【摘要】 目的 评估早期康复治疗在多发关节挛缩畸形中治疗的效果。方法 回顾纳入 2019 年 5 月至 2024 年 2 月，在我院门诊就诊的关节挛缩患儿，对 0～3 个月婴儿采用手法康复操治疗，对挛缩的上肢关节进行被动活动。关节被动牵拉停留 5～10 s，若不能配合，尽量使之维持 2～5 s，然后放松。5～7 个交替为一组，每次可进行 3 组，每日可按早、中、晚时间行 3～4 次训练。3 个月以上婴儿在进行手法康复操治疗的同时，辅以白天睡眠及夜间佩戴支具。1 岁半以上患儿白天增加挛缩关节的主动牵拉训练，包括肩、肘、腕及手指关节。每 3～6 个月门诊定期复查一次。对患者资料进行统计学描述分析，分类变量以总数 (百分比) 表示。对数值变量进行 Shapiro-Wilk 正态分析，非正态分布的连续变量以中位数 (25 分位数，75 分位数) 表示，采用 Mann-Whitney U 检验，治疗前、后挛缩程度改善情况，采用配对设计的 Mann-Whitney U 检验，对不同年龄段患儿治疗后关节活动度改善情况进行 2 个独立样本的 Mann-Whitney U 检验，以 P < 0.05 为差异有统计学意义。结果 本组共纳入 36 例，就诊年龄 2～168 个月，中位年龄 36.0 (8.3，73.5) 个月，随访 6～64 个月，中位时间 11.0 (6.3，12.0) 个月。男 19 例，女 17 例。双侧上肢多发关节挛缩畸形 24 例，右侧 8 例，左侧4 例。共累及 290 个关节，涉及肩、肘、腕大关节共计 27 个，掌指及指间关节 263 个，随访期间肩、肘关节活动度平均改善 30.0°，腕关节 17.5°，拇指掌指关节改善 32.5°，其余四指的掌指关节改善 50.0°，近指间关节改善 40.0°。均可见上肢关节活动度明显改善，6 岁前开始进行康复治疗，关节活动度改善情况优于 6 岁后(P < 0.05)。结论 多发关节挛缩应尽早开始系统的康复治疗，需长期坚持、多学科参与，有助于改善肢体功能，提高患儿生活质量。更多还原

【Abstract】 Objective To evaluate the effectiveness of early rehabilitation therapy in treating multiple arthrogryposis multiplex deformities.Methods Patients with arthrogryposis multiplex in the upper limbs who visited the outpatient clinic from May 2019 to February 2024 were retrospectively included.For infants aged 0-3 months,passive motion exercises were administered.Passive joint traction was maintained for 5-10 seconds,and it was maintained for 2-5 seconds before releasing if cooperation was not possible.Five to seven exercises were performed as one set,with three sets performed each time,and training was conducted 3-4 times daily.For infants over 3 months old,manual rehabilitation exercises were supplemented with nighttime brace wear.For children over 1.5 years old,active motion training of contracted joints,including shoulder,elbow,wrist,and finger joints,was added during the day.Regular outpatient reviews were conducted every 3-6 months.Patient data were statistically described and analyzed,with categorical variables expressed as total number (percentage).Numerical variables were subjected to Shapiro-Wilk normality analysis,and continuous variables with non-normal distribution were expressed as median (25th percentile,75th percentile).Mann-Whitney U test was used in joint range of motion (ROM) before and after treatment.Two independent sample Mann-Whitney U tests were conducted to compare the improvement in joint ROM after treatment among patients of different age groups,with P < 0.05 indicating statistically significant differences.Results Thirty-sixpatients were enrolled,with ages ranging from 2 to 168 months at the time of diagnosis,and a median age of 36.0 months(range:8.3 to 73.5 months).The follow-up period ranged from 6 to 64 months,with a median of 11.0 months (range:6.3 to 12.0 months).There were 19 males and 17 females.Deformities occurred in both upper limbs in 24 cases,with 8 cases on the right side and 4 cases on the left side.A total of 290 joints were affected,including 27 large joints of the shoulder,elbow,and wrist,as well as 263 metacarpophalangeal and interphalangeal joints.During the follow-up period,the average improvement in ROM of the shoulder and elbow joints was 30.0°,the wrist joint was improved by 17.5°,the metacarpophalangeal joint of the thumb and the other four fingers were improved by 32.5° and 50.0°,and the proximal interphalangeal joint was improved by 40.0°.Rehabilitation treatment started before the age of 6,and the improvement in ROM was better than that after the age of 6 (P < 0.05).Conclusions Systematic rehabilitation therapy should be initiated as early as possible for multiple joint contractures,requiring long-term persistence and multidisciplinary involvement.This can help improve limb function and enhance the quality of life of patients.更多还原