[Genetic diagnosis and noninvasive prenatal testing of a family with Williams-Beuren syndrome]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2019 Mar 10;36(3):263-266. doi: 10.3760/cma.j.issn.1003-9406.2019.03.018.
[Article in Chinese]

Abstract

Objective: To explore the genetic basis of a fetus with ventricular septal defect (VSD) by using modified noninvasive prenatal testing (NIPT) for the detection of microdeletion syndromes.

Methods: Chromosomal karyotypes of the fetus and its parents were analyzed by G-banding technique. Next generation sequencing (NGS) was used to detect genomic copy number variations (CNVs) in cell-free fetal DNA. The results were verified by fluorescence in situ hybridization (FISH).

Results: The fetus and its parents all had a normal karyotype at 320-400 band level. NGS revealed a deletion of 1.30 Mb at 7q11.23 in the fetus, with a 93% overlap with that of Williams-Beuren syndrome (WBS). The father also had a deletion of 1.42 Mb at 7q11.23, with a 99% overlap with that of WBS. Modified NIPT also detected the 1.30 Mb deletion at 7q11.23 in the fetus. The result of FISH has confirmed the above results.

Conclusion: It is necessary to carry out genetic testing on fetuses with VSD. NGS can detect fetal microdeletion syndromes and help to trace their parental origin. The modified NIPT for fetal chromosomal microdeletions/microduplication syndromes is highly accurate.

MeSH terms

  • DNA Copy Number Variations
  • Female
  • Genetic Testing
  • Humans
  • In Situ Hybridization, Fluorescence
  • Karyotyping
  • Pregnancy
  • Prenatal Diagnosis
  • Williams Syndrome*