角膜是基因治疗的理想靶器官。角膜碱烧伤、角膜新生血管、角膜移植术后排斥反应因其病理机制复杂,所牵涉的致病因素众多而治疗困难,疗效不佳。本文就基因治疗在上述疾病中的应用加以综述,以了解基因治疗应用于角膜病变的新进展 。
Cornea is an ideal target organ for gene therapy. Corneal alkali burn, cornealneovascularization and corneal graft rejection tend to be with poor treatment elicacydue to its complex pathogenesis. This article aims to update the recent progress of genetherapy on corneal diseases.
髓上皮瘤是源自神经系统的一种少见的恶性肿瘤,多发生在中枢神经系统和睫状体,而源自视神经的恶性髓上皮瘤则很少见,国内尚未有病例报道。此病早期类似胶质瘤,易造成误诊。本文报道了 1 例 3 岁 10 个月的男性患儿,经部分肿物切除活检发现肿瘤具有典型恶性髓上皮瘤的病理特点, 部分瘤细胞向软骨细胞分化,并逐渐形成透明软骨岛,NSE 及 S-100 表达阳性,病理诊断为源自视神经的畸胎性恶性髓上皮瘤。
Medulloepithelioma is a clinically uncommon tumor originated from nervous system, often occurred in central nerve system and ciliary body, and malignant medulloepithelioma of the optic nerve is far rarer. So far, there has been no case report in China. It may be clinically misdiagnosed because it resembles glioma at the early stage of the disease. We reported a boy with a tumor in his right eye at age of 3.8 years, which was shown by biopsy of the partial tumor that there were some obviously heteromorphous neoplastic cells, karyokinesis, and moreover, some neoplastic cells differentiatied into cartilage cells, gradually formed into hyaline cartilage islands and the expressions of NSE and S-100 were positive. Teratoid malignant medulloepithelioma of optic nerve was made pathologically.
目的: 报道原发性空泡蝶鞍综合征致双眼视乳头水肿病案 1 例。方法: 回顾性研究1例原发性空泡蝶鞍综合征致双眼视乳头水肿患者的临床表现、眼底改变、CT 和 MRI 影像学检查的特征、治疗方法及疗效。结果 : 原发性空泡蝶鞍综合征致双眼视乳头水肿除有典型视乳头水肿的临床表现外,蝶鞍 MRI 亦显示垂体窝呈液型信号、垂体上缘受压凹陷、垂体变薄等典型空泡蝶鞍影像学表现。手术治疗后患者视乳头水肿改善、视力提高。结论: 蝶鞍 MRI 是诊断原发性空泡蝶鞍综合征的首选影像学检查方法。视力下降明显的患者及时行蝶鞍区手术,术后效果良好。
Purpose:To report a case of papilloedema caused by primary empty sella turcica syndrome.Methods:Retrospectively review the clinical and physical features, magnetic resonance imaging records and therapies of a patient with papilloedema caused by primary empty sella turcica syndrome.Results: Except for typical clinical manifestation of papilloedema , a characteristic magnetic resonance imaging (MRI) can be found in a case of papilloedema caused by primary empty sella turcica syndrome. These imaging features are that sella turcica expanded, the inside of sella turcica was filled with cerebrospinal fluid(CSF) signal, pituitary gland was pressed, flatted and near the basis of sella turcica. Papilloedema was relieved and acuity of vision improved after surgery.Conclusions:MRI is the preferred imaging technique for patient with papilloedema caused by primary empty sella turcica syndrome. If acuity of vision apparentlydecreases,surgery is necessary, and therapeutic effect is excellent.
