I型角膜前弹力层营养不良的组织病理学与超微结构研究

Histopathologic and Ultrastructure Changes of Corneal Dystrophy of Bowman Layer, Type I

目的:探讨Ⅰ型角膜前弹力层营养不良(cornealdystrophyofBowmanlayer,CDBI)的组织病理学和超微结构改变。方法:取2家系中4例CDBI患者板层或穿透性角膜移植术后的病变角膜,部分标本HE及特殊染色后行光学显微镜观察,部分标本做超薄切片,透射电子显微镜观察超微结构。以2例正常角膜组织标本为对照。结果:CDBI患者角膜上皮细胞微绒毛减少或消失,基底细胞间有小块高电子密度物质,基底细胞出现凋亡现象,前弹力层及前部基质中可见大量棒状高电子密度沉淀物。结论:CDBI患者角膜有特征性的结构改变,可能是造成其临床症状的原因之一。

Purpose:To explore the histopathologic and ultrastructure changes of corneal dystrophies of bowman layer,type Ⅰ (CDBⅠ) in Chinese. Methods:Cornea buttons were obtained from 4 CDB I patients in 2 pedigrees who underwent lamella or penetrating keratoplasty. Sections with HE and special staining were observed under light microscope. Ultrathin sections were performed and ultrastructure changes were investigated under transmission electron microscope. Two normal cornea specimens were used as control. Results:In the CDB I cornea epithelium, microvillus were decreased or lost. Small clumps of deposits with high electron density were found between basal cells. Some basal cells appeared apoptosis sign. A great quantity of rod-shape deposits were seen in Bowman′s layer and the anterior part of stroma. Conclusions:CDB I patients have characteristic corneal structure changes,and may be one of the reasons of the clinical symptoms.