囊膜剥脱综合征的研究进展

囊膜剥脱综合征是一种与年龄相关的全身性细胞外基质疾病,在发病过程中,可继发青光眼,加速白内障的进展,使术中和术后并发症明显增多.囊膜剥脱综合征的发生机制至今尚不清楚,因此,有必要就目前对囊膜剥脱综合征的临床、分子病理学、遗传学等方面的研究进展进行综述,旨在加深对囊膜剥脱综合征发病机制的认识.     

剥脱性青光眼发病机制的研究进展

剥脱性青光眼是继发于剥脱综合征的一种青光眼类型。近年来, 国内外学者对剥脱性青光眼的发病机制和危险因素进行了大量研究, 发现了多种危险基因、异常表达的生长因子和细胞因子以及广泛的眼前、后节改变。剥脱性青光眼与全身疾病和环境存在一定关联。本文在对这些成果进行系统总结的基础上, 指出当前亟待进行深入研究的问题, 以期为该领域提供参考。     

囊膜剥脱综合征的研究进展

囊膜剥脱综合征是一种与年龄相关的全身性细胞外基质疾病,在发病过程中,可继发青光眼,加速白内障的进展,使术中和术后并发症明显增多.囊膜剥脱综合征的发生机制至今尚不清楚,因此,有必要就目前对囊膜剥脱综合征的临床、分子病理学、遗传学等方面的研究进展进行综述,旨在加深对囊膜剥脱综合征发病机制的认识.     

囊膜剥脱综合征的研究进展

囊膜剥脱综合征是一种与年龄相关的全身性细胞外基质疾病,在发病过程中,可继发青光眼,加速白内障的进展,使术中和术后并发症明显增多.囊膜剥脱综合征的发生机制至今尚不清楚,因此,有必要就目前对囊膜剥脱综合征的临床、分子病理学、遗传学等方面的研究进展进行综述,旨在加深对囊膜剥脱综合征发病机制的认识.     

剥脱综合征性青光眼的发病机制

剥脱综合征是一种常见的年龄相关性眼病,是导致青光眼的常见原因.剥脱综合征性青光眼较慢性单纯性青光眼更严重、预后更差.在此文中对近年来,剥脱综合征性青光眼发病机制的有关论著作一综述.     

白内障合并真性囊膜剥脱综合征手术方式探讨

目的　探讨手术治疗白内障合并真性囊膜剥脱综合征的安全性。方法　回顾性分析２０１０年３月至２０１５年１月期间在河北省眼科医院白内障科就诊的白内障合并真性囊膜剥脱综合征患者４例（５眼）,其中３眼悬韧带正常,我们在撕囊和正常超声乳化手术过程中未发现任何异常;另外２眼合并晶状体不全脱位,我们使用４个虹膜拉勾固定囊袋,囊袋内预劈技术将晶状体核劈成８块,扭动超声乳化核及吸除皮质后,囊袋内植入张力环及人工晶状体。比较术前及术后最佳矫正视力、超声乳化总时间、灌注液消耗量、角膜中央内皮细胞密度、角膜内皮细胞丢失率,观察囊袋及人工晶状体有无偏心移位以及并发症的发生情况。结果　术后３个月最佳矫正视力０．８者２眼,０．６者１眼,０．５者２眼,术后３个月最佳矫正视力均较术前增加。晶状体超声乳化时间为３０．２～５２．６（４５．４±９．１）ｓ。灌注液消耗量为６３．１８～８０．２６（７１．９７±６．９４）ｍＬ。术前角膜中央内皮细胞密度２１６３～２８９２（２５０５．２±３１７．４）ｍｍ－２,术后３个月为２０７５～３０２３（２４１５．４±３０８．１）ｍｍ－２。角膜内皮细胞丢失率为３．５９％。术后５眼测得人工晶状体偏中心量为０．１２～０．４６（０．３０±０．１５）ｍｍ。５眼人工晶状体均成功植入囊袋内,术中未出现后囊膜破裂、悬韧带损伤或加重,术后第１天角膜均透明,远期未出现囊袋收缩、后囊膜混浊等并发症。结论　采用囊袋内预劈核联合虹膜拉勾囊袋固定和囊袋内植入张力环,可以增加合并真性囊膜剥脱综合征白内障手术的安全性。     