神经营养性角膜病变是一种与角膜神经退行性改变有关的疾病,角膜神经的知觉和营养功能受损,导致角膜上皮缺损、角膜溃疡甚至角膜穿孔。目前人工泪液、治疗性角膜绷带镜、泪点栓塞、羊膜移植,睑缘缝合等治疗措施仍是治疗神经营养性角膜病变的主要治疗方式,对于轻中度病变患者,具有较好的治疗效果,而对于重度病变患者,药物治疗及简单的手术干预治疗效果不佳,病情反复发作。由于重度神经营养性角膜病变患者的角膜神经完全消失,丧失角膜感觉,对未恢复角膜神经营养功能的角膜白斑或溃疡患者行角膜移植术,可能导致角膜移植术后上皮持续不愈合,因此恢复角膜神经营养功能是复明的重要保障手段。角膜神经移植术是重度神经营养性角膜病变患者恢复角膜神经营养功能,提高角膜知觉,改善角膜透明度的重要和有效的治疗方法。角膜神经移植术通过将具有正常功能的供体神经移植到麻痹眼角膜缘周围,使神经末梢重新长入角膜基质,恢复角膜知觉功能。随着角膜神经移植术的术式的不断改进,其良好的术后效果和优点已经渐渐突显。角膜神经移植术包括直接角膜神经移植和间接角膜神经移植,促使角膜神经重新生长,重建角膜神经的营养和知觉功能。角膜神经移植手术已有40年历史,1981年Samii等首次报告了角膜神经移植术,2009年Terzis等成功地实施了第1例直接角膜神经移植术,2014年Elbaz等进行了第1例以腓肠神经作为间置移植物的间接角膜神经移植。封面展示了神经营养性角膜病变患者未接受治疗前的和接受角膜神经移植术后的眼表角膜图像。由于角膜神经退行性改变,角膜失去神经支配,继而出现角膜上皮缺损,角膜缘新生血管形成,经角膜神经移植后,角膜上皮愈合,角膜透明度改善,同时角膜缘新生血管消退。
神经营养性角膜病变是一种与角膜神经退行性改变有关的疾病,角膜神经的知觉和营养功能受损,导致角膜上皮缺损、角膜溃疡甚至角膜穿孔。目前人工泪液、治疗性角膜绷带镜、泪点栓塞、羊膜移植,睑缘缝合等治疗措施仍是治疗神经营养性角膜病变的主要治疗方式,对于轻中度病变患者,具有较好的治疗效果,而对于重度病变患者,药物治疗及简单的手术干预治疗效果不佳,病情反复发作。由于重度神经营养性角膜病变患者的角膜神经完全消失,丧失角膜感觉,对未恢复角膜神经营养功能的角膜白斑或溃疡患者行角膜移植术,可能导致角膜移植术后上皮持续不愈合,因此恢复角膜神经营养功能是复明的重要保障手段。角膜神经移植术是重度神经营养性角膜病变患者恢复角膜神经营养功能,提高角膜知觉,改善角膜透明度的重要和有效的治疗方法。角膜神经移植术通过将具有正常功能的供体神经移植到麻痹眼角膜缘周围,使神经末梢重新长入角膜基质,恢复角膜知觉功能。随着角膜神经移植术的术式的不断改进,其良好的术后效果和优点已经渐渐突显。角膜神经移植术包括直接角膜神经移植和间接角膜神经移植,促使角膜神经重新生长,重建角膜神经的营养和知觉功能。角膜神经移植手术已有40年历史,1981年Samii等首次报告了角膜神经移植术,2009年Terzis等成功地实施了第1例直接角膜神经移植术,2014年Elbaz等进行了第1例以腓肠神经作为间置移植物的间接角膜神经移植。封面展示了神经营养性角膜病变患者未接受治疗前的和接受角膜神经移植术后的眼表角膜图像。由于角膜神经退行性改变,角膜失去神经支配,继而出现角膜上皮缺损,角膜缘新生血管形成,经角膜神经移植后,角膜上皮愈合,角膜透明度改善,同时角膜缘新生血管消退。
目的:通过观察对金黄色葡萄球菌性角膜溃疡的疗效,筛选克拉霉案眼用凝胶的合适浓度。方法:角膜实质层接种法建立40只家兔右眼金黄色葡萄球菌性角膜溃疡模型,将模型随机分成5组,每组8只免(8只眼),各组分别给予空白基质、0.1%克拉素眼用凝胶、0.25%克拉霉素眼用凝胶、左氧氟沙星凝胶、0.25%克拉霉素眼用凝胶联合重组牛碱性成纤维细胞生长因子(Recombinant bovine basic fibroblast growth factor, Rb-bFGF),每天4次,每次2滴,分别在第1、3、5、7、10、14 天观察角膜病变情况及溃疡面积大小。结果:在相同的给药方法下,0.1%克拉霉素眼用凝胶、0.25%克拉素眼用凝胶、左氧沙星凝胶、0.25%克拉霉素眼用凝胶联合Rb-bFCF均能使金黄色葡萄球菌性角膜溃疡面积缩小角膜病变好转,与空白基质组相比有统计学差异(P < 0.05):0.25%克拉素眼用凝胶组疗效明显优于0.1%克拉荐素眼用凝胶组(P < 0.05)。结论:制备的 0.25%克拉霉素眼用凝胶对金黄色葡萄球菌性角膜溃疡疗效肯定,可以进一步开发应用于临床。
Purpose:To screen proper concentration of clarithromycin ophthalmic gel by observingthe efficacy of different concertrations of clarithromycin ophthalmic gel for treatingstaphylococcal corneal ulcers.
