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一名新生儿的室间隔纤维瘤和多囊性肾发育不良

Interventricular fibroma and cystic renal dysplasia in a newborn.

作者信息

Schwartz J, Saldivar V, Tio F, Kohler C

出版信息

Pediatr Pathol. 1984;2(2):187-95. doi: 10.3109/15513818409025886.

Abstract

A newborn twin presented with a cardiac tumor; at autopsy microcystic dysplasia was found in the kidneys. The histology of the renal cysts were not unlike those found in many multisystem syndromes (e.g., Schwartz-Jampel, Ehlers-Danlos, and Jeune's asphyxiating thoracic dystrophy). No evidence of tuberous sclerosis or other phakomatoses were found. This case may alert other observers to carefully look for cystic dysplasia of the kidneys or other organs in association with cardiac tumors.

摘要

一名新生儿双胞胎患有心脏肿瘤;尸检时发现肾脏有微囊性发育异常。肾囊肿的组织学表现与许多多系统综合征(如施瓦茨 - 扬佩尔综合征、埃勒斯 - 丹洛斯综合征和热内氏窒息性胸廓发育不良)中发现的情况并无不同。未发现结节性硬化症或其他错构瘤病的证据。该病例可能提醒其他观察者仔细寻找与心脏肿瘤相关的肾脏或其他器官的囊性发育异常。

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