Literature DB >> 28805612

Detection of paternal uniparental disomy 9 in a neonate with prenatally detected mosaicism for a small supernumerary marker chromosome 9 and a supernumerary ring chromosome 9.

Chih-Ping Chen1, Ming Chen2, Liang-Kai Wang3, Schu-Rern Chern4, Peih-Shan Wu5, Shin-Wen Chen3, Shih-Ting Lai3, Shun-Ping Chang6, Chien-Wen Yang4, Chen-Wen Pan3, Wayseen Wang7.   

Abstract

OBJECTIVE: We present the association of paternal uniparental disomy (UPD) 9 with mosaicism for a small supernumerary marker chromosome 9 [sSMC(9)] and a supernumerary ring chromosome 9 [r(9)].
MATERIALS AND METHODS: A 38-year-old woman underwent amniocentesis at 17 weeks of gestation because of advanced maternal age. Amniocentesis revealed a karyotype of 47,XY,+mar [25]/48,XY,+mar,+r(9) [4]/47,XY,+r(9) [1]/46,XY [6]. The parental karyotypes were normal. Array comparative genomic hybridization (aCGH) of cultured amniocytes revealed a result of de novo 9p13.1q21.11 (38,792,472-71,026,063) × 2.64. The marker chromosome was determined to be an sSMC(9) by spectral karyotyping and aCGH. A phenotypically normal baby was delivered at 38 weeks of gestation. During pediatric follow-ups at age two years, the neonate manifested normal psychomotor and growth development. Cytogenetic analysis, metaphase fluorescence in situ hybridization (FISH), single nucleotide polymorphism (SNP) aCGH and polymorphic DNA marker analysis were performed on the peripheral blood of the neonate.
RESULTS: The neonate's blood had the following results. Metaphase FISH confirmed coexistence of the sSMC(9) and the supernumerary r(9). The karyotype was 47,XY,+sSMC(9) [14]/48,XY, +sSMC(9),+r(9) [10]/47,XY,+r(9) [6]/46,XY [10]. SNP aCGH revealed arr 9p22.3q21.11 (14,234,165-71,035,608) × 2-3, arr 9p24.3p22.3 (216,123-14,629,321)hmz, arr 9p21.3p13.2 (24,769,722-36,732,597)hmz and arr 9q21.11q34.3 (71,013,799-141,011,581)hmz. Polymorphic DNA marker analysis showed paternal isodisomy 9.
CONCLUSION: Individuals with mosaicism for sSMC(9) and supernumerary r(9) may be associated with paternal UPD 9.
Copyright © 2017. Published by Elsevier B.V.

Entities:  

Keywords:  9p duplication syndrome; Paternal uniparental disomy 9; Small supernumerary marker chromosome 9; Supernumerary ring chromosome 9

Mesh:

Substances:

Year:  2017        PMID: 28805612     DOI: 10.1016/j.tjog.2017.06.002

Source DB:  PubMed          Journal:  Taiwan J Obstet Gynecol        ISSN: 1028-4559            Impact factor:   1.705


  2 in total

1.  A case report and mechanism analysis of a normal phenotype mosaic 47, XXY complicated by paternal iUPD (9) who had a normal PGD result.

Authors:  Dan Li; Yun Wang; Nan Zhao; Liang Chang; Ping Liu; Chan Tian; Jie Qiao
Journal:  BMC Med Genet       Date:  2019-11-07       Impact factor: 2.103

2.  Uniparental disomy as a cause of pediatric endocrine disorders.

Authors:  Keiko Matsubara; Masayo Kagami; Maki Fukami
Journal:  Clin Pediatr Endocrinol       Date:  2018-07-31
  2 in total

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