Engelbert R H, Gulmans V A, Uiterwaal C S, Helders P J
Department of Pediatric Physical Therapy, University Medical Center, Wilhelmina Children's Hospital, Utrecht, The Netherlands.
Arch Phys Med Rehabil. 2001 Jul;82(7):943-8. doi: 10.1053/apmr.2001.23889.
To examine the perceived competence of children with different types of osteogenesis imperfecta (OI) and to investigate the possible relationships between their perceived competence and impairment parameters.
Cross-sectional study.
National referral center (hospital) for the treatment of children with OI.
Forty children with OI (type I = 17; type III = 11; type IV = 12) with a mean age +/- standard deviation of 12.6 +/- 3.2 years.
Measured joint range of motion (ROM) in the upper extremities (UEs), and lower extremities (LEs), muscle strength, functional skills, ambulation, and perceived competence.
Joint ROM in UE and LE; muscle strength (using the manual muscle testing criteria of the Medical Research Council); functional skills using the Pediatric Evaluation of Disability Inventory in 3 domains (self-care, mobility, social function). Ambulation (according to Bleck and classified as nonwalking, therapy walking, household walking, neighborhood walking, community walking with or without the use of crutches), and perceived competence (using the Harter Self-Perception Profile for Children, which was cross-culturally validated for Dutch children).
In children with type I, joint ROM and muscle strength were almost comparable to the healthy population. In children with type III, a severe decrease in joint ROM was measured, especially in the LEs, and muscle strength was severely decreased in the UEs and LEs. In children with type IV, joint ROM and muscle strength decreased, especially in the LEs. In all types, fairly to strongly positive perceived competence was measured except for fairly negative perceived competence in the athletic performance subscale in type I and a fairly negative perceived competence in the romance subscale in type III. No correlations were found between (1) joint ROM and athletic performance and physical appearance, (2) muscle strength and athletic performance or physical appearance, or (3) the functional skills, concerning self-care and mobility, with the subscales of the perceived competence.
Although joint ROM, muscle strength, and functional and walking ability were related to the severity of the disease and differed significantly between the different types of OI, overall perceived competence in children with OI was fairly to strongly positive, without significant differences between the different types of OI.
研究不同类型成骨不全症(OI)患儿的自我感知能力,并探讨其自我感知能力与损伤参数之间的可能关系。
横断面研究。
全国OI患儿治疗转诊中心(医院)。
40例OI患儿(I型 = 17例;III型 = 11例;IV型 = 12例),平均年龄±标准差为12.6±3.2岁。
测量上肢(UEs)和下肢(LEs)的关节活动范围(ROM)、肌肉力量、功能技能、步行能力和自我感知能力。
UE和LE的关节ROM;肌肉力量(采用医学研究委员会的徒手肌力测试标准);使用残疾儿童评定量表在3个领域(自我护理、移动性、社会功能)评估功能技能。步行能力(根据Bleck分类,分为非步行、治疗性步行、家庭步行、社区附近步行、使用或不使用拐杖的社区步行),以及自我感知能力(使用儿童哈特自我感知量表,该量表已针对荷兰儿童进行了跨文化验证)。
I型患儿的关节ROM和肌肉力量与健康人群几乎相当。III型患儿的关节ROM严重下降,尤其是在LEs,UEs和LEs的肌肉力量严重下降。IV型患儿的关节ROM和肌肉力量下降,尤其是在LEs。在所有类型中,除了I型患儿在运动表现子量表中自我感知能力为中等负面,III型患儿在浪漫子量表中自我感知能力为中等负面外,自我感知能力均为中等至强烈正面。未发现以下各项之间存在相关性:(1)关节ROM与运动表现和外貌;(2)肌肉力量与运动表现或外貌;(3)与自我护理和移动性相关的功能技能与自我感知能力的子量表。
尽管关节ROM、肌肉力量、功能和步行能力与疾病严重程度相关,且在不同类型的OI之间存在显著差异,但OI患儿的总体自我感知能力为中等至强烈正面,不同类型的OI之间无显著差异。