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真性囊膜剥脱综合征合并白内障一例
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Abstract:
真性囊膜剥脱综合征(True Exfoliation Syndrome, TEX)是一种罕见的全身性细胞外基质疾病，在眼部则累及眼前节组织，当疾病发展到一定程度，其典型表现为晶状体前囊膜发生板层剥离，游离端在前房水中飘动，常合并晶状体混浊，部分合并晶状体前悬韧带的断裂。由于眼部表现有相似性，常易于与假性囊膜剥脱综合征混淆。TEX确切的发病机制未明，可能与长期高温环境下工作、暴露于热辐射、红外线、眼外伤、年龄相关性退行性变化、炎症等因素有关，也有相当一部分为特发性。文章报道一例真性囊膜剥脱综合征合并老年性白内障的病例，旨在提高对本病诊断和治疗的认知。
True Exfoliation Syndrome (TEX) is a rare systemic extracellular matrix disorder that primarily affects anterior segment structures in the eye. As the disease progresses, it is characterized by lamellar delamination of the anterior lens capsule, with free-floating edges visible in the aqueous humor. TEX often coexists with lens opacities and, in some cases, is accompanied by a break of the anterior zonular fibers. Due to similar ocular presentations, TEX is frequently mistaken for Pseudo-Exfoliation Syndrome. The exact pathogenesis of TEX remains unclear but is believed to be associated with long-term occupational exposure to high temperatures, thermal radiation, infrared light, ocular trauma, age-related degenerative changes, and inflammation, while a significant number of cases are idiopathic. This report presents a case of True Exfoliation Syndrome associated with age-related cataracts, aiming to improve awareness of the diagnosis and treatment of this rare condition.
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