新疆喀什地区假性剥脱综合征白内障复明手术探讨

目的评价和分析新疆喀什地区假性剥脱综合征（pseudoexfoliation syndrom，PES）白内障手术。方法本资料来自2004年6～8月在我国新疆喀什地区“健康快车”完成的晶状体超声乳化和人工晶状体植入术。诊断PES组有74例（74眼）。间隔随机抽样设置非PES组作对照组（448例）。比较两组手术效果，和手术复杂性（合并小瞳孔、晶状体悬韧带离断、晶状体半脱位或全脱位等）。结果两组术后视力差异无统计学意义（P〉0．05）。两组手术复杂性的差异有统计学意义。结论PES白内障手术后仍可获得良好的复明效果。新疆喀什地区（3个月）比较集中发现PES白内障，推测该地区是我国PES高发区。     

剥脱综合征发病机制的研究进展

剥脱综合征(exfoliation syndrome,XFS)是一种与年龄相关的、迟发性、多因素参与的系统性疾病,是继发性开角型青光眼的主要原因之一.XFS发病与遗传因素和环境因素有关,但具体发病机制尚未阐明.本文将近年来XFS发病机制研究进展进行总结,从LOXL1基因多态性、基因甲基化、环境因素、自噬等可能参与XFS形成和发展多方面进行阐述,以利于XFS的防治.     

假性剥脱综合征中感觉神经性听力丧失

目的：评估眼部假性剥脱综合征(pseudoexfoliation syndrome, PXF)与感觉神经性听力丧失(sensory-neural hearing loss, SNHL)之间的关系。方法：该前瞻性病例对照研究选取2010-03/12在伊朗乌尔米耶,伊玛何梅尼医疗中心普通眼科门诊就诊的患者。常规眼科检查后,确诊为PXF的患者建议去耳鼻喉科,被选病例（纳入排除标准评估后）被推荐到听力检测科。分别在1, 2, 3 kHz对每只耳进行了纯音听阈级检测(Pure tone hearing threshold level, HTL),并将检测结果与国际标准(International Standard,ISO 7029)在1, 2, 3 kHz平均年龄相关性听力丧失(age associated hearing loss, AAHL)进行比较。结果：在1, 2, 3kHz,50例患者中21例(42.0%)HTL高于ISO 7029平均AAHL,包括男性组9例14耳,女性组12例21耳。大约12.0%的患者同时伴有青光眼,不过,PXF患者和同时伴有青光眼的患者在SNHL的患病率和严重程度上无明显差别。同年龄-性别匹配对照组相比,眼部PXF的患者SNHL更普遍(P<0.05)。结论：大部分眼部PXF患者有SNHL,与年龄-性别匹配对照组相比,可能是由于其内耳的PXF纤维造成。这些发现表明PXF可能是一个系统性疾病。     

Orbital blood flow parameters in unilateral pseudoexfoliation syndrome

Purpose The evaluation of orbital blood flow parameters in patients with unilateral pseudoexfoliation syndrome (XFS) and in age–sex-matched control subjects using color Doppler imaging. Methods This prospective, comparative case series included 33 unilateral XFS patients and 33 age–sex-matched control subjects. Color Doppler imaging was used to evaluate the XFS-affected (Group 1) and -unaffected (Group 2) eyes that were matched with the corresponding side (Groups 3 and 4) of control subjects. All subjects were given a complete ophthalmological examination. A masked radiologist obtained the peak systolic velocity (PSV) and end diastolic velocity (EDV) of ophthalmic (OA), central retinal (CRA), nasal posterior ciliary (NPCA) and temporal posterior ciliary arteries (TPCA) as well as mean velocity of central retinal vein (CRV). Resistivity indices (RI) were calculated. Results Analysis of peak systolic and end diastolic velocity (PSV, EDV) and RI between Groups 1–2 and 3–4 did not show any significant difference. Comparing Groups 1–3 (corresponding eyes of pseudoexfoliation-affected and control subjects) and Groups 2–4 (corresponding eyes of pseudoexfoliation-unaffected and control subjects) revealed significantly low values for OA, PSV and EDV in both eyes of the XFS patients. The OA RI value in Group 1 was found to be significantly higher than that in Group 3 with the cut-off value of 0.72. Conclusion These findings suggest that ophthalmic artery hemodynamic parameters change in both eyes of unilateral XFS patients, illustrating out the systemic nature of the disease. We conclude that XFS decreases OA blood flow velocities and increases vascular resistance through progressive build-up.     

