Literature DB >> 30933946

Mosaic Trisomy 12 Associated with Overgrowth Detected in Fibroblast Cell Lines.

Yanca Gasparini, Marília M Montenegro, Gil M Novo-Filho, José R M Ceroni, Rachel S Honjo, Évelin A Zanardo, Alexandre T Dias, Amon M Nascimento, Taís V M M Costa, Fabrícia A Madia, Samar N Chehimi, Jullian G Damasceno, Chong A Kim, Leslie D Kulikowski.   

Abstract

Mosaic trisomy 12 is a rare anomaly, and only 9 cases of live births with this condition have been reported in the literature. The clinical phenotype is variable, including neuropsychomotor developmental delay, congenital heart disease, microcephaly, cutaneous spots, facial asymmetry, prominent ears, hypotonia, retinopathy, and sensorineural hearing loss. A 2-year-old female presented with neuropsychomotor developmental delay, prominent forehead, dolichocephaly, patchy skin pigmentation, and unexpected overgrowth at birth. Cytogenetic analysis of her peripheral blood showed normal results, suggesting the presence of a chromosomal alteration in other tissues. Further studies using G-banding and FISH performed on fibroblasts from both hyper- and hypopigmented regions identified a 47,XX,+12/46,XX karyotype. To the best of our knowledge, no patients with mosaic trisomy 12 associated with overgrowth have been reported to date. Congenital overgrowth and neonatal overgrowth have been frequently linked to Pallister-Killian syndrome (PKS; OMIM 601803). This case suggests the possibility of an association of genes present in the 12p region with fetal overgrowth, considering that chromosomal duplications could lead to an increase in the production of aberrant transcripts and disturbing gene dosage effects. This case highlights the importance of cytogenetic analysis in different tissues to provide relevant information to the specific genotype/phenotype correlation.
© 2019 S. Karger AG, Basel.

Entities:  

Keywords:  Cutaneous mosaicism; Cytogenetics; FISH; Fibroblasts; Genotype-phenotype correlation; Trisomy 12

Mesh:

Year:  2019        PMID: 30933946     DOI: 10.1159/000498836

Source DB:  PubMed          Journal:  Cytogenet Genome Res        ISSN: 1424-8581            Impact factor:   1.636


  3 in total

1.  Long-term Culture of EBV-induced Human Lymphoblastoid Cell Lines Reveals Chromosomal Instability.

Authors:  Marianne Volleth; Martin Zenker; Ivana Joksic; Thomas Liehr
Journal:  J Histochem Cytochem       Date:  2020-02-28       Impact factor: 2.479

2.  Cellular consequences of small supernumerary marker chromosome derived from chromosome 12: mosaicism in daughter and father.

Authors:  M O Freitas; A O Dos Santos; L S Barbosa; A F de Figueiredo; S P Pellegrini; N C K Santos; I S Paiva; A Rangel-Pozzo; L Sisdelli; S Mai; M G P Land; M G Ribeiro; M C M Ribeiro
Journal:  Braz J Med Biol Res       Date:  2022-06-22       Impact factor: 2.904

Review 3.  Mosaic trisomy 12 diagnosed in a female patient: clinical features, genetic analysis, and review of the literature.

Authors:  Daniela Hainz; Marcus Krüger; Daniela Reber; Karl Mehnert; Theresa Brunet; Gabriele Lederer; Sabine Langer-Freitag; Julia Hoefele
Journal:  World J Pediatr       Date:  2021-07-14       Impact factor: 2.764

  3 in total

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