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Journal of Pediatric Genetics logoLink to Journal of Pediatric Genetics
. 2012 Jun;1(2):115–124. doi: 10.3233/PGE-2012-019

Inherited and de novo 22q11.2 distal duplications in two patients with autistic features, speech delay and no dysmorphology

Feras M Hantash a,*, Boris T Wang a, Renius Owen a, Leslie P Ross b, Loretta W Mahon c, Fatih Z Boyar a, Arturo Anguiano a, Charles M Strom a
PMCID: PMC5020930  PMID: 27625811

Abstract

In a screen of patients by fluorescence in-situ hybridization and array comparative genomic hybridization in the past two years (July 2007--July 2009), we identified two patients with duplications in the 22q11.22-23, occurring outside the common DiGeorge syndrome/valocardiofacial syndrome region. Fluorescent in-situ hybridization, multiplex ligation-dependent probe amplification and high density bacterial artificial chromosomes and oligo arrays were used to identify the extent of the duplications. In one patient the duplication extended from LCR22-E/5 to LCR22-H/8, which is similar to recently described 22q11.2 distal duplications, while in the second patient, a de novo duplication was identified extending between LCR22-E/5 to LCR22-F/6. The second proband also harbored a de novo 15q14 duplication, complicating phenotype interpretation. The patients were affected with speech delay and autistic features, but neither reported cardiac concern or dysmorphic features.

Keywords: 22q11.2, duplication, mental retardation, autism, tic behavior, speech, DiGeorge syndrome/valocardiofacial syndrome

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Footnotes

**

These authors contributed equally to this work and should be considered first co-authors.


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