Methods:Corneal ulcer was induced in the right eye of 40 rabbits, 3.0 x 10°CFU/mlstaphylococcus aureus suspension was injected midstromally into the central cornel.These rabbits were divided randomly into $ groups ,each group received respectivelytopical blank matrix, clarithromycin ophthalmic gel 0.1%, clarithromycin ophthalmicgel 0.25%,levofloxacin ophthalmic gel, clarithromycin ophthalmic gel 0.25% andrecombinant bovine basic fibroblast growth factor (Rb-bFGF), 4 times every day, 2drops each time. The eyes were examined respectively with the slit lamp beforetreatment(day0), on day3, day5, day7, day 10, day 14 to observe theprogression of corneal ulceration. including the area of the corneal ulcer and mark of keratitis.Resuls:Under the same way of giving medicine, experimental coreal ulcer studiesshowed a statistically significant decrease in all tratement groups on measurements ofthe area of the comeal ulcer and mark of keratitis(P<0.05), and clarithromycinophthalmic gel 0.25% had a better action than clarithromycin ophthalmic gel 0.1%against staphylococcus aureus corneal ulcer.
Conclusion:Clarithromycin ophthalmic gel 0,25% was proved to be an effective ocularmedication for the therapy of gram-positive bacterial corneal ulcer.
目的: 观察老年性黄斑变性(Age-related macular degeneration, AMD) 和息肉状脉络膜视网膜病变(Polypoidal choroidal vasculopathy, PCV) 患者眼底陈旧性出血在吲哚青绿血管造影(Indocyanine green angiography, ICGA) 中的自发荧光表现。方法: 对伴有眼底陈旧性出血的AMD和PCV患者共36例(36只眼)行ICGA检查。受试者在进行ICGA检查前, 均经过详细的眼底镜检查、眼底彩色照相及荧光素眼底血管造影(Fundus fluorescein angiography, FFA) 检查。结果: 眼底陈旧性出血灶 ICGA 均表现出相应的自发荧光。陈旧性出血灶呈浅灰黄色, 其自发荧光的形态大小与眼底彩色图像所示的陈旧性出血灶相一致, 边界清晰; 造影后期陈旧性出血灶的自发荧光强度最强,与 AMD 的斑状及焦点状脉络膜新生血管 (Choroidal neovasculari-zation, CNV) 及 PCV 的息肉状脉络膜血管扩张灶的荧光表现不同。陈旧性眼底出血的自发荧光多与 CNV 或息肉状病灶重叠或位于其边缘(27只眼, 75%) 。结论: ICGA 中陈旧性眼底出血所致的自发荧光易与 CNV 及息肉状病灶性强荧光相混淆, 将眼底彩色图像与 ICGA 图像对比分析及掌握其与CNV及息肉状血管扩张灶的不同荧光特性有助于鉴别诊断。
Objective: To investigate the autofluorescence of stale fundus haemorrhage in age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV) with indocyanine green angiography (ICGA) .Methods: The color photographs and ICGA were performed in 36 eyes of 36 cases of exudative AMD or PCV with stale fundus haemorrhage. All of the cases were examined by funduscopy and fundus fluorescein angiography (FFA) .Results: Autofluorescence could be observed in all of the stale haemorrhage cases. Stale haemorrhage showed grayish color and the shapes and sizes of autofluoresence in ICGA were in accordance with those of the stale haemorrhage in the color photographs. The boundaries of autofluorescence were clear and the intensities were strong. The percentage of choroidal neovascularization (CNV) or PCV in or beside stale haemorrh-age was significantly higher than that outside the stale haemorrhage (27 eyes, 75%) .Conclusions: Autofluorescence of stale haemorrhage in ICGA can be mixed up with the high fluorescence of CNV and grapes-like polypoidal dilatation. It is helpful to compare the color photographs with ICGA and recognize the different ICGA characteristics in the assessment of ICGA results in these circumstances.
