Shriners Hospital for Children-Canada, Montreal, Canada.
Schulich Library of Physical Sciences, Life Sciences, and Engineering, McGill University, Montreal, Canada.
Health Qual Life Outcomes. 2021 Nov 29;19(1):263. doi: 10.1186/s12955-021-01896-5.
Arthrogryposis multiplex congenita (AMC) is a group of congenital conditions characterized by joint contractures in two or more body areas. Management of AMC starts early in life and focuses on improving mobility and function through intensive rehabilitation and surgical interventions. Psychosocial wellbeing is an important determinant of health and the psychosocial experience of individuals with AMC should be considered in the management of this condition. The aim of this scoping review was to explore what is known about the psychosocial wellbeing of children and adults with AMC, to identify the outcome measures used and to explore the factors associated with psychosocial outcomes in this population.
A comprehensive search in four databases was conducted. Articles discussing psychosocial outcomes and outcome measures used with children or adults with AMC were included. Data on the measures used, psychosocial outcomes, and factors associated with psychosocial outcomes, were extracted and analyzed descriptively and synthesized narratively.
Seventeen articles were included in this scoping review, ten including the pediatric population, six including adults and one article including both children and adults with AMC. The most commonly used outcome measures were the PODCI in the pediatric studies, and the SF-36 in studies on adults. In the pediatric studies, psychosocial outcomes were often secondary, compared to the studies on adults. Results showed that in both children and adults, psychosocial outcomes are comparable with the levels of the general population. Qualitative studies reflected the affective needs of this population and issues with emotional wellbeing. Factors such as fatigue and pain were associated with poorer psychosocial outcomes in adults with an impact on social relationships, intimacy and family planning.
Validated outcome measures, qualitative approaches and longitudinal studies are needed to better understand the psychosocial outcomes in AMC over time. Psychosocial support should be part of the multidisciplinary management of AMC throughout the lifespan.
先天性多发性关节挛缩症（AMC）是一组以两个或多个身体部位关节挛缩为特征的先天性疾病。AMC 的管理始于生命早期，重点是通过强化康复和手术干预来提高活动能力和功能。心理健康是健康的一个重要决定因素，应该在管理这种疾病时考虑到 AMC 患者的心理社会体验。本综述的目的是探讨已知的儿童和成人 AMC 患者的心理社会健康状况，确定用于评估的结果测量方法，并探讨该人群中与心理社会结果相关的因素。
在四个数据库中进行了全面搜索。纳入了讨论儿童或成人 AMC 患者心理社会结果和使用的结果测量方法的文章。提取并描述性地分析了有关使用的措施、心理社会结果以及与心理社会结果相关的因素的数据，并进行了综合叙述。
本范围综述纳入了 17 篇文章，其中 10 篇纳入了儿科人群，6 篇纳入了成人，1 篇纳入了儿童和成人 AMC 患者。最常用的结果测量方法是儿科研究中的 PODCI 和成人研究中的 SF-36。在儿科研究中，心理社会结果往往次于成人研究。结果表明，在儿童和成人中，心理社会结果与普通人群的水平相当。定性研究反映了该人群的情感需求和情绪健康问题。疲劳和疼痛等因素与成人较差的心理社会结果相关，对社交关系、亲密关系和计划生育产生影响。
需要使用经过验证的结果测量方法、定性方法和纵向研究，以便更好地了解 AMC 随时间推移的心理社会结果。心理社会支持应成为 AMC 多学科管理的一部分，贯穿整个生命周期。
