Literature DB >> 34303382

Partial deletion of chromosome 6p causing developmental delay and mild dysmorphisms in a child: molecular and developmental investigation and literature search.

Nikolaos Vrachnis1,2,3, Ioannis Papoulidis4, Dionysios Vrachnis5, Elisavet Siomou4, Nikolaos Antonakopoulos6,7, Stavroula Oikonomou8, Dimitrios Zygouris7, Nikolaos Loukas9, Zoi Iliodromiti10, Efterpi Pavlidou11, Loretta Thomaidis8, Emmanouil Manolakos4.   

Abstract

BACKGROUND: The interstitial 6p22.3 deletions concern rare chromosomal events affecting numerous aspects of both physical and mental development. The syndrome is characterized by partial deletion of chromosome 6, which may arise in a number of ways. CASE
PRESENTATION: We report a 2.8-year old boy presenting with developmental delay and mild dysmorphisms. High-resolution oligonucleotide microarray analysis revealed with high precision a 2.5 Mb interstitial 6p deletion in the 6p22.3 region which encompasses 13 genes.
CONCLUSIONS: Identification and in-depth analysis of cases presenting with mild features of the syndrome will sharpen our understanding of the genetic spectrum of the 6p22.3 deletion.
© 2021. The Author(s).

Entities:  

Keywords:  6p22.3 deletion; Behavioral abnormalities; Developmental delay; Dysmorphism; High-resolution microarray analysis; Intellectual disability; Syndrome

Year:  2021        PMID: 34303382     DOI: 10.1186/s13039-021-00557-y

Source DB:  PubMed          Journal:  Mol Cytogenet        ISSN: 1755-8166            Impact factor:   2.009


  31 in total

1.  Delineation of two distinct 6p deletion syndromes.

Authors:  A F Davies; G Mirza; G Sekhon; P Turnpenny; F Leroy; F Speleman; C Law; N van Regemorter; E Vamos; F Flinter; J Ragoussis
Journal:  Hum Genet       Date:  1999-01       Impact factor: 4.132

2.  Polyneuropathy, scoliosis, tall stature, and oligodontia represent novel features of the interstitial 6p deletion phenotype.

Authors:  Birgit Zirn; Maja Hempel; Andreas Hahn; Bernd Neubauer; Janine Wagenstaller; Núria Rivera-Bruguès; Tim Matthias Strom; Angelika Köhler
Journal:  Am J Med Genet A       Date:  2008-11-15       Impact factor: 2.802

3.  A detailed investigation of two cases exhibiting characteristics of the 6p deletion syndrome.

Authors:  A F Davies; M G Olavesen; R J Stephens; R Davidson; D Delneste; N Van Regemorter; E Vamos; F Flinter; I Abusaad; J Ragoussis
Journal:  Hum Genet       Date:  1996-10       Impact factor: 4.132

4.  Focal segmental glomerulosclerosis and partial deletion of chromosome 6p: a case report.

Authors:  A Izu; H Yanagida; K Sugimoto; S Fujita; M Okada; T Takemura
Journal:  Clin Nephrol       Date:  2011-07       Impact factor: 0.975

5.  A patient with an interstitial deletion of the short arm of chromosome 6.

Authors:  E van Swaay; G C Beverstock; J J van de Kamp
Journal:  Clin Genet       Date:  1988-02       Impact factor: 4.438

6.  Deletions in chromosome 6p22.3-p24.3, including ATXN1, are associated with developmental delay and autism spectrum disorders.

Authors:  Patrícia Bs Celestino-Soper; Cindy Skinner; Richard Schroer; Patricia Eng; Jayant Shenai; Malgorzata Mj Nowaczyk; Deborah Terespolsky; Donna Cushing; Gayle S Patel; Ladonna Immken; Alecia Willis; Joanna Wiszniewska; Reuben Matalon; Jill A Rosenfeld; Roger E Stevenson; Sung-Hae L Kang; Sau Wai Cheung; Arthur L Beaudet; Pawel Stankiewicz
Journal:  Mol Cytogenet       Date:  2012-04-05       Impact factor: 2.009

7.  Terminal deletion of 6p: report of a new case.

Authors:  A Plaja; R Vidal; D Soriano; X Bou; T Vendrell; C Mediano; J M Pueyo; X Labraña; E Sarret
Journal:  Ann Genet       Date:  1994

8.  An interstitial deletion of 7.1Mb in chromosome band 6p22.3 associated with developmental delay and dysmorphic features including heart defects, short neck, and eye abnormalities.

Authors:  Anna Bremer; Jacqueline Schoumans; Magnus Nordenskjöld; Britt-Marie Anderlid; Maibritt Giacobini
Journal:  Eur J Med Genet       Date:  2009-07-01       Impact factor: 2.708

9.  Haploinsufficiency of two histone modifier genes on 6p22.3, ATXN1 and JARID2, is associated with intellectual disability.

Authors:  Tuva Barøy; Doriana Misceo; Petter Strømme; Asbjørg Stray-Pedersen; Asbjørn Holmgren; Olaug Kristin Rødningen; Anne Blomhoff; Johan Robert Helle; Alice Stormyr; Bjørn Tvedt; Madeleine Fannemel; Eirik Frengen
Journal:  Orphanet J Rare Dis       Date:  2013-01-07       Impact factor: 4.123

10.  6p22.3 deletion: report of a patient with autism, severe intellectual disability and electroencephalographic anomalies.

Authors:  Daniela Di Benedetto; Giuseppa Di Vita; Corrado Romano; Mariangela Lo Giudice; Girolamo Aurelio Vitello; Marinella Zingale; Lucia Grillo; Lucia Castiglia; Sebastiano Antonino Musumeci; Marco Fichera
Journal:  Mol Cytogenet       Date:  2013-01-17       Impact factor: 2.009

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