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遗传性疾病的产前诊断。

Prenatal diagnosis of genetic disorders.

作者信息

Niermeijer M F, Sachs E S, Jahodova M, Tichelaar-Klepper C, Kleijer W J, Galjaard H

出版信息

J Med Genet. 1976 Jun;13(3):182-94. doi: 10.1136/jmg.13.3.182.

Abstract

Three hundred and fifty pregnancies were monitored by transabdominal amniocentesis in the fourteenth to sixteenth week of gestation followed by karyotyping or biochemica assays of cultured amniotic fluid cells and analysis of alpha-fetoprotein in the amniotic fluid supernatant. The pregnancy was interrupted in 36 cases (10%) either becasue of a fetal abnormality or the presence of a male fetus in pregnancies at risk for an X-linked disease. Four chromosomal aberrations were found in 87 pregnancies tested because of advanced maternal age. In 101 pregnancies with a recurrence risk of Down's syndrome, 2 fetuses with an abnormal karyotype were detected. In 11 cases, in which 1 parent was a carrier of a balanced translocation, 2 unbalanced fetal karyotypes were found. Fetal chromosome studies in 43 pregancies at risk for an X-linked disease indicated the presence of a male fetus in 21 cases. Prenatal diagnosis of 11 different metabolic diseases was performed in a total of 34 cases. Microchemical techniques were used to allow completion of the diagnosis of seven different enzyme deficiencies within 9 to 22 days after amniocentesis. Alpha-fetoprotein assay in the amniotic fluid supernatant of 47 pregnancies at risk for an open neural tube defect resulted in the detection of 3 anencephalic fetuses during the second half of pregnancy. The safety and reliability of amniocentesis and the possible effects on the outcome of pregnancy are evaluated. Prenatal diagnosis offers a promising alternative for parents who are at risk of having a child with a genetic disease which can be detected in amniotic fluid or in cultured amniotic fluid cells.

摘要

在妊娠14至16周时,通过经腹羊膜腔穿刺术对350例妊娠进行监测,随后对培养的羊水细胞进行核型分析或生化检测,并对羊水上清液中的甲胎蛋白进行分析。36例(10%)妊娠因胎儿异常或X连锁疾病高危妊娠中存在男性胎儿而终止。在因母亲年龄偏大而接受检测的87例妊娠中发现了4例染色体畸变。在101例有唐氏综合征复发风险的妊娠中,检测到2例核型异常的胎儿。在11例父母一方为平衡易位携带者的病例中,发现2例胎儿核型不平衡。对43例X连锁疾病高危妊娠进行胎儿染色体研究,结果显示21例存在男性胎儿。共对34例进行了11种不同代谢疾病的产前诊断。采用微量化学技术,在羊膜腔穿刺术后9至22天内完成了7种不同酶缺乏症的诊断。对47例开放性神经管缺陷高危妊娠的羊水上清液进行甲胎蛋白检测,结果在妊娠后半期检测到3例无脑儿。对羊膜腔穿刺术的安全性和可靠性以及对妊娠结局可能产生的影响进行了评估。产前诊断为有生育患可在羊水或培养的羊水细胞中检测到的遗传病患儿风险的父母提供了一个有前景的选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c3de/1013391/d3ece05746e0/jmedgene00310-0017-a.jpg

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