视神经脊髓炎谱系疾病（neuromyelitis optica spectrum disorder, NMOSD）是一种罕见的中枢神经系统自身免疫性疾病，主要表现为视神经炎和纵向广泛性横贯性脊髓炎，复发率高，反复发作可导致不可逆的神经功能损害。文章报告了一例44岁女性的NMOSD患者，以反复发作的视神经炎为特征，经过长达11年的随访，共历经4次临床发作。患者首次发作时，因治疗过程中糖皮质（激素）减量过快和自行停药导致病情恶化，后续发作中采用了规范的急性期治疗和缓解期预防复发方案，包括大剂量激素冲击、免疫抑制剂（硫唑嘌呤、吗替麦考酚酯）和静脉注射免疫球蛋白（intravenous immunoglobulin, IVIG）等，显著延长了缓解期。最近一次复发中，患者首次使用了新型生物制剂伊奈丽珠单抗（CD19单克隆抗体）预防复发治疗。文章通过具体病例分析和文献综述，反映NMOSD急性期和缓解期预防复发治疗策略的时代变迁，并探索新型生物制剂使用过程中需要注意的问题。
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease that affects the central nervous system. It is predominantly characterized by optic neuritis and longitudinally extensive transverse myelitis. Given its high relapse rate, repeated attacks of NMOSD can result in irreversible nerve damage. The article details the case of a 44-year-old female patient with NMOSD, whose primary manifestion was recurrent optic neuritis.The patient was followed up over an 11-year span, during which she experienced a total of four clinical relapses. During the initial attack, the patient's condition deteriorated because of the rapid tapering of steroid therapy and her self-initiated discontinuation of medication. In subsequent attacks, standardized treatments for the acute phase and strategies for preventing relapses were implemented. These included high-dose steroid pulses, immunosuppressants (azathioprine, mycophenolate mofetil), and intravenous immunoglobulin (IVIG). These interventions significantly prolonged the remission period. During the most recent relapse, the patient received treatment with the novel biologic agent inebilizumab, which is a CD19 monoclonal antibody. Through a comprehensive case analysis and a review of relevant literature , this report sheds light on the evolving treatment strategies for managing the acute phase and preventing relapses during remission in NMOSD. It also highlights key considerations in the practical application of the new biologic therapies.
目的：观察和评价改良联合筋膜鞘(combined fascial sheath，CFS)悬吊术治疗复发性上睑下垂的可行性和临床效果。方法：纳入2017年3月至2020年3月于河北省眼科医院眼整形门诊就诊的26例复发性重度上睑下垂患者(32只眼)，均采用改良CFS悬吊术予以矫正。术后随访1年，分别于术后1个月、3个月、6个月及1年时复诊，对患者术后上睑缘中点到角膜反光点的距离(marginal reflex distance-1，MRD1)、眼皮折皱深度、长度、弧度和并发症等情况为主要观察指标给予记录和效果评估。结果：26例患者共32只患眼，临床治愈27只眼(84.4%)，改善4只眼(12.5%)，无效1只眼(3.1%)，对1只无效眼术后6个月进行再次调整，调整后达到临床治愈标准。1例出现结膜脱垂，经过药物与加压包扎治疗后结膜脱垂症状消失，无暴露性角膜炎或其他并发症发生病例。结论：改良CFS悬吊术矫正复发性重度上睑下垂，具有操作简单、创伤小、治疗效果确切、并发症少等优点，值得临床推广与应用。
Objective: To observe and evaluate the feasibility and clinical effect of modified combined fascial sheath (CFS) suspension on the treatment of recurrent severe blepharoptosis. Methods: A total of 26 patients (32 eyes) with recurrent severe ptosis treated in Hebei Eye Hospital from March 2017 to March 2020 were included. All patients were corrected by modified CFS suspension. The patients were followed up for 1 year, and returned to the hospital at 1 month, 3 months, 6 months and 1 year respectively. The marginal reflex distance-1 (MRD1), eyelid crease depth, length, radian, and complications were recorded as the main observation indexes and the effect was evaluated. Results: In the 32 eyes of 26 patients, 27 eyes (84.4%) were completely corrected, 4 eyes (12.5%) were improved, while 1 eye (3.1%) had no improvement, the invalid eye was adjusted again 6 months after operation, and reached the effective standard after adjustment. Conjunctival prolapse occurred in 1 case, and the symptoms of conjunctival prolapse disappeared after the treatment with drugs and pressure dressing, and no case of exposure keratitis or other complications occurred. Conclusion: Modified combined fascial sheath suspension is an effective method on the treatment of recurrent severe blepharoptosis with the advantages of simple operation, less trauma, definite therapeutic effect, and fewer complications, which is worthy of clinical promotion and application.
