Literature DB >> 22246919

A de novo interstitial deletion of 2p23.3-24.3 in a boy presenting with intellectual disability, overgrowth, dysmorphic features, skeletal myopathy, dilated cardiomyopathy.

Moneef Shoukier1, Julia Schröder, Barbara Zoll, Peter Burfeind, Clemens Freiberg, Lars Klinge, Thomas Kriebel, Michael Lingen, Alexander Mohr, Knut Brockmann.   

Abstract

Interstitial deletions of the distal part of chromosome 2p are rare, with only six reported cases involving regions from 2p23 to 2pter. Most of these were cytogenetic investigations. We describe a 14-year-old boy with an 8.97 Mb deletion of 2p23.3-24.3 detected by array comparative genomic hybridization (array CGH) who had intellectual disability (ID), unusual facial features, cryptorchidism, skeletal myopathy, dilated cardiomyopathy (DCM), and postnatal overgrowth (macrocephaly and tall stature). We compared the clinical features of the present case to previously described patients with an interstitial deletion within this chromosomal region and conclude that our patient exhibits a markedly different phenotype. Additional patients are needed to further delineate phenotype-genotype correlations.
Copyright © 2012 Wiley Periodicals, Inc.

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Year:  2012        PMID: 22246919     DOI: 10.1002/ajmg.a.34427

Source DB:  PubMed          Journal:  Am J Med Genet A        ISSN: 1552-4825            Impact factor:   2.802


  3 in total

1.  Aortic root dilatation and dilated cardiomyopathy in an adult with Tatton-Brown-Rahman syndrome.

Authors:  Alana C Cecchi; Amier Haidar; Isabella Marin; Callie S Kwartler; Siddharth K Prakash; Dianna M Milewicz
Journal:  Am J Med Genet A       Date:  2021-10-13       Impact factor: 2.578

2.  PTRHD1 (C2orf79) mutations lead to autosomal-recessive intellectual disability and parkinsonism.

Authors:  Hamidreza Khodadadi; Luis J Azcona; Vajiheh Aghamollaii; Mir Davood Omrani; Masoud Garshasbi; Shaghayegh Taghavi; Abbas Tafakhori; Gholam Ali Shahidi; Javad Jamshidi; Hossein Darvish; Coro Paisán-Ruiz
Journal:  Mov Disord       Date:  2016-10-18       Impact factor: 10.338

3.  Co-occurrence of a maternally inherited DNMT3A duplication and a paternally inherited pathogenic variant in EZH2 in a child with growth retardation and severe short stature: atypical Weaver syndrome or evidence of a DNMT3A dosage effect?

Authors:  Katarzyna Polonis; Patrick R Blackburn; Raul A Urrutia; Gwen A Lomberk; Teresa Kruisselbrink; Margot A Cousin; Nicole J Boczek; Nicole L Hoppman; Dusica Babovic-Vuksanovic; Eric W Klee; Pavel N Pichurin
Journal:  Cold Spring Harb Mol Case Stud       Date:  2018-08-01
  3 in total

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