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超声表现及产前诊断完全 17p 三体综合征:病例报告及文献复习。

Ultrasonographic findings and prenatal diagnosis of complete trisomy 17p syndrome: A case report and review of the literature.

机构信息

Center for Reproductive Medicine and Center for Prenatal Diagnosis, First Hospital, Jilin University, Changchun, China.

出版信息

J Clin Lab Anal. 2021 Jan;35(1):e23582. doi: 10.1002/jcla.23582. Epub 2020 Sep 20.

Abstract

BACKGROUND

Trisomy of the short arm of chromosome 17 is a rare genomic disorder. The clinical features of complete trisomy 17p syndrome have been described. Most cases of this syndrome have been found in infants and children, but only a few cases were found by ultrasound in the prenatal period.

METHODS

We report a case of complete trisomy 17p syndrome, which was inherited from paternal balanced translocation t(15;17)(q11.2;q11.2). A pregnant woman underwent an ultrasound examination at 24 weeks of gestation. Amniotic fluid was collected by amniocentesis. Cytogenetic and single nucleotide polymorphism array analyses were performed. We further reviewed the relationship between duplication regions and the clinical phenotype.

RESULTS

Ultrasonographic evaluation showed intrauterine growth retardation and a right choroid plexus cyst, but the gallbladder was not observed. The fetal karyotype was 46,XX,der(17)t(15;17)(q11.2;q11.2)pat. The father's karyotype was 46,XY,t(15;17)(q11.2;q11.2). The single nucleotide polymorphism array results showed arr[GRCh37] 17p13.3q11.1(525-25309337)×3, which indicated a 25.309-Mb duplication.

CONCLUSION

Complete trisomy 17p syndrome shows severe malformations. Intrauterine growth retardation is the most typical manifestation of this syndrome as shown by ultrasonography in the second trimester of pregnancy. The genotype-phenotype relationships of complete trisomy 17p syndrome are not completely consistent. To further determine these relationships, additional cases are necessary to provide more information from ultrasonographic findings during pregnancy.

摘要

背景

17 号染色体短臂三体是一种罕见的基因组疾病。完全 17p 三体综合征的临床特征已有描述。这种综合征的大多数病例发生在婴儿和儿童中,但只有少数病例在产前通过超声发现。

方法

我们报告了一例完全 17p 三体综合征病例,该病例由父源性平衡易位 t(15;17)(q11.2;q11.2)遗传而来。一名孕妇在妊娠 24 周时接受了超声检查。通过羊膜穿刺术采集羊水。进行了细胞遗传学和单核苷酸多态性微阵列分析。我们进一步回顾了重复区域与临床表型之间的关系。

结果

超声评估显示宫内生长迟缓,右侧脉络丛囊肿,但未观察到胆囊。胎儿核型为 46,XX,der(17)t(15;17)(q11.2;q11.2)pat. 父亲的核型为 46,XY,t(15;17)(q11.2;q11.2)。单核苷酸多态性微阵列结果显示 arr[GRCh37]17p13.3q11.1(525-25309337)×3,提示存在 25.309-Mb 重复。

结论

完全 17p 三体综合征表现出严重的畸形。宫内生长迟缓是该综合征在妊娠中期超声检查中最典型的表现。完全 17p 三体综合征的基因型-表型关系并不完全一致。为了进一步确定这些关系,需要更多的病例提供来自妊娠期间超声检查的更多信息。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3798/7843288/ce91fab22bbc/JCLA-35-e23582-g001.jpg

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