[Genetic diagnosis of a child with aortic stenosis and thumb aplasia]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2018 Aug 10;35(4):531-534. doi: 10.3760/cma.j.issn.1003-9406.2018.04.016.
[Article in Chinese]

Abstract

Objective: To analyze the molecular mechanism and prognosis of a child with aortic stenosis and thumb aplasia.

Methods: The karotypes of the child and his parents were analyzed with routine G-banding. Their genomic DNA was also analyzed with array comparative genomic hybridization(aCGH) for chromosomal duplications/deletions.

Results: No karyotypic abnormality was detected at cytogenetic level for the child and his parents. aCGH identified a de novo 5.86 Mb deletion at 2q22.3-q23.3 in the child.

Conclusion: The child was diagnosed with 2q23.1 microdeletion syndrome. MBD5 may be the key gene for the 2q23.1 microdeletion syndrome.

Publication types

  • Case Reports

MeSH terms

  • Aortic Valve Stenosis / genetics*
  • Child
  • Chromosome Banding
  • Chromosome Deletion
  • Chromosome Disorders / diagnosis
  • Chromosome Disorders / genetics*
  • Chromosomes, Human, Pair 2 / genetics*
  • Comparative Genomic Hybridization
  • DNA-Binding Proteins / genetics
  • Humans
  • Male
  • Thumb / abnormalities*

Substances

  • DNA-Binding Proteins
  • MBD5 protein, human