先天性膜性白内障临床研究进展

Progress in clinical research of congenital membranous cataract

摘要:

先天性白内障是导致世界范围内儿童盲的主要原因，其中先天性膜性白内障是临床上一种罕见的特殊类型的先天性白内障，由于晶状体纤维在母体内发生退行性变，皮质逐渐被吸收形成先天性膜性白内障。先天性膜性白内障临床表现类似于后发性白内障，但无屈光能力，因此先天性膜性白内障也被称为假性无晶状体。目前，国内外关于先天性膜性白内障的报道较少，而针对先天性膜性白内障发病机制的研究更少。明确先天性膜性白内障的发病机制，尤其是对基因遗传学的研究，有助于理解先天性白内障的发病机制和晶状体发育的分子机制。

Abstract:

Congenital cataract is one of the leading causes of childhood blindness and congenital membranous cataract is a rare and special type of congenital cataract. The lens fibre of congenital membranous cataract is degenerative and its cortex is absorbed gradually. Congenital membranous cataract also has another name, pseudoaphakia, due to the similar phenotype with posterior capsule opacification after cataract surgery, but without intraocular refractive power. There are few reports on congenital membranous cataract at home and abroad, and the research on the pathogenesis of congenital membranous cataract is even less. Clarifying the pathogenesis of congenital membranous cataract, especially the genetics, is very helpful for us to understand the pathogenesis of congenital cataract and the molecular mechanism of lens development.