Cornelia de Lange syndrome (CdLS): prenatal and autopsy findings

Prenat Diagn. 2009 May;29(5):489-94. doi: 10.1002/pd.2228.

Abstract

Cornelia de Lange Syndrome (CdLS) is a multisystem disorder characterized by somatic defects and mental retardation. Prenatal diagnosis of this severe condition is difficult in view of the non-specific ultrasound abnormalities. We report three cases with prenatally suspected CdLS based on the ultrasound findings as well as low PAPP-A detected on first trimester screening in one case, and the results of the autopsy and the NIPBL gene mutation analysis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autopsy
  • Cell Cycle Proteins
  • De Lange Syndrome / diagnosis*
  • De Lange Syndrome / pathology*
  • Female
  • Humans
  • Male
  • Mutation
  • Pregnancy
  • Pregnancy-Associated Plasma Protein-A / analysis
  • Pregnancy-Associated Plasma Protein-A / genetics
  • Proteins / analysis
  • Proteins / genetics
  • Ultrasonography, Prenatal*
  • Young Adult

Substances

  • Cell Cycle Proteins
  • NIPBL protein, human
  • Proteins
  • Pregnancy-Associated Plasma Protein-A