目的:研究形觉剥夺性和光学离焦性近视豚鼠视紫红质的表达变化,探讨视紫红质表达与实验性近视眼之间的关系。方法:40只出生后1周的豚鼠随机分为形觉剥夺组和光学离焦组(n=20),形觉剥夺组单眼戴半透明(半透明薄膜贴于平镜表面)平光硬性角膜接触镜片(Rigidgass-pemmeable contactlens, RCP),光学离焦组单眼藏-4.0DRGP镜片,另一只眼为对照组。戴镜干预后1、2周各组分别测量屈光度、眼轴长度、玻璃体腔深度,并于上午10~12 点钟取材,实时荧光定量PCR观察视紫红质 mRNA 的变化 Westem-blot 观察视紫红质的变化,进行对比比较,统计分析。结果:实验干预后1周,形觉剥夺组和光学离焦组与对照组相比各项指标无显著性差(除形觉剥夺组屈光度以外)。实验干预后2周,与对照组相比较,形觉剥夺组和光学离焦组明显发生近视、眼轴延长、玻璃体腔加深(t=22.20、18.32、19.65、15.78、6.18、11.20.P<0.01):形觉剥夺组视紫红质及其 mRNA 表达均增加(t=17,489、14.31.P<0.05)光学离焦组视紫红质及其mRNA表达无明显变化。结论:视紫红质的表达可能参与了形觉剥夺性近视眼的形成,而在光学离焦性近视眼中作用有限。
Purpose:To investigate the rhodopsin expression in form-deprived and defocus myopiain guinea pig and study the relationship between the rhodopsin expression andexperimental myopia.Methods:Fourty guinea pigs were randomized into the form-deprived group and thedefocus group (n= 20 ). Guinea pigs in the form-deprived group wore a diffuser(rigidgass-permeable contact lens(RGP)on one eye since one week after birth. Those in defocus group wore a -4 D RGPon one eye. The contralateral eyes were left ascontrol. Refraction, axial length and depth of vitreous cavity were measured after 1and 2 weeks respectively. Retina were dissected at 10 ~ 12 o'clock in the moring.The level of rhodopsin and its mRNA were observed through Western-blot and real-time PCR respectively.Result:There is no difference between form-deprived group, defocus group and controlgroups(except refraction in form-deprived group). One week later, there is nodifference between the form-deprived group, the defocus group and the control groups(except refraction in form-deprived group). Two weeks later, eyes in the form-deprivedgroup and the defocus group became myopic. Its axial length lengthened and depth ofvitreous cavity appeared deep. The form-deprived groups showed an increasedexpression of rhodopsin and its mRNA compared to the control groups. There is nodifference between the defocus group and the control groups.Conclusion : Expression of rhodopsin might involve formation of form-deprived myopia,but has less influence on defocus myopia.
