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Weill-Marchesani综合征中球形晶状体的组织病理学研究。

Histopathological study of microspherophakia in the Weill-Marchesani syndrome.

作者信息

Nagata M, Takagi S, Yamasaki A, Tsunematsu S, Kumagami T, Itamochi C, Tamai A

机构信息

Department of Ophthalmology, Faculty of Medicine, Tottori University, Japan.

出版信息

Jpn J Ophthalmol. 1995;39(1):89-95.

PMID:7643490
Abstract

A surgically obtained lens from a 66-year-old man with the Weill-Marchesani syndrome was examined histopathologically by light microscopy, transmission electron microscopy and scanning electron microscopy. The lens was diffusely opaque and brown. It was microspherophakic in appearance, 6.0 mm in equatorial diameter and 4.8 mm in anteroposterior diameter. The lens fibers were well preserved, and ran circularly in the cortex and elliptically in the fetal nucleus. The lens fibers had undergone hyaloid degeneration in the area from the deep cortex to the superficial portion of the adult nucleus. Hyaloid degeneration also extended from the anterior to the posterior pole and was more marked in the equator. The distribution of lens fibers suggested that microspherophakia had developed postnatally. It is surmised that later changes in the shape of the lens affected the lens fibers, inducing hyaloid degeneration.

摘要

对一名患有Weill-Marchesani综合征的66岁男性手术摘除的晶状体进行了组织病理学检查,采用了光学显微镜、透射电子显微镜和扫描电子显微镜。该晶状体弥漫性混浊且呈褐色。外观上为小球形晶状体,赤道直径6.0毫米,前后径4.8毫米。晶状体纤维保存良好,在皮质呈环形排列,在胎儿核呈椭圆形排列。晶状体纤维在从深层皮质到成人核浅层的区域发生了玻璃体样变性。玻璃体样变性也从前极延伸至后极,在赤道处更为明显。晶状体纤维的分布表明小球形晶状体是在出生后形成的。据推测,晶状体形状的后期变化影响了晶状体纤维,导致了玻璃体样变性。

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