Meng Fanrong, Ju Duan, Wang Xiuyan, Shi Yunfang, Yang Meng, Li Xiaozhou
Tianjin Key Laboratory for Female Reproductive Health and Birth Health, Department of Gynecology and Obstetrics, Tianjin Medical University General Hospital, Tianjin 300052, China.
Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2023 Nov 10;40(11):1414-1419. doi: 10.3760/cma.j.cn511374-20221202-00830.
To carry out prenatal diagnosis for a fetus with mosaicism Yq deletion.
A fetus with high risk of sex chromosomes indicated by non-invasive prenatal testing (NIPT) at Tianjin Medical University General Hospital in July 2021 was selected as the study subject. Prenatal diagnosis of the fetus was performed with combined G-banded chromosomal karyotyping, fluorescence in situ hybridization (FISH), copy number variation sequencing (CNV-seq), real-time fluorescence PCR (QF-PCR), and ultrasound examination.
Analysis of the amniocytes at 23 gestational weeks had yielded a 45,X karyotype. However, FISH had shown signals of Y chromosome. Re-examination by cordocentesis had shown a mosaicism of 46,X,+mar[33]/45,X[17]. FISH showed that 69% of the cells had contained Y chromosome signals. The result of CNV-seq was seq[19]del(Y)(q11.1q12)(mos) chrY: g.13200001_ 28820000del (mosaicism rate = 64%), which suggested mosaicism for a Yq deletion, which encompassed the azoospermia factor (AZF) region. Deletion of the AZF region was verified by QF-PCR. The fetal karyotype was ultimately determined as mos46,X,del(Y)(q11.1)[33]/45,X[17]. Although ultrasound examination had shown no abnormality in the fetus, the couple had opted to terminate the pregnancy, and the induced fetus had a normal male appearance.
The combined use of multiple techniques is beneficial for accurate and rapid prenatal diagnosis. For fetuses with mosaicism chromosomal abnormalities, it may be difficult to accurately predict the postnatal phenotype. It is therefore necessary to further explore their genotype-phenotype correlation in order to provide better guidance upon genetic counseling.
对一名Yq缺失嵌合体胎儿进行产前诊断。
选取2021年7月在天津医科大学总医院经无创产前检测(NIPT)提示性染色体高风险的一名胎儿作为研究对象。采用G显带染色体核型分析、荧光原位杂交(FISH)、拷贝数变异测序(CNV-seq)、实时荧光定量PCR(QF-PCR)及超声检查对该胎儿进行产前诊断。
孕23周羊水细胞分析显示核型为45,X。然而,FISH显示有Y染色体信号。经脐血穿刺复查显示为46,X,+mar[33]/45,X[17]嵌合体。FISH显示69%的细胞含有Y染色体信号。CNV-seq结果为seq[19]del(Y)(q11.1q12)(mos) chrY: g.13200001_28820000del(嵌合率 = 64%),提示存在Yq缺失嵌合体,该缺失包含无精子症因子(AZF)区域。通过QF-PCR验证了AZF区域的缺失。胎儿最终核型确定为mos46,X,del(Y)(q11.1)[33]/45,X[17]。尽管超声检查显示胎儿无异常,但这对夫妇选择终止妊娠,引产胎儿外观为正常男性。
多种技术联合应用有利于准确、快速地进行产前诊断。对于染色体异常嵌合体胎儿,可能难以准确预测其出生后的表型。因此,有必要进一步探索其基因型与表型的相关性,以便在遗传咨询时提供更好的指导。