Pietro Mangiantini, Fabiana Mallone, Mattia D'Andrea, Lorenzo Albanesi, Luca Lucchino, Luca Celli, Mauro Celli, Alessandro Lambiase, Antonietta Moramarco
{"title":"成骨不全患者的角膜改变：一项体内角膜共聚焦显微镜研究。","authors":"Pietro Mangiantini, Fabiana Mallone, Mattia D'Andrea, Lorenzo Albanesi, Luca Lucchino, Luca Celli, Mauro Celli, Alessandro Lambiase, Antonietta Moramarco","doi":"10.2147/OPTH.S470183","DOIUrl":null,"url":null,"abstract":"<p><strong>Purpose: </strong>Osteogenesis imperfecta (OI) is a rare hereditary disorder of the connective tissue. Despite recent attention to corneal abnormalities in OI, understanding remains limited. This study aimed to comprehensively evaluate corneal changes in a large sample of OI patients compared to controls using in vivo confocal microscopy (IVCM).</p><p><strong>Patients and methods: </strong>Nineteen OI patients (mean age: 34.0 ± 16.00 years; 9 females, 10 males) and 20 healthy controls (mean age: 35.5 ± 12.00; 12 females, 8 males) were included, matched for age and gender. The integrity of corneal cell layers, with a focus on Bowman's layer and sub-epithelial stroma, was evaluated. Additionally, we conducted a quantitative analysis of the corneal sub-basal nerve plexus (CSNP), measuring nerve fiber density (NFD), nerve branch density (NBD), nerve fiber length (NFL), and dendritic cells (DCs) density. Clinical parameters including blue discoloration of the sclera, corneal thickness and sensitivity were also evaluated.</p><p><strong>Results: </strong>Bowman's layer alterations were observed in 42.11% of OI patients. NFD was significantly reduced in OI patients (27,3±6.98 vs controls 37.85±13,74 fiber/mm2; p-value=0.005). NBD and NFL were lower in OI patients but did not reach statistical significance (p=0.650 and p=0.120, respectively). DCs density was higher in OI patients than controls (11,37 ± 12.79 vs 2.09±2,91 cells/mm2; p-value < 0.001). Corneal thickness and sensitivity were significantly reduced in OI patients compared to controls (p<0.001, p=0.001, respectively). OI patients with blue sclera or abnormal Bowman's layer exhibited even lower central corneal thickness (CCT) (p=0.010, p=0.005, respectively).</p><p><strong>Conclusion: </strong>OI patients demonstrated Bowman's layer abnormalities, neuropathic changes and higher inflammatory cell count. These results suggest potential corneal complications, and hold promise for diagnostic applications and intervention strategies in OI.</p>","PeriodicalId":93945,"journal":{"name":"Clinical ophthalmology (Auckland, N.Z.)","volume":"18 ","pages":"3977-3988"},"PeriodicalIF":0.0000,"publicationDate":"2024-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11687199/pdf/","citationCount":"0","resultStr":"{\"title\":\"Corneal Alterations in Patients with Osteogenesis Imperfecta: An in vivo Corneal Confocal Microscopy Study.\",\"authors\":\"Pietro Mangiantini, Fabiana Mallone, Mattia D'Andrea, Lorenzo Albanesi, Luca Lucchino, Luca Celli, Mauro Celli, Alessandro Lambiase, Antonietta Moramarco\",\"doi\":\"10.2147/OPTH.S470183\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Purpose: </strong>Osteogenesis imperfecta (OI) is a rare hereditary disorder of the connective tissue. Despite recent attention to corneal abnormalities in OI, understanding remains limited. This study aimed to comprehensively evaluate corneal changes in a large sample of OI patients compared to controls using in vivo confocal microscopy (IVCM).</p><p><strong>Patients and methods: </strong>Nineteen OI patients (mean age: 34.0 ± 16.00 years; 9 females, 10 males) and 20 healthy controls (mean age: 35.5 ± 12.00; 12 females, 8 males) were included, matched for age and gender. The integrity of corneal cell layers, with a focus on Bowman's layer and sub-epithelial stroma, was evaluated. Additionally, we conducted a quantitative analysis of the corneal sub-basal nerve plexus (CSNP), measuring nerve fiber density (NFD), nerve branch density (NBD), nerve fiber length (NFL), and dendritic cells (DCs) density. Clinical parameters including blue discoloration of the sclera, corneal thickness and sensitivity were also evaluated.