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美人鱼综合征:胚胎发生理论综述及与尾椎退化综合征差异的讨论

Sirenomelia: a review of embryogenic theories and discussion of the differences from caudal regression syndrome.

作者信息

Isik Kaygusuz Ecmel, Kurek Eken Meryem, Sivrikoz Oya Nermin, Cetiner Handan

机构信息

a Department of Pathology and.

b Department of Gynecology , Zeynep Kamil Maternity and Children's Training and Research Hospital , Istanbul , Turkey , and.

出版信息

J Matern Fetal Neonatal Med. 2016 Mar;29(6):949-53. doi: 10.3109/14767058.2015.1026254. Epub 2015 Sep 25.

Abstract

OBJECTIVE

To discuss the pathological features of sirenomelia in the light of our 10 cases and review the current theories.

METHODS

We identified 10 patients with sirenomelia from our hospital database. All clinical details and the autopsy features of 10 cases were noted.

RESULTS

Of the 10 children with sirenomelia seven had bilateral renal agenesis, three had bladder agenesis and one had a renal hypoplasia. Single umbilical artery was found in 60% of children with sirenomelia. External genitalia was ambiguous in seven of 10 patients.

CONCLUSIONS

Even though the etiology of caudal regression syndrome (CRS) and sirenomelia remains unknown we tend to believe that sirenomelia and CRS might be different entities.

摘要

目的

结合我们的10例病例探讨并腿畸形的病理特征,并回顾当前的理论。

方法

我们从医院数据库中识别出10例并腿畸形患者。记录了所有10例病例的临床细节和尸检特征。

结果

10例并腿畸形患儿中,7例双侧肾缺如,3例膀胱缺如,1例肾发育不全。60%的并腿畸形患儿发现单脐动脉。10例患者中有7例外生殖器模糊不清。

结论

尽管尾椎退化综合征(CRS)和并腿畸形的病因尚不清楚,但我们倾向于认为并腿畸形和CRS可能是不同的实体。

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