不同劈核方法对假性剥脱综合征性白内障手术的疗效观察

目的观察不同劈核方法治疗维吾尔族患者假性剥脱综合征(PEX)白内障的临床疗效。方法回顾性研究。选取2018年10月至2019年10月在中国人民解放军新疆军区总医院全军眼科中心行超声乳化手术治疗的维吾尔族患者PEX性白内障122例(122眼), 根据手术方式分为两组:拦截劈核病例作为观察组, 60例(60眼);预劈核病例作为试验组, 62例(62眼)。比较两组术后1 d、1周及3个月的视力、角膜水肿及角膜内皮损失率情况。结果观察组平均有效超声时间为(14.50±5.63)s, 较试验组(12.32±7.34)s长(t=15.12, P<0.05);试验组术后1 d、1周的视力优于观察组(Z=-5.070, -6.230;均P<0.001), 术后1 d、3 d的角膜水肿程度低于观察组(Z=-3.490, -3.710;均P<0.001)。试验组角膜内皮丢失率较观察组较少(t=2.110, P=0.001)。结论与拦截劈核相比较, 预劈核技术在维吾尔族PEX患者白内障术中超声时间较短、能量较低、角膜内皮细胞丢失率较少, 视力恢复较快。     

Retrobulbar hemodynamic parameters in pseudoexfoliation syndrome and pseudoexfoliative glaucoma

OBJECTIVE: To compare the hemodynamic parameters in the retrobulbar vessels in pseudoexfoliative syndrome (PXS), pseudoexfoliative glaucoma (PXG), and age-matched healthy subjects by using color Doppler imaging (CDI). SUBJECTS AND METHODS: 72 eyes from 72 patients with PXS, 70 eyes from 70 patients with PXG, and 66 eyes from 66 age-matched healthy subjects who met the inclusion/exclusion criteria were included in this prospective cross-sectional study. Peak systolic velocity (PSV), end-diastolic velocity (EDV), and Pourcelot resistance index (RI) were assessed in the ophthalmic artery (OA), central retinal artery (CRA), and temporal short-posterior ciliary arteries (SPCA). Visual function was assessed using the 24-2 Swedish Interactive Threshold Algorithm (SITA). The main outcomes of the study were PSV, EDV and RI in the OA, SPCA, and CRA. RESULTS: The EDV in the OA, SPCA and the CRA was decreased significantly (p < 0.001, <0.001, and 0.003 respectively) in the eyes of PXG patients compared with controls and PSX respectively. The RI in the OA, SPCA, and the CRA was significantly higher (p = 0.022, 0.005, and 0.007 respectively) in the eyes of PXG patients compared with healthy controls and PSX patients respectively. The mean difference in mean EDV in the OA between the control group and the PXS was 0.18 cm/s, 95% confidence interval (CI) -0.60 to 0.95, p = 0.661. The mean difference in mean EDV in the SPCA between the control healthy subjects and the pseudoexfoliative subjects was -0.18 cm/s, 95% CI -0.45 to 0.08, p = 0.176. Multivariate regression analysis showed that in the PXG patients the PSV and EDV in the CRA were significantly positively correlated with the mean defect (p = 0.006 and 0.002 respectively). The RI in the CRA was significantly negatively correlated with the mean defect in PXG patients, p = 0.009. CONCLUSION: The results of this study showed that the retrobulbar hemodynamics might be disturbed in patients with PXG, especially in the central retinal artery. Our results have found no significant differences in the retrobulbar hemodynamic parameters between pseudoexfoliative syndrome patients and age-matched healthy subjects.     

MicroRNA-related polymorphisms in pseudoexfoliation syndrome,pseudoexfoliative glaucoma,and primary open-angle glaucoma

Background: Pseudoexfoliation syndrome (PEX) and glaucoma (pseudoexfoliative glaucoma; PEXG, primary open-angle glaucoma; POAG) have mainly been studied for their associations with genes’ polymorphisms. The purpose of this exploratory study was to investigate the role of polymorphisms in genes encoding for micro RNAs (miRNAs) and in genes related to miRNA biogenesis.

Material and Methods: In the present genetic association study, ninety-two polymorphisms were investigated for their contribution to PEX (n = 203), PEXG (n = 38), and POAG (n = 40) pathogenesis compared to a control group (n = 188). The next generation sequencing (NGS) genotypic analysis revealed data for additional 28 variants.

