17p13.3 quadruplication: a prenatal and postpartum clinical characterization of a copy number variant

Cold Spring Harb Mol Case Stud. 2018 Jun 1;4(3):a002196. doi: 10.1101/mcs.a002196. Print 2018 Jun.

Abstract

Prenatal genetic testing has advanced rapidly in the past decade. However, not all results, including variants, are well understood. We report the finding of a 2.5-Mb gene region quadruplication of Chromosome 17p13.3. This region is well characterized for the deletion leading to Miller-Dieker syndrome but has an unclear replication phenotype. Invasive testing performed after ultrasound abnormalities were seen revealed the quadruplication sequence as well as a short segment (850 kb) with x5 copy number variation. This region has previously been reported in a collection of duplications with shared phenotype; our quadruplication suggests similarities in phenotype. This raises the hypothesis of a potential spectrum or copy number variant-based phenotype.

Keywords: aplasia/hypoplasia of the corpus callosum; congenital microcephaly; facial hypotonia; mild fetal ventriculomegaly; severe global developmental delay.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Chromosome Banding
  • Chromosome Duplication*
  • Chromosomes, Human, Pair 17*
  • Classical Lissencephalies and Subcortical Band Heterotopias / diagnosis
  • Classical Lissencephalies and Subcortical Band Heterotopias / genetics
  • DNA Copy Number Variations*
  • Female
  • Genetic Association Studies*
  • Genome-Wide Association Study / methods
  • Humans
  • In Situ Hybridization, Fluorescence
  • Infant
  • Phenotype*
  • Postpartum Period
  • Pregnancy
  • Prenatal Diagnosis
  • Ultrasonography, Prenatal