Disease coding systems for arthrogryposis multiplex congenita

Am J Med Genet C Semin Med Genet. 2019 Sep;181(3):304-309. doi: 10.1002/ajmg.c.31718. Epub 2019 Jun 24.

Abstract

Arthrogryposis multiplex congenita (AMC) encompasses many different conditions, involves many different genes and thus can be very complex. Using historical disease coding systems to document syndrome diagnoses and anomalies associated with AMC is often challenging. However, disease coding systems are necessary to provide opportunities for a standard language to be maintained and pertinent data to be identified in the pediatric AMC registry, congenital anomalies surveillance systems, and routine or administrative health information systems. The ICD-10, Orphanet, Online Mendelian Inheritance in Man, and the Human Phenotype Ontology coding and classification systems are described to establish a comprehensive coding strategy. This strategy will provide a necessary tool to contribute to a better understanding of AMC and ultimately improve the health of individuals with AMC.

MeSH terms

  • Arthrogryposis / diagnosis*
  • Arthrogryposis / genetics*
  • Gene Ontology
  • Humans
  • Phenotype