目的: 比较新鲜羊膜和保存羊膜治疗睑球粘连的疗效差异, 分析影响疗效的因素。方法: 共 51 例 55 只眼因陈旧性化学伤、热烧伤或 Stevens-Johnson 综合征而发生睑球粘连的临床连续病例接受睑球粘连分离联合新鲜羊膜移植(22 只眼) 或保存羊膜移植(33 只眼) 。其中男 30 例 32 只眼, 女 21 例 23 只眼。年龄 4~51 岁, 平均(34.2 ± 4.3) 岁。其中 11 只眼在烧伤后5~11个月, 平均(7.4 ± 1.6) 个月时接受手术, 40 只眼在烧伤后 1~8 年, 平均(2.0 ± 0.7) 年进行手术。结果: 术后随访 12~32 个月, 平均(19.3 ± 4.1) 个月 。所有移植在结膜眼表面的羊膜(包括新鲜羊膜) 植片在术后早期均未见溃烂和溶解, 周边对合良好。31/55(56.4%) 只眼形成了足够深的穹窿部且恢复了眼球运动功能。9/55(16.4%) 只眼发生部分睑球粘连, 眼球运动轻度受限,但其面积远较术前为小。15 /55(27.3%) 眼术后发生中度以上的睑球粘连。新鲜羊膜和保存羊膜重建眼结膜表面的效果相似( X 2 = 0.466, P = 0.797) ; 不同程度睑球粘连的患者其羊膜移植术后的效果不同(新鲜羊膜, X2=27.995, P=0.000; 保存羊膜, X2=33.610, P=0.000) ; 在眼表烧伤后1年内手术比 1 年以上进行羊膜移植的效果也不同(X 2= 4.243, P = 0.039) 。结论: 新鲜羊膜和保存羊膜一样可以有效地用于重建睑球粘连解除后的结膜眼表。患眼术前睑球粘连程度以及烧伤后其眼表炎症是否处于安静状态等因素都会直接影响羊膜移植重建眼结膜表面的远期疗效。
Purpose: To compare the effect of fresh versus preserved amniotic membrane transplant-ation for conjunctival surface reconstruction after symblepharon lysis and analyze the associated factors.Methods: Fifty-one consecutive cases (55 eyes) with symblepharon at different degree due to eye burns or Stevens-Johnson syndrome were accepted lysis of symblepharon and amniotic membrane transplantation. Twenty-two eyes of them were performed with fresh amnion grafts, the others (33 eyes) with preserved human amniotic membrane. Eleven eyes were performed within 1 year and forty eyes in 1 to 8 years (mean value, 2.0 ± 0.7 years) after eye burns.Results: The follow-up time varied from 12 to 32 months (mean value, 19.3 ± 4.1 months) . Fifty-six point four percent (31/55) eyes got enough deep conjunctival fornix and resolution of eye movement restrict. Sixteen percent of them (9/55) recurred less symblepharon and remained slightly eye movement restrict. Fifteen- five eyes of them (27.3%) recurred moderate symblepharon. The effects of surgery were similar between fresh and preserved AMT (X 2 = 0.466, P = 0.797) . The effects of AMT for those patients with symblepharon at different degree had significant difference statistically (fresh amnions, X 2= 27.995, P = 0.000; preserved amnions, X 2 =33.610, P = 0.000) . The same results were observed between those patients who were performed in different time periods after eye burns ( X 2 = 4.243, P = 0.039) .Conclusion: Fresh amnion has the same effect as preserved one for conjunctival surface reconstruction. The degree of symblepharon and the surgical environment of the ocular surface in the affected eye before surgery will influence the results of amniotic membrane transplantation for conjunctival surface reconstruction.
目的: 探讨小梁切除术两种不同结膜瓣术后功能性滤过泡形成情况。方法: 比较 372 例(372 只眼) 原发性青光眼不同结膜瓣小梁切除术术后功能性滤过泡形成情况, 其中以角巩缘为基底的结膜瓣 165 只眼, 以穹窿部为基底的结膜瓣 207 只眼。结果: 术后 1 周对所有患者进行了观察, 3 个月时只有 127 只眼进行了随访。以角巩缘为基底的结膜瓣手术组术后 1 周滤过泡成功率为 83.03 %, 3 个月时为 78.69 %。以穹窿部为基底的结膜瓣手术组术后 1 周滤过泡成功率为 55.07 %, 3 个月时为 53.03 %。两组比较有明显差别。结论: 本组研究发现, 传统的以角巩缘为基底的结膜瓣术后滤过泡成功率高于以穹窿部为基底的结膜瓣, 其术后降压效果相应亦较好。
Purpoes: To assess the success rate of the formation of functional filtration bleb in eyes undergoing different incision of conjunctiva flap after trabeculectomy.Methods: To study the formation of functional filtration bleb after trabeculectomy, the result of trabeculectomy with the limbus based conjunctival flap in 165 eyes and the fornix based conjunctival flap in 207 eyes (372 eyes in 372 cases with primary glaucoma in total) was compared.Results: All patients were observed after trabeculectomy in 1 week, but only 127 eyes were followed up in 3 months. In patients with trabeculectomy using the limbus based conjunctival flap, the success rate of the formation of the filtration bleb was 83.03 % in 1 week and 78.69 % in three months postoperatively, while in those using the fornix based conjunctival flap, the success rate was 55.07 % in 1 week and 53.03 % in 3 months. There was significant difference between the two groups in terms of the success rate of the formation of the filtration bleb.Conclusions: The result showed that limbus based conjunctival flap was especially effective and had better postoperative intraocular pressure decline.
