[Lethal osteogenesis imperfecta. Prenatal diagnosis]

Presse Med. 2004 Jun 5;33(10):658-60. doi: 10.1016/s0755-4982(04)98709-x.
[Article in French]

Abstract

Introduction: Osteogenesis imparfecta (OI) comprises a group of disorders principally affecting type I collagen, which result in increased bone fragility. Lethal forms are rare and are characterised by micromelia with malformation of the limbs.

Case report: A prenatal diagnosis of lethal OI was made by ultrasonography at 18 weeks of gestation and therapeutic abortion was indicated.

Comments: Molecular biology and genetic studies offer new possibilities of prenatal diagnosis, but ultrasonography remains the investigation of choice. It confirms the diagnosis by revealing an increase in bone transparency.

Publication types

  • Case Reports

MeSH terms

  • Abortion, Therapeutic
  • Adult
  • Amniocentesis
  • Collagen / genetics
  • Collagen Type I / genetics
  • Collagen Type I, alpha 1 Chain
  • Female
  • Genetic Counseling
  • Humans
  • Mutation / genetics
  • Osteogenesis Imperfecta / classification
  • Osteogenesis Imperfecta / diagnosis*
  • Osteogenesis Imperfecta / epidemiology
  • Osteogenesis Imperfecta / genetics
  • Pedigree
  • Pregnancy
  • Pregnancy Trimester, Second
  • Thanatophoric Dysplasia / genetics
  • Ultrasonography, Prenatal* / methods

Substances

  • Collagen Type I
  • Collagen Type I, alpha 1 Chain
  • Collagen