Common data elements for arthrogryposis multiplex congenita: An international framework

Dev Med Child Neurol. 2024 Oct;66(10):1340-1347. doi: 10.1111/dmcn.15898. Epub 2024 Mar 16.

Abstract

Aim: To facilitate multisite studies and international clinical research, this study aimed to identify consensus-based, standardized common data elements (CDEs) for arthrogryposis multiplex congenita (AMC).

Method: A mixed-methods study comprising of several focus group discussions and three rounds of modified Delphi surveys to achieve consensus using two tiered-rating scales were conducted.

Results: Overall, 45 clinical experts and adults with lived experience (including 12 members of an AMC consortium) participated in this study from 11 countries in North America, Europe, and Australia. The CDEs include 321 data elements and 19 standardized measures across various domains from fetal development to adulthood. Data elements pertaining to AMC phenotypic traits were mapped according to the Human Phenotype Ontology. A universal governance structure, local operating protocols, and sustainability plans were identified as the main facilitators, whereas limited capacity for data sharing and the need for a federated informatics infrastructure were the main barriers.

Interpretation: Collection of systematic data on AMC using CDEs will allow investigations on etiological pathways, describe epidemiological profile, and establish genotype-phenotype correlations in a standardized manner. The proposed CDEs will facilitate international multidisciplinary collaborations by improving large-scale studies and opportunities for data sharing, knowledge translation, and dissemination.

What this paper adds: The common data elements (CDEs) for arthrogryposis multiplex congenita include 321 data elements from perinatal period to adulthood. A universal governance structure, partner-specific operating protocols, and sustainability plans will facilitate multi-institutional implementation of CDEs. Inconsistent electronic infrastructure, different terminologies for databases, and feasibility issues are the main barriers for international implementation of CDEs.

MeSH terms

  • Adult
  • Arthrogryposis* / diagnosis
  • Australia
  • Common Data Elements*
  • Consensus
  • Delphi Technique
  • Europe
  • Humans
  • Phenotype