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双侧肾缺如中的先天性囊性腺瘤样畸形。其对波特综合征的缓解作用。

Congenital cystic adenomatoid malformation in bilateral renal agenesis. Its mitigation of Potter's syndrome.

作者信息

Krous H F, Harper P E, Perlman M

出版信息

Arch Pathol Lab Med. 1980 Jul;104(7):368-70.

PMID:6893124
Abstract

Potter's syndrome develops secondary to a deficiency of amniotic fluid, such as occurs in renal agenesis. Congenital cystic adenomatoid malformation (CCAM), on the other hand, is frequently accompanied by polyhydramnios. We describe a newborn with both renal agenesis and CCAM who had only mild features of Potter's syndrome. The pathogenesis of polyhydramnios in CCAM is discussed with regard to the ultrastructural findings of numerous type 2 pneumocytes lining the cysts. The association between CCAM and bilateral renal anomalies is emphasized.

摘要

波特综合征继发于羊水过少,如肾缺如时发生的情况。另一方面,先天性囊性腺瘤样畸形(CCAM)常伴有羊水过多。我们描述了一名患有肾缺如和CCAM的新生儿,其仅具有波特综合征的轻微特征。根据囊肿内衬大量2型肺细胞的超微结构发现,讨论了CCAM中羊水过多的发病机制。强调了CCAM与双侧肾脏异常之间的关联。

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