Literature DB >> 23653585

Report on a patient with a 12q24.31 microdeletion inherited from an insulin-dependent diabetes mellitus father.

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


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