| Literature DB >> 26059276 |
Zornitza Stark1, Joanna Behrsin2, Trent Burgess1,3, Anna Ritchie1, Alison Yeung1, Tiong Y Tan1,3, Natasha J Brown1,3, Ravi Savarirayan1,3, Neil Patel2.
Abstract
Chromosomal abnormalities are an important factor in the pathogenesis of congenital diaphragmatic hernia (CDH), a relatively common congenital defect associated with high morbidity and mortality. The adoption of array-based platforms for chromosome analysis has resulted in the identification of numerous copy number variants (CNVs) in infants with CDH, highlighting the potential pathogenic role of many novel genes. We identified a retrospective cohort of 28 infants treated for CDH at a single institution who had microarray testing to determine the proportion of microarray abnormalities and whether these were contributory to CDH pathogenesis. Eight patients (29%) had microarray abnormality. Seven (25%) were considered likely contributory to CDH pathogenesis, including two mosaic trisomy 9s, a 9q22.31q22.32 microduplication, two atypical 22q11.21 microdeletions, a 2q35q36.1 microdeletion, and a 15q11.2 microdeletion, offering insights into the genetic mechanisms underlying CDH development.Entities:
Keywords: 9q22.3 duplication; CRKL; PAX3; TBX1; congenital diaphragmatic hernia; microarray
Mesh:
Year: 2015 PMID: 26059276 DOI: 10.1002/ajmg.a.37177
Source DB: PubMed Journal: Am J Med Genet A ISSN: 1552-4825 Impact factor: 2.802