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马凡综合征的眼部表现:角膜生物力学特性与全身评分增加相关。

Ocular manifestation in Marfan syndrome: corneal biomechanical properties relate to increased systemic score points.

作者信息

Scheibenberger Dido, Frings Andreas, Steinberg Johannes, Schüler Helke, Druchkiv Vasyl, Katz Toam, von Kodolitsch Yskert, Linke Stephan

机构信息

Department of Ophthalmology, University Hospital Hamburg-Eppendorf, Hamburg, Germany.

University of Hamburg, Medical School, University Hospital Hamburg-Eppendorf, Hamburg, Germany.

出版信息

Graefes Arch Clin Exp Ophthalmol. 2018 Jun;256(6):1159-1163. doi: 10.1007/s00417-018-3946-4. Epub 2018 Mar 10.

DOI:10.1007/s00417-018-3946-4
PMID:29525839
Abstract

PURPOSE

To evaluate corneal deformation to an air puff as a new noninvasive tool to document disease status in Marfan syndrome (MFS) METHODS: Prospective observational cohort study. We included patients diagnosed with MFS who had their routine cardiovascular follow-up and applied the revised Ghent nosology to define two subgroups according to a high (≥ 7) and a low (< 7 points) systemic score. Dynamic Scheimpflug-based biomechanical analyses (CorvisST® [CST; Oculus GmbH]) were performed. The main outcome measure was the displacement of the corneal apex as given by the parameters highest concavity (HC; in ms), peak distance (PD; in mm), and highest concavity deformation amplitude (DA; mm).

RESULTS

Forty-three eyes of 43 individuals (19 female, 24 male; mean age 42.0 ± 12.0 years, range 18-67 years) diagnosed with MFS were included. Applying the Ghent criteria, 21 patients had an advanced systemic score of ≥ 7, and 22 had score points < 7. There were no differences in age or sex between both groups. In contrast, HC was faster (P = 0.004), and PD (P < 0.001) was longer in those individuals with systemic score ≥ 7; maximum DA did not result in a statistically significant difference between the groups (P = 0.250).

CONCLUSIONS

In vivo noninvasive biomechanical analyses with CST offer a new, non-invasive method to identify pathologic corneal deformation responses in adults with MFS. In the future, corneal deformation to an air puff could thus assist early identification of patients with high Ghent score as an adjunct to existing diagnostic tests.

摘要

目的

评估对气流冲击的角膜变形情况,作为记录马凡综合征(MFS)疾病状态的一种新型非侵入性工具。方法:前瞻性观察队列研究。我们纳入了被诊断为MFS且进行常规心血管随访的患者,并应用修订后的根特分类法根据高(≥7)和低(<7分)全身评分定义两个亚组。进行了基于动态Scheimpflug的生物力学分析(CorvisST®[CST;Oculus GmbH])。主要结局指标是角膜顶点的位移,由最高凹陷度(HC;以毫秒为单位)、峰值距离(PD;以毫米为单位)和最高凹陷度变形幅度(DA;毫米)等参数给出。

结果

纳入了43例被诊断为MFS的个体的43只眼(19例女性,24例男性;平均年龄42.0±12.0岁,范围18 - 67岁)。应用根特标准,21例患者的全身评分≥7为晚期,22例患者的评分<7分。两组之间在年龄或性别上没有差异。相比之下,全身评分≥7的个体中,HC更快(P = 0.004),PD更长(P < 0.001);最大DA在两组之间没有统计学上的显著差异(P = 0.250)。

结论

使用CST进行的体内非侵入性生物力学分析提供了一种新的非侵入性方法,用于识别成年MFS患者的病理性角膜变形反应。未来,气流冲击引起的角膜变形因此可以辅助早期识别高根特评分的患者,作为现有诊断测试的补充。

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