Wall Lindley B, Vuillerman Carley, Miller Patricia E, Bae Donald S, Goldfarb Charles A
Department of Orthopaedic Surgery, Washington University School of Medicine, Saint Louis, MO.
Department of Orthopaedic Surgery, Boston Children's Hospital, Boston, MA.
J Pediatr Orthop. 2020 Aug;40(7):357-360. doi: 10.1097/BPO.0000000000001527.
Little is known about patient-reported health status in children and adolescents with arthrogryposis. Utilizing the Patient-Reported Outcome Measurement Information System (PROMIS) and Pediatric Outcomes Data Collection Instrument (PODCI) questionnaires, we investigated functional and psychosocial measures in arthrogryposis.
A total of 118 patients with arthrogryposis were identified from a prospective longitudinal cohort (the Congenital Upper Limb Difference Registry) from 2014 to 2018. Demographics and patient-reported outcome measures were evaluated, including the PROMIS [upper extremity (UE) function, pain, depression, anxiety, and peer relations] and PODCI questionnaires (UE function, pain, happiness, and global function).
A total of 29 arthrogrypotic patients had complete PROMIS and PODCI data. This cohort was divided into distal arthrogryposis and amyoplasia groups, with 15 and 14 patients in each group, respectively. There were 8 males in the distal arthrogryposis group with a median age of 9 years and 7 males in the amyoplasia group with a median age of 8 years. For both cohorts, the median UE function PROMIS scores were significantly below population norms, 31 for distal arthrogryposis and 22 for amyoplasia. PODCI UE function was statistically lower for amyoplasia compared with the distal arthrogryposis cohort. PROMIS pain, depression, anxiety, and peer relations were in the normal range for both amyopasia and distal arthrogryposis. Median PODCI pain and happiness ranged from 85 to 88 for all patients with no statistical difference between groups.
Arthrogryposis patients have lower UE function scores compared with population normals, but they have emotional states that are consistent with populations norms. Amyoplasia patients were functionally worse than distal arthrogryposis patients.
Level II.
关于关节挛缩症儿童和青少年患者自我报告的健康状况，人们了解甚少。我们使用患者报告结局测量信息系统（PROMIS）和儿科结局数据收集工具（PODCI）问卷，对关节挛缩症患者的功能和心理社会指标进行了调查。
从2014年至2018年的前瞻性纵向队列（先天性上肢差异登记处）中识别出118例关节挛缩症患者。对人口统计学和患者报告的结局指标进行了评估，包括PROMIS[上肢（UE）功能、疼痛、抑郁、焦虑和同伴关系]和PODCI问卷（UE功能、疼痛、幸福感和整体功能）。
共有29例关节挛缩症患者有完整的PROMIS和PODCI数据。该队列分为远端关节挛缩症组和先天性肌发育不全组，每组分别有15例和14例患者。远端关节挛缩症组有8名男性，中位年龄为9岁；先天性肌发育不全组有7名男性，中位年龄为8岁。对于这两个队列，UE功能PROMIS评分中位数均显著低于人群正常值，远端关节挛缩症组为31，先天性肌发育不全组为22。与远端关节挛缩症队列相比，先天性肌发育不全组的PODCI UE功能在统计学上更低。PROMIS疼痛、抑郁、焦虑和同伴关系在先天性肌发育不全组和远端关节挛缩症组中均处于正常范围。所有患者的PODCI疼痛和幸福感中位数在85至88之间，两组之间无统计学差异。
与人群正常值相比，关节挛缩症患者的UE功能评分较低，但他们的情绪状态与人群正常值一致。先天性肌发育不全患者的功能比远端关节挛缩症患者更差。
二级。
