Review of the recurrent 8q13.2q13.3 branchio-oto-renal related microdeletion, and report of an additional case with associated distal arthrogryposis

Am J Med Genet A. 2016 Nov;170(11):2984-2987. doi: 10.1002/ajmg.a.37695. Epub 2016 Aug 19.

Abstract

Recurrent 2.65 Mb deletions of 8q13.2q13.3 encompassing EYA1 have been recently described in the literature as a cause of branchio-oto-renal syndrome (BOR). Other clinical features of this recurrent microdeletion syndrome are still being delineated. We describe an additional patient with BOR due to microdeletion of 8q13.2q13.3. In addition to BOR related features, our patient presented with distal arthrogryposis that was detected prenatally, a phenotype that has not previously been described in patients with this deletion. © 2016 Wiley Periodicals, Inc.

Keywords: 8q13; EYA1; NCOA2; SULF1; branchio-oto-renal; congenital; distal arthrogryposis; hand deformities; microarray analysis; microdeletion.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Arthrogryposis / diagnosis*
  • Arthrogryposis / genetics*
  • Branchio-Oto-Renal Syndrome / diagnosis*
  • Branchio-Oto-Renal Syndrome / genetics*
  • Branchio-Oto-Renal Syndrome / therapy
  • Chromosome Deletion*
  • Chromosomes, Human, Pair 8*
  • Comparative Genomic Hybridization
  • Facies
  • Female
  • Humans
  • Infant
  • Male
  • Phenotype
  • Pregnancy
  • Recurrence
  • Ultrasonography, Prenatal