| Literature DB >> 23653585 |
E Chouery1, N Choucair, J Abou Ghoch, S El Sabbagh, S Corbani, A Mégarbané.
Abstract
We report a 2.3-year-old female patient with global developmental delay, infantile spasms, hypotonia, microcephaly, flat face, full cheeks, macroglossia, highly arched palate, retro-gnathia, narrow ear orifices, and café-au-lait spots. Molecular karyotyping revealed approximately a 1-Mb interstitial deletion of the long arm of one chromosome 12, del(12)(q24.31). The same deletion was identified in her father who presents insulin-dependent diabetes mellitus (IDDM) diagnosed at 14 years. Only one other patient with a similar de novo deletion has been reported previously [Mol Syndromol 2010;1:42-45]. A phenotype-genotype correlation is discussed, and the description of a novel rare microdeletion entity is raised.Entities:
Keywords: Chromosome 12q; Developmental delay; HNF1A; Haploinsufficient; Microdeletion 12q24.31
Year: 2013 PMID: 23653585 PMCID: PMC3638924 DOI: 10.1159/000346473
Source DB: PubMed Journal: Mol Syndromol ISSN: 1661-8769