Literature DB >> 23523603

Mowat-Wilson syndrome in a fetus with antenatal diagnosis of short corpus callosum: advocacy for standard autopsy.

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.
Copyright © 2013 Elsevier Masson SAS. All rights reserved.

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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


  2 in total

1.  Experience of Mowat-Wilson syndrome prenatal diagnosis for a Chinese family.

Authors:  Qian Jiang; Xiaoxiao Zhang; Yinan Ma; Qi Li; Chunhua Zheng; Yuchun Yan; Zhen Zhang; Ping Xiao; Lin Su; Wei Cheng; Hong Pan; Long Li
Journal:  Clin Case Rep       Date:  2016-11-17

2.  Neuropathology of genetically defined malformations of cortical development-A systematic literature review.

Authors:  Stefanie Brock; Filip Cools; Anna C Jansen
Journal:  Neuropathol Appl Neurobiol       Date:  2021-02-14       Impact factor: 8.090

  2 in total

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