Progressive extreme heterotopic calcification

Am J Med Genet A. 2013 Jul;161A(7):1706-13. doi: 10.1002/ajmg.a.35944. Epub 2013 May 17.

Abstract

The formation of ectopic calcifications in soft tissues can occur either sporadically or as a genetically determined condition, and is seen only infrequently. We report on a girl in whom widespread, rapidly progressive ectopic calcifications were detected shortly after birth. Calcifications became present around all joints, tendons and ligaments, but did not involve internal organs and skin, and eventually caused almost complete immobility of the child at 2 years. There were no other health problems and cognitive development was normal. We compare the manifestations in the child to the characteristics of known entities causing ectopic calcifications and conclude the child differs to each. Laboratory evaluation failed to identify autoimmune phenomena as well as calcium metabolism or other biochemical abnormalities; molecular studies did not identify occurrence of mutations in disease genes known to be involved in ectopic calcifications. We conclude the manifestations in the child represent an unreported entity of hitherto unknown etiology.

Publication types

  • Case Reports

MeSH terms

  • Activin Receptors, Type I / genetics
  • Calcinosis / etiology*
  • Calcinosis / genetics
  • Child, Preschool
  • Chromogranins
  • Female
  • GTP-Binding Protein alpha Subunits, Gs / genetics
  • Humans
  • Infant
  • Ligaments / pathology
  • Magnetic Resonance Imaging
  • Male
  • Mutation
  • Tendons / pathology

Substances

  • Chromogranins
  • ACVR1 protein, human
  • Activin Receptors, Type I
  • GNAS protein, human
  • GTP-Binding Protein alpha Subunits, Gs