</p><p><strong>Results: </strong>Bowman's layer alterations were observed in 42.11% of OI patients. NFD was significantly reduced in OI patients (27,3±6.98 vs controls 37.85±13,74 fiber/mm2; p-value=0.005). NBD and NFL were lower in OI patients but did not reach statistical significance (p=0.650 and p=0.120, respectively). DCs density was higher in OI patients than controls (11,37 ± 12.79 vs 2.09±2,91 cells/mm2; p-value < 0.001). Corneal thickness and sensitivity were significantly reduced in OI patients compared to controls (p<0.001, p=0.001, respectively). OI patients with blue sclera or abnormal Bowman's layer exhibited even lower central corneal thickness (CCT) (p=0.010, p=0.005, respectively).</p><p><strong>Conclusion: </strong>OI patients demonstrated Bowman's layer abnormalities, neuropathic changes and higher inflammatory cell count. These results suggest potential corneal complications, and hold promise for diagnostic applications and intervention strategies in OI.</p>\",\"PeriodicalId\":93945,\"journal\":{\"name\":\"Clinical ophthalmology (Auckland, N.Z.)\",\"volume\":\"18 \",\"pages\":\"3977-3988\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-12-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11687199/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Clinical ophthalmology (Auckland, N.Z.)\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.2147/OPTH.S470183\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical ophthalmology (Auckland, N.Z.)","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.2147/OPTH.S470183","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/1/1 0:00:00","PubModel":"eCollection","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
摘要
目的：成骨不全症是一种罕见的结缔组织遗传性疾病。尽管最近对成骨不全患者的角膜异常有所关注，但了解仍然有限。本研究旨在利用体内共聚焦显微镜（IVCM）全面评估与对照组相比，成骨不全患者大样本的角膜变化。患者与方法：成骨不全患者19例(平均年龄34.0±16.00岁；女性9名，男性10名)，健康对照20名(平均年龄：35.5±12.00；包括12名女性，8名男性)，年龄和性别相匹配。评估角膜细胞层的完整性，重点是鲍曼层和亚上皮间质。此外，我们对角膜基底下神经丛（CSNP）进行了定量分析，测量了神经纤维密度（NFD）、神经分支密度（NBD）、神经纤维长度（NFL）和树突状细胞（DCs）密度。临床参数包括巩膜蓝色变色，角膜厚度和敏感性也进行了评估。结果：42.11%的成骨不全患者出现鲍曼层改变。成骨不全患者的NFD显著降低(27.3±6.98 vs对照组37.85±13.74纤维/mm2；假定值= 0.005)。NBD、NFL在成骨不全患者中较低，但无统计学意义（p=0.650、p=0.120）。成骨不全患者的dc密度高于对照组(11.37±12.79 vs 2.09±2.91细胞/mm2；p值< 0.001)。与对照组相比，成骨不全患者的角膜厚度和敏感性显著降低(结论：成骨不全患者表现出鲍曼层异常、神经性改变和较高的炎症细胞计数。这些结果提示了潜在的角膜并发症，并为成骨不全的诊断应用和干预策略提供了希望。
Corneal Alterations in Patients with Osteogenesis Imperfecta: An in vivo Corneal Confocal Microscopy Study.
Purpose: Osteogenesis imperfecta (OI) is a rare hereditary disorder of the connective tissue. Despite recent attention to corneal abnormalities in OI, understanding remains limited. This study aimed to comprehensively evaluate corneal changes in a large sample of OI patients compared to controls using in vivo confocal microscopy (IVCM).
Patients and methods: Nineteen OI patients (mean age: 34.0 ± 16.00 years; 9 females, 10 males) and 20 healthy controls (mean age: 35.5 ± 12.00; 12 females, 8 males) were included, matched for age and gender. The integrity of corneal cell layers, with a focus on Bowman's layer and sub-epithelial stroma, was evaluated. Additionally, we conducted a quantitative analysis of the corneal sub-basal nerve plexus (CSNP), measuring nerve fiber density (NFD), nerve branch density (NBD), nerve fiber length (NFL), and dendritic cells (DCs) density. Clinical parameters including blue discoloration of the sclera, corneal thickness and sensitivity were also evaluated.
Results: Bowman's layer alterations were observed in 42.11% of OI patients. NFD was significantly reduced in OI patients (27,3±6.98 vs controls 37.85±13,74 fiber/mm2; p-value=0.005). NBD and NFL were lower in OI patients but did not reach statistical significance (p=0.650 and p=0.120, respectively). DCs density was higher in OI patients than controls (11,37 ± 12.79 vs 2.09±2,91 cells/mm2; p-value < 0.001). Corneal thickness and sensitivity were significantly reduced in OI patients compared to controls (p<0.001, p=0.001, respectively). OI patients with blue sclera or abnormal Bowman's layer exhibited even lower central corneal thickness (CCT) (p=0.010, p=0.005, respectively).
Conclusion: OI patients demonstrated Bowman's layer abnormalities, neuropathic changes and higher inflammatory cell count. These results suggest potential corneal complications, and hold promise for diagnostic applications and intervention strategies in OI.
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
