| Literature DB >> 23523603 |
Emmanuel Spaggiari1, Clarisse Baumann, Marianne Alison, Jean-François Oury, Nadia Belarbi, Céline Dupont, Fabien Guimiot, Anne-Lise Delezoide.
Abstract
Mowat-Wilson syndrome (MWS) is a genetic disease caused by heterozygous mutations or deletions of the ZEB2 gene rarely diagnosed prenatally and with little fetal description reported. It is mainly characterized by moderate-to-severe intellectual disability, epilepsy, facial dysmorphism and various malformations including Hirschsprung disease and corpus callosum anomalies. Here we report a fetal case of MWS well described, suspected at standard autopsy. The association of a corpus callosum hypoplasia with a histological Hirschsprung disease and a typical facial gestalt allowed the guiding of genetic testing. Classical fetopathological examination still keeps indications in cases of syndromic association in the era of virtual autopsy.Entities:
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Year: 2013 PMID: 23523603 DOI: 10.1016/j.ejmg.2013.03.003
Source DB: PubMed Journal: Eur J Med Genet ISSN: 1769-7212 Impact factor: 2.708