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胎儿中具有不平衡t(X;9)易位的新发衍生染色体X的分子细胞遗传学特征及文献综述

Molecular cytogenetic characterization of a de novo derivative chromosome X with an unbalanced t(X;9) translocation in a fetus and literature review.

作者信息

Wu Qiong, Kong Hui, Shen Yanyan, Chen Jing

机构信息

Department of Central Laboratory, Women and Children's Hospital, School of Medicine, Xiamen University, Xiamen, 361003, Fujian, China.

Department of Child Health, Women and Children's Hospital, School of Medicine, Xiamen University, Xiamen, 361003, Fujian, China.

出版信息

Mol Cytogenet. 2022 Jun 27;15(1):24. doi: 10.1186/s13039-022-00603-3.

Abstract

Partial trisomy 9p is one of the most frequent autosome anomalies in newborn infants featured by craniofacial dysmorphism, intellectual disability and psychomotor growth. Female patients carrying monosomy Xq usually show mild symptoms due to skewed X-chromosome inactivation (XCI). Unbalanced translocation between chromosome X and chromosome 9 is rare in prenatal diagnosis. The skewed inactivation of abnormal X would spread into the extra segment of chromosome 9 presented in the der(X) leading to mild phenotypes. We reported on a fetus with high risk of trisomy 9p(13.32 Mb 9p23-p24.3 duplication)suggested by noninvasive prenatal testing (NIPT), the fetus was normal by ultrasonography. G-banding with trypsin-giemsa (GTG), copy number variations sequencing (CNV-seq) and fluorescence in situ hybridization (FISH) were carried out to delineate the nature of rearrangement. Final karyotype of the fetus was identified as 46,X,der(X)t(X;9)(q27;p23)dn. An unbalanced X-autosome translocation with a deletion of Xqter-q27.2 and a duplication of 9pter-p23 led to mild phenotypes with no obvious alteration by prenatal ultrasonography, or obvious pathological alterations after pregnancy termination.

摘要

9p部分三体是新生儿中最常见的常染色体异常之一,其特征为颅面畸形、智力残疾和精神运动发育迟缓。携带Xq单体的女性患者通常由于X染色体失活(XCI)偏斜而表现出轻微症状。X染色体与9号染色体之间的不平衡易位在产前诊断中较为罕见。异常X的失活偏斜会扩散到der(X)中出现的9号染色体额外片段,导致表型轻微。我们报告了一例经无创产前检测(NIPT)提示9p三体风险高(9p23-p24.3重复13.32 Mb)的胎儿,超声检查显示该胎儿正常。采用胰蛋白酶-吉姆萨(GTG)显带、拷贝数变异测序(CNV-seq)和荧光原位杂交(FISH)来确定重排的性质。该胎儿的最终核型被鉴定为46,X,der(X)t(X;9)(q27;p23)dn。X-常染色体不平衡易位,伴有Xqter-q27.2缺失和9pter-p23重复,导致表型轻微,产前超声检查无明显改变,终止妊娠后也无明显病理改变。

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