目的: 探讨泪膜改变对 Goldmann 压平眼压计测量值的影响。方法: 将 68 例(136 只眼) 受检者随机分为 2 组, 每组 34 例。随机选择一只眼作为实验眼, 另一只眼作为对照眼。A 组的实验眼采用右旋糖苷 70 滴眼液滴眼, B 组的实验眼采用粘弹剂 Viscoat 滴眼。采用 Goldmann 眼压计测量 2 次眼压, 比较滴眼前、后测量值的差异。结果: A 组对照眼第 1、2 次的眼压测量值分别为(14.44±2.68) mmHg(1 mmHg = 0.133 KPa) 、(14.47 ± 2.69) mmHg, 两次眼压测量值的差异无统计学意义(t = - 0.329, P = 0.744) 。实验眼滴右旋糖苷 70 滴眼液前、后的眼压测量值分别为(14.41 ±2 .63) mmHg、(12.94 ± 2.59) mmHg, 两次眼压测量值的差异有统计学意义 (t = 13.949, P = 0.000) 。B 组对照眼第 1、2 次的眼压测量值分别为(14.29 ± 2.96) mmHg、(14.35 ± 3.12) mmHg, 两次眼压测量值的差异无统计学意义(t = - 0.466, P = 0.644) 。实验眼滴 Viscoat 前、后的眼压测量值分别为 (14.53 ± 3.13) mmHg、(11.18 ± 3.07) mmHg, 两次眼压测量值的差异有统计学意义( t = 22.126, P = 0.000) 。两组的实验眼滴眼后的眼压均呈一致性的下降。结论: 泪膜的改变可以使 Goldmann 压平眼压计的测量值产生偏差。
Purpose: To explore the effect of tear film changes on the intraocular pressure (IOP) measurement by Goldmann applantion tonometer.Methods: Sixty-eight normal subjects were randomly divided into two groups (Gruop A and B) , 34 in each group. One eye of each subject by random selection was given Dextran (in Group A) and Viscoat (in Group B) , respectively. And the fellow eyes were used as controls. IOP was measured in all subjects twice using Goldmann applantion tonometer. And the difference between first and second measurements was compared.Result: In the control eyes of Group A, the average IOP of first and second measurements were (14.44 ± 2.68) mmHg (1 mmHg= 0.133 KPa) and (14.47 ± 2.69) mmHg, there was no difference between the two measurements (t =- 0.329, P = 0.744) . In eyes given Dextran, the average IOP of two measurements were (14.41 ± 2.63) mmHg and (12.94 ± 2.59) mmHg, there was significant difference between the two mea-surements (t =13.949, P= 0.000) . In control eyes of Group B, the average IOP of first and second measurements were (14.53 ± 3.13) mmHg and (11.18 ± 3.07) mmHg, and there was no difference between the two measurements (t = -0.466, P = 0.644) . In eyes given Viscoat, the average IOP of two measurements were (14.53 ± 3.13) mmHg and (11.18 ± 3.07) mmHg, and there was significant difference between the two measurements (t = 22.126, P = 0.000) . After being given Dextran or Viscoat, the IOP values decreased consistently in both Group A and Group B.Conclusions: The change of tear film components can affect IOP values by Goldmann applantion tonometer.