Systemic diseases associated with exfoliation syndrome

BettisD.I.; AllinghamR.R.; WirostkoB.M.

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Systemic diseases associated with exfoliation syndrome

机译：与剥脱综合征相关的全身性疾病

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Exfoliation syndrome (XFS) is a common, complex inherited disorder. Although ocular associations are well known, systemic manifestations of XFS are rarely noted or described. Ocular findings include exfoliative glaucoma (XFG), angle closure glaucoma, well-characterized anterior segment signs, increased risk of ocular complications with cataract surgery, and an increased risk of central retinal vein occlusion. What has been more challenging is to attempt to characterize associated systemic disorders. Exfoliative material (XFM) composed of characteristic microfibrils is found in multiple ocular tissues and systemically, including the skin, heart, lungs, liver, kidneys, and cerebral meninges of individuals with clinical XFS/XFG. XFS is a microfibrillopathy that is strongly associated with variants of the gene lysyl oxidase-like 1 (LOXL1). LOXL1 is intimately associated with elastogenesis and is expressed systemically.

机译：剥脱综合征（XFS）是一种常见的复杂遗传性疾病。尽管眼部关联是众所周知的，但是XFS的全身表现很少被提及或描述。眼部检查结果包括剥脱性青光眼（XFG），闭角型青光眼，特征明确的前节征，白内障手术引起的眼部并发症风险增加以及视网膜中央静脉阻塞的风险增加。更具挑战性的是试图表征相关的全身性疾病。由特征性微纤维组成的剥脱性物质（XFM）在多个眼部组织和全身中都发现，包括具有临床XFS / XFG的个体的皮肤，心脏，肺，肝，肾和脑膜。 XFS是一种微纤颤病，与基因赖氨酰氧化酶样1（LOXL1）的变体密切相关。 LOXL1与弹性发生密切相关，并系统表达。

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《International ophthalmology clinics》 |2014年第4期|共14页
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BettisD.I.; AllinghamR.R.; WirostkoB.M.;
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正文语种 eng
中图分类眼科学;
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1. Systemic diseases associated with exfoliation syndrome [J] . BettisD.I., AllinghamR.R., WirostkoB.M. International ophthalmology clinics . 2014,第4期

机译：与剥脱综合征相关的全身性疾病
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Systemic diseases associated with exfoliation syndrome

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