Literature DB >> 34602957

Deletion of 16q22.2q23.3 in a Boy with a Phenotype Reminiscent of Silver-Russell Syndrome.

Anna Lengyel1, Éva Pinti1, Thomas Eggermann2, György Fekete1, Irén Haltrich1.   

Abstract

A 15-month-old boy presented with growth and global developmental delay, feeding difficulties, sleep disturbance and several minor anomalies, including a large anterior fontanel, relative macrocephaly, and a triangular face. Clinical suspicion prompted genetic investigations for Silver-Russell syndrome and related disorders. SNP array analysis led to the diagnosis of an approximately 10-Mb large deletion of the long arm in chromosome 16q22.2q23.3. Interstitial deletions of 16q show a wide variability of related features; however, considering the differences in size and location of the deletions in the known patients, the phenotypic overlap is surprising. Here, we report a novel microdeletion, compare the proband with data from scientific literature and international databases, and discuss possible diagnostic implications.
Copyright © 2021 by S. Karger AG, Basel.

Entities:  

Keywords:  Chromosome 16q; Copy number variation; Deletion; Growth delay; Neurodevelopmental disorders

Year:  2021        PMID: 34602957      PMCID: PMC8436670          DOI: 10.1159/000515941

Source DB:  PubMed          Journal:  Mol Syndromol        ISSN: 1661-8769


  13 in total

Review 1.  Deletion of 16q with prolonged survival and unusual radiographic manifestations.

Authors:  A C Casamassima; R M Klein; P L Wilmot; P Brenholz; L R Shapiro
Journal:  Am J Med Genet       Date:  1990-12

2.  A small deletion of 16q23.1-->16q24.2 [del(16)(q23.1q24.2).ish del(16)(q23.1q24.2)(D16S395+, D16S348-, P5432+)] in a boy with iris coloboma and minor anomalies.

Authors:  W Werner; S Kraft; D F Callen; O Bartsch; G K Hinkel
Journal:  Am J Med Genet       Date:  1997-06-27

3.  Interstitial deletion for a region in the long arm of chromosome 16.

Authors:  C C Lin; R B Lowry; F F Snyder
Journal:  Hum Genet       Date:  1983       Impact factor: 4.132

4.  A 16q22.2-q23.1 deletion identified in a male infant with West syndrome.

Authors:  Tatsuo Mori; Aya Goji; Yoshihiro Toda; Hiromichi Ito; Kenji Mori; Tomohiro Kohmoto; Issei Imoto; Shoji Kagami
Journal:  Brain Dev       Date:  2019-07-25       Impact factor: 1.961

5.  Interstitial deletion of chromosome 16q: 16q22 is critical for 16q- syndrome.

Authors:  M Fujiwara; T Yoshimoto; Y Morita; M Kamada
Journal:  Am J Med Genet       Date:  1992-06-01

6.  High resolution mapping of interstitial long arm deletions of chromosome 16: relationship to phenotype.

Authors:  D F Callen; H Eyre; S Lane; Y Shen; I Hansmann; N Spinner; E Zackai; D McDonald-McGinn; S Schuffenhauer; J Wauters
Journal:  J Med Genet       Date:  1993-10       Impact factor: 6.318

7.  Multiple congenital anomalies syndrome with myopathy in chromosome 16 abnormality.

Authors:  V Ionasescu; S Patil; M Hart; W Rhead; W Smith
Journal:  Am J Med Genet       Date:  1987-01

8.  DECIPHER: Database of Chromosomal Imbalance and Phenotype in Humans Using Ensembl Resources.

Authors:  Helen V Firth; Shola M Richards; A Paul Bevan; Stephen Clayton; Manuel Corpas; Diana Rajan; Steven Van Vooren; Yves Moreau; Roger M Pettett; Nigel P Carter
Journal:  Am J Hum Genet       Date:  2009-04-02       Impact factor: 11.025

9.  Further delineation of Aymé-Gripp syndrome and use of automated facial analysis tool.

Authors:  Shivarajan M Amudhavalli; Randi Hanson; Brad Angle; Kelly Bontempo; Karen W Gripp
Journal:  Am J Med Genet A       Date:  2018-07       Impact factor: 2.802

10.  Mutations Impairing GSK3-Mediated MAF Phosphorylation Cause Cataract, Deafness, Intellectual Disability, Seizures, and a Down Syndrome-like Facies.

Authors:  Marcello Niceta; Emilia Stellacci; Karen W Gripp; Giuseppe Zampino; Maria Kousi; Massimiliano Anselmi; Alice Traversa; Andrea Ciolfi; Deborah Stabley; Alessandro Bruselles; Viviana Caputo; Serena Cecchetti; Sabrina Prudente; Maria T Fiorenza; Carla Boitani; Nicole Philip; Dmitriy Niyazov; Chiara Leoni; Takaya Nakane; Kim Keppler-Noreuil; Stephen R Braddock; Gabriele Gillessen-Kaesbach; Antonio Palleschi; Philippe M Campeau; Brendan H L Lee; Celio Pouponnot; Lorenzo Stella; Gianfranco Bocchinfuso; Nicholas Katsanis; Katia Sol-Church; Marco Tartaglia
Journal:  Am J Hum Genet       Date:  2015-04-09       Impact factor: 11.025

View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.