Nicolaides K H, Snijders R J, Gosden C M, Berry C, Campbell S
Harris Birthright Research Centre for Fetal Medicine, King's College Hospital, Denmark Hill, London, UK.
Lancet. 1992 Sep 19;340(8821):704-7. doi: 10.1016/0140-6736(92)92240-g.
Screening for fetal chromosomal abnormalities on the basis of maternal age has not resulted in a substantial fall in the proportion of infants born with an abnormal karyotype. Most fetuses with major chromosomal abnormalities have defects that can be recognised on detailed ultrasonographic examination. Therefore, provided the cardinal signs of each chromosomal syndrome are recognised, it is possible that screening by ultrasound examination could have a greater impact. We karyotyped 2086 fetuses after ultrasonographic examination had revealed fetal malformations, growth retardation, or both. Chromosomal abnormalities were detected in 301 (14%) cases and were more common among fetuses with multisystem malformations (29%) than among those with isolated defects (2%). The commonest chromosomal abnormality was trisomy 18, followed by trisomy 21, triploidy, Turner's syndrome, unbalanced chromosomal rearrangements, and trisomy 13. Trisomy 18 was associated with strawberry-shaped head, choroid plexus cysts, facial cleft, micrognathia, heart defects, exomphalos, malformations of hands and feet, and growth retardation. In trisomy 21, the associated defects were subtle and included nuchal oedema, macroglossia, atrioventricular septal defects, mild hydronephrosis, clinodactyly, and sandal gap. The frequency of autosomal abnormalities increased with maternal age, but if fetal karyotyping had been restricted to mothers older than 35 years, large proportions of chromosomally abnormal fetuses would not have been diagnosed prenatally (64-97%). Our findings provide guidelines as to which defects to search for in screening studies for the detection of chromosomal abnormalities.
基于孕妇年龄对胎儿染色体异常进行筛查,并未使染色体核型异常婴儿的出生比例大幅下降。大多数患有主要染色体异常的胎儿具有可在详细超声检查中识别的缺陷。因此,只要识别出每种染色体综合征的主要体征,超声检查筛查就有可能产生更大影响。在超声检查发现胎儿畸形、生长迟缓或两者皆有时,我们对2086例胎儿进行了染色体核型分析。在301例(14%)病例中检测到染色体异常,在患有多系统畸形的胎儿中(29%)比在患有孤立缺陷的胎儿中(2%)更为常见。最常见的染色体异常是18三体,其次是21三体、三倍体、特纳综合征、染色体不平衡重排和13三体。18三体与草莓状头、脉络丛囊肿、面部裂隙、小颌畸形、心脏缺陷、脐膨出、手足畸形和生长迟缓有关。在21三体中,相关缺陷较为隐匿,包括颈部水肿、巨舌症、房室间隔缺损、轻度肾积水、小指内弯和草鞋足间隙。常染色体异常的发生率随孕妇年龄增加而升高,但如果胎儿染色体核型分析仅限于35岁以上的母亲,很大比例的染色体异常胎儿将无法在产前被诊断出来(64 - 97%)。我们的研究结果为在检测染色体异常的筛查研究中寻找哪些缺陷提供了指导方针。