目的：分析复发与初发睑板腺囊肿患者的睑板腺组织形态学改变在活体共聚焦显微镜(in vivo confocal microscope，IVCM)下的表现及特点。方法：采用横断面研究方法，选取2018年10月至2019年4月在汕头大学·香港中文大学联合汕头国际眼科中心门诊就诊的10例复发性睑板腺囊肿患者、10例初发性睑板腺囊肿以及10例对照组作为观察对象。所有对象行眼科常规检查及IVCM检查。IVCM检测指标包括睑板腺开口面积、开口最短径、开口最长径、睑板腺开口附近腺管形态、睑板腺腺泡样结构形态，分析比较三组的计量指标。结果：复发性睑板腺囊肿组睑板腺开口短径(109.08±49.96) μm，开口长径(144.95±68.10) μm，开口面积为11 621.62 (3 976.49~24 828.82) μm2 ；初发性睑板腺囊肿组睑板腺开口短径(101.53±29.55) μm，开口长径(130.08±45.21) μm，开口面积10 615.07(5 813.29~18 275.44) μm2 ；对照组睑板腺开口短径(44.14±14.37) μm，开口长径(55.98±13.46) μm，开口面积2 233.29(1 437.72~2 945.65) μm2 。与对照组相比，复发性、初发性睑板腺囊肿组睑板腺开口短径、开口长径及开口面积均明显扩大，差异有统计学意义(P<0.05)；复发与初发睑板腺囊肿组之间差异不具有统计学意义(P>0.05)。复发性睑板腺囊肿组睑板腺腺管扩张，周边腺泡样结构纤维组织增生，伴有炎症细胞浸润。初发性睑板腺囊肿组睑板腺腺管扩张，周边腺泡样结构未见明显纤维组织增生。结论：IVCM可在活体下观察睑板腺囊肿患者睑板腺形态学上的微观改变，复发性睑板腺囊肿睑板腺腺泡样结构形态与初发性睑板腺囊肿表现有差异。
Background: To analyze the morphological changes of meibomian glands in patients with recurrent and primary meibomian gland cyst under in vivo confocal microscope (IVCM). Methods: A cross-sectional study was performed in Shantou International Eye Center from September 2018 to April 2019. Ten patients with recurrent meibomian cyst, 10 patients with primary meibomian cyst and 10 control subjects were selected in this clinical trial. All subjects received routine ophthalmologic examination and IVCM examination. IVCM examination parameters included open area of meibomian gland, and the maximum and minimum diameter of meibomian gland opening. Relevant parameters were analyzed and statistically compared among different groups. Results: The average minimum diameter of meibomian glands opening in the recurrent meibomian gland cyst group was (109.08±49.96) μm, the average maximum diameter of meibomian glands opening was (144.95±68.10) μm, and the median open area of meibomian gland was 11 621.62 (3 976.49–24 828.82) μm2 . In the primary meibomian gland cyst group, the average minimum diameter of meibomian glands opening was (101.53±29.55) μm, the average maximum diameter of meibomian glands opening was (130.08±45.21) μm, and the median open area of meibomian gland was 10 615.07 (5 813.29–18 275.44) μm2 . The opening of meibomian glands in both the recurrent and primary meibomian gland cyst groups was enlarged, which significantly differed from that in the control group (both P<0.05). No statistical significance was noted between the recurrent and primary meibomian gland cyst groups (P>0.05). The acinus structure around the gland tube was manifested with serious hypertrophic scar complicated with inflammatory cell infiltration. Conclusion: IVCM can detect the morphological changes of meibomian glands in meibomian gland cyst patients. The IVCM findings of recurrent and primary meibomian gland cyst are different.
目的：探讨血管生成拟态(vasculogenic mimicry，VM)与翼状胬肉初发型及复发型的相关性。方法：采用血小板-内皮细胞黏附分子/过碘酸雪夫(platelet endothelial cell adhesion molecule-1/periodic acid-schiff，CD31/PAS)免疫组织化学双重染色法检测139例翼状胬肉组织(初发型105例；复发型34例)和10例正常结膜中VM的表达，分析VM与初发型及复发型翼状胬肉的相关性及其与患者性别、年龄等因素的关系。原代培养人翼状胬肉成纤维细胞(human pterygium fibroblasts，HPFs)，免疫细胞化学染色法鉴定，利用三维培养及PAS染色观察初发型和复发型HPFs构成VM管腔个数的差异。结果：10例正常结膜均未见VM结构，初发型翼状胬肉VM阳性率43.81%，复发型翼状胬肉VM阳性率82.35%，差异具有统计学意义(P<0.001)。相关性分析显示VM与复发型翼状胬肉呈显著正相关(r=0.332)。不同性别、年龄及病程的翼状胬肉患者VM的表达差异均无统计学意义(均P>0.05)。原代培养的HPFs Vimentin表达阳性，符合成纤维细胞特性。细胞三维培养及PAS染色结果提示HPFs具有构建体外VM模型的能力，且复发型HPFs构成的VM管腔数明显高于初发型，差异具有统计学意义(P<0.01)。结论：翼状胬肉组织中存在VM结构，可作为其血供途径之一。VM与翼状胬肉的复发具有密切关系。
Objective: The purpose of this study was to investigate the correlation of vasculogenic mimicry in the primary and recurrent pterygium. Methods: Platelet endothelial cell adhesion molecule-1/periodic acid-schiff (CD31/PAS)immunohistochemical double staining method was adopted to detect the expression of VM in 139 cases of pterygium (105 cases of primary pterygium and 34 cases of recurrent pterygium)and 10 cases of normal conjunctival tissues. The correlation between VM and primary pterygium, recurrent pterygium and the factors such as gender and age of patients were analyzed. Human pterygium fibroblasts (HPFs) were primary cultured and identified by immunocytochemical staining. The differences in the number of VM channels between primary HPFs and recurrent HPFs were observed by three-dimensional culture and PAS staining. Results: There was no VM structure in 10 normal conjunctiva and the positive rate of VM was 43.81% in primary pterygium and 82.35% in recurrent pterygium with a significantly difference (P<0.001). Correlation analysis showed a significant positive correlation between VM and recurrent pterygium (r=0.332). There was no significant difference in the expression of VM in pterygium patients with different sex, age and course (all P>0.05). Vimentin was positive in the primary cultured cells, which was consistent with the characteristics of fibroblasts. The results of three-dimensional culture and PAS staining indicated that HPFs had the ability to construct VM model in vitro, and the number of VM channels constituted by recurrent HPFs was significantly higher than that by primary HPFs, the difference was statistically significant (P<0.01). Conclusion: VM exists in pterygium tissues, and it can be used as one of the blood supply routes, which is closely related to the recurrence of pterygium.
