【摘要】 目的:探讨家族性先天性无虹膜症眼部病变的发生发展规律。方法:对先天性无虹膜家族患病成员进行基因分析的基础上,进行眼部的各种检查,包括视力、角膜、晶状体、玻璃体、房角、小梁网、眼底、眼压、泪液分泌实验和泪道的检查等。结果:该家系先天性无虹膜的发病率为51%,致盲率占30%;在该家系中,年龄越小的患者病变越轻,30岁以上年龄的患者已经表现出严重病变。结论:该先天性无虹膜家系眼部病变随着年龄增长,不断加重,30岁以后病变达到严重程度,致盲率高。更多还原

【Abstract】 · AIM: To discuss the principle of the development of ocular pathological changes in familial congenital aniridia.· METHODS: On the basis of the genetic analysis of the family of congenital aniridia, physical examinations were carried out, which included visual acuity, cornea, lens, vitreous body, anterior chamber angle, fundus, IOP, Schirmer test and lacrimal canal, etc.· RESULTS: Incidence of congenital aniridia is 51%, and the rate of blindness is 30%. Pathological change is mild in baby, while severe in the age above 30.· CONCLUSIONS: The severity of the ocular pathological changes in congenital aniridia correlates with the age. As age increases, pathological changes aggravate. Pathological changes are severe in the age above 30, and the rate of blindness is high.·更多还原