Combined immunodeficiency in a 3-year-old boy with 16p11.2 and 20p12.2-11.2 chromosomal duplications

Am J Med Genet A. 2014 Feb;164A(2):535-41. doi: 10.1002/ajmg.a.36305. Epub 2013 Dec 5.

Abstract

We report for the first time on a 3-year-old boy with paternally inherited 212.85 kb-16p11.2 and 7.8 Mb-20p12.2-11.23 interstitial microduplications associated with having congenital cardiac defect, dysmorphic facial features, and combined T-, B-, and NK cell immunodeficiency. In addition the 7.8 Mb-20p12.2-11.23 microduplication is unique showing novel breakpoints among all partial trisomy/duplication 20p reported to date, narrowing down the critical region for trisomy 20p syndrome.

Keywords: 16p11.2 duplication; 20p12.2-11.23 duplication; combined immunodeficiency; congenital heart disease; partial trisomy 20p syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Child, Preschool
  • Chromosome Duplication*
  • Chromosomes, Human, Pair 16*
  • Chromosomes, Human, Pair 20*
  • Comparative Genomic Hybridization
  • Facies
  • Humans
  • Male
  • Severe Combined Immunodeficiency / diagnosis
  • Severe Combined Immunodeficiency / genetics*
  • Severe Combined Immunodeficiency / immunology*