Unilateral or asymmetric pseudoexfoliation syndrome? : An ultrastructural study
Unilateral or asymmetric pseudoexfoliation syndrome? : An ultrastructural study
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DOI：
10.1001/archopht.119.7.1023
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发表时间：
2001-07-01
影响因子：
--
通讯作者：
Naumann, GOH
中科院分区：
文献类型：
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作者：
Hammer, T;Schlötzer-Schrehardt, U;Naumann, GOH
背景：临床上，大多数假性剥脱（PEX）综合征患者仅表现出单侧眼部受累。然而，该疾病的普遍性表明 PEX 综合征在临床上是不对称的，而不是严格单侧的。目的：对单侧 PEX 综合征患者的对侧眼睛进行超微结构研究。方法：使用抗人类自然杀伤 (HNK-1) 表位的抗体，通过透射电子显微镜和光学和电子显微镜免疫组织化学方法对五对具有单侧 PEX 综合征裂隙灯显微镜、肉眼和光学显微镜证据的供体眼睛和 6 只正常对照眼睛进行研究以及针对潜在转化生长因子β (1) 结合蛋白，这两种标记物均用于识别 PEX 沉积物。结果：在所有明显未涉及的对侧眼的眼前段组织中观察到超微结构改变。这些包括（1）典型的 PEX 原纤维沉积在虹膜和睫状上皮以及虹膜扩张肌中； (2)细胞外基质的积累增加，包括虹膜血管周围、扩张肌和小梁网的近小管组织中的微纤维和重复的基底膜材料，以及(3)虹膜色素上皮和扩张肌细胞的退行性改变。在所有受影响眼和对侧眼的虹膜血管周围和扩张肌中均检测到潜在的转化生长因子 β (1) 结合蛋白和 HNK-1 阳性沉积物，表明 PEX 物质积聚，但在对照眼中未检测到。结论：临床上所谓的单侧 PEX 综合征中对侧眼的这些亚临床改变支持这样的概念：PEX 综合征是一种普遍的基本上双侧疾病，具有临床上明显的不对称表现。虹膜变化可能是早期阶段特征性临床症状的原因，例如黑色素分散、瞳孔周围萎缩、小梁网色素沉着和不对称瞳孔散大不足。研究结果应在患者的临床管理中加以考虑。 临床相关性：鉴于 PEX 综合征是全世界开角型青光眼最常见的可识别病因，并且它是多种眼部并发症的重要危险因素，特别是在白内障手术期间，因此 PEX 过程中双眼的潜在参与具有临床意义。
Background: Clinically, most patients with pseudoexfoliation (PEX) syndrome reveal only unilateral ocular involvement. However, the generalized nature of the disorder suggests that PEX syndrome is clinically asymmetric rather than strictly unilateral.Objective: To perform an ultrastructural study of the contralateral eyes in patients with unilateral PEX syndrome.Methods: Five pairs of donor eyes with slitlamp microscopic, macroscopic, and light microscopic evidence of unilateral PEX syndrome and 6 normal control eyes were investigated by transmission electron microscopy and light and electron microscopic immunohistochemistry using antibodies against the human natural killer (HNK-1) epitope and against latent transforming growth factor beta (1)-binding protein, both markers for the identification of PEX deposits.Results: Ultrastructural alterations were observed in anterior segment tissues of all apparently not involved fellow eyes. These included (1) deposits of typical PEX fibrils on the iris and ciliary epithelia and in the dilator muscle of the iris; (2) increased accumulation of extracellular matrix, including microfibrils and reduplicated basement membrane material in the periphery of iris vessels, in the dilator muscle and in the juxtacanalicular tissue of the trabecular meshwork, and (3) degenerative changes of the iris pigment epithelium and dilator muscle cells. Latent transforming growth factor beta (1)-binding protein- and HNK-1-positive deposits indicating PEX material accumulations were detected in the periphery of iris vessels and in the dilator muscle in all affected and contralateral eyes, but not in the control eyes.Conclusions: These subclinical alterations of contralateral eyes in clinically so-called unilateral PEX syndrome support the concept that PEX syndrome is a generalized basically bilateral disorder with a clinically marked asymmetric manifestation. The iris changes may account for the clinical signs characteristic of early stages, such as melanin dispersion, peripupillary atrophy, trabecular meshwork pigmentation, and insufficient asymmetric mydriasis. The findings should be considered in the clinical management of the patients.Clinical Relevance: In view of the fact that PEX syndrome is the most common identifiable cause of open-angle glaucoma worldwide and as it is an important risk factor for a wide spectrum of ocular complications, particularly during cataract surgery, the potential involvement of both eyes in the PEX process is of clinical significance.