Yue Fagui, Jiang Yuting, Pan Yuan, Li Leilei, Li Linlin, Liu Ruizhi, Wang Ruixue
Center for Reproductive Medicine and Center for Prenatal Diagnosis, The First Hospital, Jilin University, Changchun, Jilin 130021, P.R. China.
Jilin Engineering Research Center for Reproductive Medicine and Genetics, Jilin University, Changchun, Jilin 130021, P.R. China.
Exp Ther Med. 2019 Aug;18(2):1267-1275. doi: 10.3892/etm.2019.7695. Epub 2019 Jun 20.
Trisomy 16q is a rare disorder with severe abnormalities, which always leads to early postnatal mortality. It usually results from a parental translocation, exhibiting 16q duplication associated with another chromosomal deletion. The present study reports on the clinical presentation and molecular cytogenetic results of a small-for-gestational-age infant, consisting of partial trisomy 16q21→qter and monosomy 2p25.3→pter. The proband presented with moderately low birthweight, small anterior fontanelles, prominent forehead, low hairline, telecanthus, flat nasal bridge, choanal atresia, clinodactyly of the fifth fingers, urogenital anomalies, congenital muscular torticollis and congenital laryngomalacia. The last two traits have not previously been reported in any trisomy 16q and monosomy 2p cases. The proband was trisomic for the 16q21→qter chromosomal region with the karyotype 46,XY,der(2)t(2;16)(p25;q21)pat. The chromosomal anomaly was the result of unbalanced segregation of a paternal balanced translocation, 46,XY,t(2;16)(p25;q21). In this case, molecular cytogenetic analysis had a critical role in delineating the proband's clinical phenotype. Although this patient had a 16q21→qter duplication and a 2p25.3→pter deletion, the latter may have had mild phenotypic effects when associated with trisomy 16q. The literature was also reviewed, focusing on cases with the same breakpoints, localizations and clinical features reported in recent years.
16号染色体长臂三体是一种罕见的伴有严重异常的疾病,常导致出生后早期死亡。它通常源于父母一方的染色体易位,表现为16号染色体长臂重复并伴有另一条染色体缺失。本研究报告了一名小于胎龄儿的临床表现及分子细胞遗传学结果,该患儿存在16号染色体长臂21区带至末端部分三体及2号染色体短臂25.3区带至末端单体。先证者表现为出生体重中度偏低、前囟小、前额突出、发际线低、内眦距增宽、鼻梁扁平、后鼻孔闭锁、第五指屈曲指、泌尿生殖系统异常、先天性肌性斜颈和先天性喉软化。后两种特征此前在任何16号染色体长臂三体和2号染色体短臂单体病例中均未被报道。先证者16号染色体长臂21区带至末端染色体区域三体,核型为46,XY,der(2)t(2;16)(p25;q21)pat。该染色体异常是父亲平衡易位46,XY,t(2;16)(p25;q21)不平衡分离的结果。在本病例中,分子细胞遗传学分析在明确先证者临床表型方面起了关键作用。尽管该患者存在16号染色体长臂21区带至末端重复及2号染色体短臂25.3区带至末端缺失,但后者与16号染色体长臂三体相关时可能具有轻微的表型效应。本文还对近年来报道的具有相同断点、定位及临床特征的病例进行了文献综述。