Results: A protective association was found between PEX and polymorphism 11382316 (mir-3161) [odds ratio (OR) = 0.64, 95% confidence interval (CI): 0.47–0.86, p = 0.003], rs2155248 (mir-1304) [OR = 0.66, 95%CI: 0.47–0.94, p = 0.019], and rs28635903 (mir-1268a) [OR = 0.30, 95%CI: 0.10–0.94, p = 0.029]. Polymorphism rs113297757 (mir-3196) was associated with an increased risk of POAG [OR = 7.75, 95%CI: 2.13–28.76, p = 3 × 10^?4]. Polymorphism rs1057035 (DICER) and rs55671916 (XPO5) in the 3?-UTR of genes related to miRNA biogenesis was associated with decreased risk of PEX [OR = 0.65, 95%CI: 0.46–0.92, p = 0.014] and increased risk of PEXG [OR = 2.84, 95%CI: 1.02–7.94, p = 0.038], respectively. The aforementioned associations according to the allelic model were further supported by the genotypic models of statistical analyses.

Conclusions: This is the first study to report distinct associations of PEX, PEXG, and POAG in the same population with variants of genes involved in miRNA biogenesis and with miRNA genes’ polymorphisms. Further studies in larger groups of patients of various origins are needed to confirm the reported preliminary results.     

The role of the cytokines in the pathogenesis of pseudoexfoliation syndrome

AIM: To examine the mechanism of the development of pseudoexfoliation (PSX) syndrome via both cytokine formation and endothelial vasorelaxing and growth factors that will provide us new therapeutic insights for the treatment.METHODS: This is a cross sectional study included two groups; Group 1:control patients with nuclear cataract (n=20, aged 51-80 years). Group 2:PSX patients with nuclear cataract (n=18, aged 50-90 years). Patients with other ophthalmic problems and systemic diseases were excluded. Vascular endothelial growth factor (VEGF), interleukin-6 (IL-6) and interleukin-1β (IL-1β) and nitrotyrosine levels were determined through serum samples by Enzyme-linked immunosorbent assay (ELISA) method. Nitrite-nitrate levels were measured with photometric endpoint determination.RESULTS: There were no significant differences between the groups in terms of age, VEGF, IL-1β, nitrite-nitrate and nitrotyrosine. The significant results were the mean IL-6 levels that were higher in PSX group 2 (37.68±29.52 pg/mL) compared to that in control group 1 (15.32±10.08 pg/mL) (P<0.001).CONCLUSION: Several interacting and extending biochemical pathways may lead to the promotion of VEGF and IL-6 expressions. IL-6 which is the only altered marker in our study may indirectly cause an increase of vascular permeability and neovascularization. We suggest inflammation as a factor that can be involved in etiopathogenesis of PSX.     

假性剥脱综合征中的Charles Bonnet综合征(英文)

目的:报道1例进展期假性剥脱性青光眼中的Charles Bonnet综合征。方法:病例报告。结果:一例92岁的马来男性诉2a前出现幻视,并加重2mo。称看见成人及百余儿童生动活泼地出现在不同场合。他甚至有几次看见一只兔子偶尔跑过他的房间。但是,他觉察到这是一种异常的经历。与同年龄人相比,他的认知功能是正常的。精神病学评估包括精神状态检查是正常的。他曾经被诊断患有开角型青光眼,左眼以前接受过常规的白内障手术和小梁切除术。其视力为右眼手动,左眼6/7.5(0.8)伴管状视野。眼底镜检查显示左眼进行性青光眼性视杯加深。目前诊断为右眼大泡性角膜病变合并绝对期青光眼,左眼进展期假性剥脱性青光眼,有功能性滤过泡。结论:对诉有幻视的老年患者,特别是在痴呆和精神病的诊断标准不明确的情况下,诊断通常是很困难的。如果这些患者洞察到不复存在的东西进入其视觉范围,并伴有视觉功能恶化,应高度怀疑患有Charles Bonnet综合征的可能。     

剥脱综合征临床分析

目的探讨剥脱综合征（PEX）的临床表现及其特点,寻求有效的治疗途径。方法对34例49眼剥脱综合征患者的临床资料进行分析。结果最小发病年龄61岁,平均73岁。全部伴有不同程度的白内障。就诊时眼压已超过21mmHg者占全部PEX眼的55.1%,而已经发生青光眼性视野改变者也达到了全部视野检查的PEX眼的48.48%。25眼行超声乳化白内障摘除手术前后平均眼压分别为24.41mmHg和17.23mmHg。结论剥脱综合征是一种老年性的、以纤维状物质在许多眼部组织中的产生和进行性蓄积为特征的细胞外基质代谢紊乱性疾病。开角型青光眼发生风险大,在青光眼早期对所伴有的影响视力的白内障施行超声乳化摘除术可能会阻止其青光眼的进展。所伴青光眼病理过程严重,需要滤过手术介入。提高对本病的认识是避免延误其所伴青光眼治疗以及预防各种手术并发症风险的重要因素。