高 Z 评分与被动肩关节前屈和外展之间存在中度相关性（R = 0.697，p < 0.001 和 R = 0.63，p < 0.001）。衰减患者的限制进展较慢。（iii）在生命的第二年之前，已经观察到肩关节前屈和外展受限。（iv）ROM 限制随着年龄的增长而加剧且变得更加严重。（v）日常生活活动取决于 MPS II 患者的认知障碍。
MPS II 患者的 ROM 限制与患者的身高相关，随患者年龄的增长而增加，在 MPS II 的严重形式中更为明显。
