Abstract：
Arthrogryposis multiplex congenita (AMC) is defined as \"a group of congenital conditions characterized by joint contractures in two or more body areas.\" Given its heterogeneity, the definition of AMC has changed multiple times. This scoping review provides an overview of how AMC is defined in scientific publications, on existing knowledge and trends regarding the concept of AMC. Our review illuminates possible knowledge gaps and provides directions for future research. A scoping review was conducted in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews guidelines. Quantitative studies on AMC from 1995 to date were included. We summarized information about definitions/descriptions of AMC, study objectives, study designs, methods, funding, and involvement of patient organizations. A total of 2729 references were screened, and 141 articles fulfilled our inclusion criteria. Our scoping revealed that the majority of publications were cross-sectional or retrospective studies of children and young people, commonly about orthopedic management. Explicit or good definitions of AMC were provided in 86% of the cases. Recent publications on AMC mostly used consensus-based definitions. The research gaps were primarily related to adults, aging, etiology, and new medical treatment, in addition to implications on daily life.
摘要：
多发性先天性关节病（AMC）定义为一组先天性疾病，其特征是两个或多个身体区域的关节挛缩。“鉴于它的异质性，AMC的定义已多次更改。本范围审查概述了如何在科学出版物中定义AMC，关于AMC概念的现有知识和趋势。我们的评论阐明了可能的知识差距，并为未来的研究提供了方向。根据范围审查指南的系统审查和荟萃分析扩展的首选报告项目进行范围审查。包括1995年至今对AMC的定量研究。我们总结了有关AMC定义/描述的信息，研究目标,研究设计，方法,资金,以及患者组织的参与。总共筛选了2729个参考文献，141篇文章符合我们的纳入标准。我们的范围研究表明，大多数出版物都是针对儿童和年轻人的横断面或回顾性研究，通常关于骨科管理。在86％的病例中提供了AMC的明确或良好定义。最近关于AMC的出版物大多使用基于共识的定义。研究差距主要与成年人有关，老化，病因学,和新的医疗方法，除了对日常生活的影响。
