Branchial arch defects and 19p13.12 microdeletion: defining the critical region into a 0.8 M base interval

Am J Med Genet A. 2011 Sep;155A(9):2212-4. doi: 10.1002/ajmg.a.33908. Epub 2011 Aug 3.

Abstract

We present a patient with preauricular tags, preauricular and branchial pits, stenosis of the external auditory canals, mild hearing loss, and mild developmental delay who had a de novo 19p13.12 submicroscopic deletion. The size of the deletion was 760-kb, extending from 15,300,338 to 16,064,271 (hg18; NCBI Build 36.1). Our finding supports the notion that 19p13.12 represents a unique microdeletion syndrome characterized by branchial arch defects and the concept of exclusion mapping indicates that the putative locus for the branchial arch development is included in the 0.8-Mb interval defined by the deletion in the presently reported patient.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple / genetics
  • Branchial Region / abnormalities*
  • Child
  • Chromosome Deletion*
  • Chromosomes, Human, Pair 19 / genetics*
  • Constriction, Pathologic
  • Developmental Disabilities / genetics
  • Ear, External / abnormalities
  • Female
  • Humans
  • Japan
  • Phenotype