肾、肝和胰腺发育异常：Ivemark综合征一种变异型的报告。

Dysplasia of the kidneys, liver, and pancreas: report of a variant of Ivemark's syndrome.

作者信息

Strayer D S, Kissane J M

出版信息

Hum Pathol. 1979 Mar;10(2):228-34. doi: 10.1016/s0046-8177(79)80011-8.

Abstract

A newborn girl with respiratory distress due to bilateral pneumothorax was found to be anuric, and died at 18 hours of age. Autopsy revealed a large pancreatic cyst, multiple large hepatic cysts, congenital hepatic fibrosis, bilateral dysplastic kidneys, and dysplasia of the pancreas. These findings constitute a variant of Ivemark's syndrome of dysplasia of the pancreas, liver, and kidneys.

摘要

一名因双侧气胸导致呼吸窘迫的新生女婴被发现无尿，并于18小时龄时死亡。尸检显示有一个大的胰腺囊肿、多个大的肝囊肿、先天性肝纤维化、双侧发育不良的肾脏以及胰腺发育异常。这些发现构成了胰腺、肝脏和肾脏发育异常的伊韦马克综合征的一种变体。