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[两例携带Y染色体部分缺失胎儿的产前诊断]

[Prenatal diagnosis for two fetuses carrying partial deletion of Y chromosome].

作者信息

Pang Hong, Gao Ming, Hua Jun, Tong Dan, Zhao Yanhui, Feng Xiaojing

机构信息

Shenyang Women's and Children's Hospital, Shenyang, Liaoning 110001, China.

出版信息

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2020 Feb 10;37(2):182-185. doi: 10.3760/cma.j.issn.1003-9406.2020.02.021.

Abstract

OBJECTIVE

To perform prenatal diagosis for two fetuses carrying partial deletion of Y chromosome.

METHODS

Routine G- and C-banding were carried out to analyze the chromosomal karyotypes of the fetuses and their fathers. Fetal DNA was also subjected to low-coverage massively parallel copy number variation sequencing (CNV-seq), fluorescence in situ hybridization (FISH), SRY gene and AZF factor testing.

RESULTS

Both fetuses showed a 46, XN, del(Y) (q11.2) karyotype at 320-400 band level by the analysis of amniotic fluid chromosomes. FISH with Y chromosome centromere probe indicated that in both cases the number of Y chromosome was normal. Both fathers had an apparently normal karyotype at 320-400 band level. For fetus 1, CNV-seq test revealed a 12.88 Mb deletion at Yq11.221-q12, which encompassed the whole of AZFb+AZFc regions and may lead to male infertility, sperm deficiency and/or severe oligospermia. In fetus 2, CNV-seq also detected a 14.84 Mb deletion at Yq11.21-q12, which encompassed the whole of the AZF region and may lead to severe spermatogenesis disorder resulting in severe oligoasthenospermia and azoospermia. In both cases, testing of SRY gene was positive. No point mutation of the SRY gene was identified. Analysis of amniotic fluid DNA confirmed partial or total absence of AZF in the two fetuses, respectively.

CONCLUSION

Combined use of various technologies can enable accurate detection of structural abnormalities of the Y chromosome and facilitate genetic counseling. CNV-seq can help with rapid screening of Y chromosome microdeletions and may be used as a complementary test for chromosomal karyotyping.

摘要

目的

对两例携带Y染色体部分缺失的胎儿进行产前诊断。

方法

采用常规G显带和C显带技术分析胎儿及其父亲的染色体核型。对胎儿DNA进行低覆盖度大规模平行拷贝数变异测序(CNV-seq)、荧光原位杂交(FISH)、SRY基因及AZF因子检测。

结果

羊水染色体分析显示,两例胎儿在320-400条带水平均呈现46,XN,del(Y)(q11.2)核型。用Y染色体着丝粒探针进行FISH检测表明,两例胎儿Y染色体数目均正常。两位父亲在320-400条带水平核型均明显正常。对于胎儿1,CNV-seq检测显示Yq11.221-q12存在12.88 Mb的缺失,该缺失涵盖整个AZFb+AZFc区域,可能导致男性不育、精子缺乏和/或严重少精子症。对于胎儿2,CNV-seq也检测到Yq11.21-q12存在14.84 Mb的缺失,该缺失涵盖整个AZF区域,可能导致严重的生精障碍,从而导致严重的少弱畸精子症和无精子症。两例胎儿SRY基因检测均为阳性,未发现SRY基因点突变。羊水DNA分析证实两例胎儿分别部分或完全缺失AZF。

结论

多种技术联合应用可准确检测Y染色体结构异常,有助于遗传咨询。CNV-seq有助于快速筛查Y染色体微缺失,可作为染色体核型分析的补充检测手段。

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