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单绒毛膜、单羊膜囊双胎中双侧肾缺如-发育不全(围产期致死性肾病)不一致的产前和产后发现。

Prenatal and postnatal findings in monochorionic, monoamniotic twins discordant for bilateral renal agenesis-dysgenesis (perinatal lethal renal disease).

作者信息

Cilento B G, Benacerraf B R, Mandell J

机构信息

Department of Surgery, Children's Hospital, Boston, Massachusetts.

出版信息

J Urol. 1994 Apr;151(4):1034-5. doi: 10.1016/s0022-5347(17)35169-8.

DOI:10.1016/s0022-5347(17)35169-8
PMID:8126783
Abstract

We report on a male twin born with no functional renal tissue and without the extrarenal manifestations of Potter facies, skin changes, club feet and pulmonary hypoplasia. The monoamniotic co-twin had normal renal function, thereby maintaining sufficient amniotic fluid to avoid the classic presentation of Potter's syndrome. Prenatal diagnosis of this condition allowed prompt confirmation of these findings and support for the parents without unnecessary intervention. This experiment of nature demonstrates the necessity of normal renal function in the maintenance of amniotic fluid and its relationship to the proper development of the pulmonary and integumentary systems.

摘要

我们报告了一名男性双胞胎,其出生时没有功能性肾组织,也没有波特面容、皮肤改变、马蹄内翻足和肺发育不全等肾外表现。单羊膜囊的双胎具有正常肾功能,从而维持了足够的羊水,避免了波特综合征的典型表现。对这种情况的产前诊断能够迅速证实这些发现,并在不进行不必要干预的情况下为父母提供支持。这种自然实验证明了正常肾功能在维持羊水量及其与肺和皮肤系统正常发育关系中的必要性。

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