Literature DB >> 15581840

A submicroscopic unbalanced subtelomeric translocation t(2p;10q) identified by fluorescence in situ hybridization: fetus with increased nuchal translucency and normal standard karyotype with later growth and developmental delay, rhombencephalosynapsis (RES).

J Lespinasse1, H Testard, F Nugues, M Till, M P Cordier, M Althuser, F Amblard, S Fert-Ferrer, C Durand, F Dalmon, C Pourcel, P S Jouk.   

Abstract

Reaching an accurate diagnosis in children with mental retardation associated or not with dysmorphic signs is important to make precise diagnosis of a syndrome and for genetic counseling. A female case with severe growth and development delay, dysmorphic features and feeding disorder is presented. Antenataly, the fetus was observed to have increased nuchal translucency and a slight hypoplastic cerebellum. A standard karyotype was normal. RES and a submicroscopic unbalanced subtelomeric translocation t(2p; 10q) were demonstrated after birth. We show that within the framework of a collaborative approach, a concerted research of submicroscopic subtelomeric rearrangements should be performed in case of mental retardation associated with facial dysmorphic features, and when other etiologies or non-genetic factors (iatrogenic, toxic, infectious, metabolic...) have been ruled out.

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Year:  2004        PMID: 15581840     DOI: 10.1016/j.anngen.2004.07.005

Source DB:  PubMed          Journal:  Ann Genet        ISSN: 0003-3995


  6 in total

1.  Recurrent partial rhombencephalosynapsis and holoprosencephaly in siblings with a mutation of ZIC2.

Authors:  Melissa B Ramocki; Fernando Scaglia; Pawel Stankiewicz; John W Belmont; Jeremy Y Jones; Gary D Clark
Journal:  Am J Med Genet A       Date:  2011-06-02       Impact factor: 2.802

2.  Rhombencephalosynapsis in a severely polymalformed fetus with non-mosaic tetrasomy 9p, in intracytoplasmic-sperm-injection pregnancy.

Authors:  Elena di Vera; Marco Liberati; Claudio Celentano; Giuseppe Calabrese; Paolo Emilio Guanciali-Franchi; Elisena Morizio; Sigfried Rotmensch
Journal:  J Assist Reprod Genet       Date:  2008-10-25       Impact factor: 3.412

3.  Array-CGH Analysis Suggests Genetic Heterogeneity in Rhombencephalosynapsis.

Authors:  F Démurger; L Pasquier; C Dubourg; V Dupé; I Gicquel; C Evain; L Ratié; S Jaillard; M Beri; B Leheup; J Lespinasse; D Martin-Coignard; S Mercier; C Quelin; P Loget; P Marcorelles; A Laquerrière; C Bendavid; S Odent; V David
Journal:  Mol Syndromol       Date:  2013-08-01

Review 4.  Rhombencephalosynapsis: Fused cerebellum, confused geneticists.

Authors:  Kimberly A Aldinger; Jennifer C Dempsey; Hannah M Tully; Megan E Grout; Michele G Mehaffey; William B Dobyns; Dan Doherty
Journal:  Am J Med Genet C Semin Med Genet       Date:  2018-12       Impact factor: 3.908

5.  Rhombencephalosynapsis: a hindbrain malformation associated with incomplete separation of midbrain and forebrain, hydrocephalus and a broad spectrum of severity.

Authors:  Gisele E Ishak; Jennifer C Dempsey; Dennis W W Shaw; Hannah Tully; Margaret P Adam; Pedro A Sanchez-Lara; Ian Glass; Tessa C Rue; Kathleen J Millen; William B Dobyns; Dan Doherty
Journal:  Brain       Date:  2012-03-26       Impact factor: 13.501

6.  Beyond Gómez-López-Hernández syndrome: recurring phenotypic themes in rhombencephalosynapsis.

Authors:  Hannah M Tully; Jennifer C Dempsey; Gisele E Ishak; Margaret P Adam; Cynthia J R Curry; Pedro Sanchez-Lara; Alasdair Hunter; Karen W Gripp; Judith Allanson; Christopher Cunniff; Ian Glass; Kathleen J Millen; Daniel Doherty; William B Dobyns
Journal:  Am J Med Genet A       Date:  2012-09-10       Impact factor: 2.802

  6 in total

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