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法因-卢宾斯基综合征的胎儿表现。短头畸形、耳聋、白内障、小口畸形和智力发育迟缓综合征,并发皮埃尔-罗宾异常和羊水过多。

Fetal manifestation of the Fine-Lubinsky syndrome. Brachycephaly, deafness, cataract, microstomia and mental retardation syndrome complicated by Pierre-Robin anomaly and polyhydramnios.

作者信息

Schoner Katharina, Bald Rainer, Fritz Barbara, Rehder Helga

机构信息

Institute of Pathology, Philipps University Marburg, Marburg, Germany.

出版信息

Fetal Diagn Ther. 2008;23(3):228-32. doi: 10.1159/000116746. Epub 2008 Feb 20.

Abstract

OBJECTIVES

We report on a female fetus of 24 weeks gestational age with Fine-Lubinsky syndrome (FLS), representing the 7th case published so far.

METHODS

Prenatal ultrasound was performed at 22+1 weeks of gestation and thorough postmortem examination was made after termination of pregnancy.

RESULTS

The diagnosis of FLS in the fetus was based on characteristic features that were already apparent in early prenatal life, such as growth deficiency, brachycephaly, flat face with associated dysmorphic signs, microstomia and cataract, while deafness and mental retardation, which are syndrome-specific functional disorders and evident only postnatally, could not be taken into account.

CONCLUSIONS

This case demonstrates the diagnostic problems in fetal syndromology if syndrome-specific features are not yet recognizable and additional complications occur that had not been observed in this disorder.

摘要

目的

我们报告一例孕24周的患有芬-卢宾斯基综合征(FLS)的女性胎儿,这是迄今为止已发表的第7例病例。

方法

在妊娠22 + 1周时进行了产前超声检查,并在终止妊娠后进行了全面的尸检。

结果

胎儿FLS的诊断基于产前早期就已明显的特征,如生长发育迟缓、短头畸形、伴有相关畸形体征的扁平脸、小口畸形和白内障,而耳聋和智力迟钝是该综合征特有的功能障碍,仅在出生后才明显,故未予以考虑。

结论

该病例表明,如果尚未识别出综合征特异性特征且出现了该疾病中未观察到的其他并发症,胎儿综合征学的诊断会存在问题。

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