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[富克斯内皮性角膜营养不良的发病机制、纤维层及个体化治疗]

[Pathogenesis of Fuchs endothelial corneal dystrophy, the fibrillar layer and individualized treatment].

作者信息

Howaldt Antonia, Clahsen Thomas, Mestanoglu Mert, Odenthal Margarete, Tahmaz Volkan, Cursiefen Claus, Matthaei Mario

机构信息

Department of Ophthalmology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Kerpener Str. 62, 50937, Köln, Deutschland.

Center for Molecular Medicine Cologne (CMMC), Faculty of Medicine and University Hospital Cologne, University of Cologne, Köln, Deutschland.

出版信息

Ophthalmologie. 2024 Oct;121(10):787-795. doi: 10.1007/s00347-024-02123-4. Epub 2024 Oct 1.

Abstract

Fuchs endothelial corneal dystrophy (FECD) is a genetic and age-associated corneal disease characterized by an accelerated loss of corneal endothelial cells and an increased subendothelial deposition of extracellular matrix (ECM). Clinically, advanced disease leads to corneal edema with subsequent reduction in visual acuity. In the majority of patients with advanced FECD, a fibrillar layer (FL) appears on the posterior corneal surface. This FL is mostly localized in the inferotemporal corneal quadrant, marks areas with significantly reduced endothelial cell density and increased corneal thickness in the sense of edema and can be visualized and measured using Scheimpflug backscatter analysis due to increased backscatter. FECD is currently the most common indication for corneal transplantation worldwide, usually in the form of Descemet membrane endothelial keratoplasty (DMEK). New treatment approaches include variations of DMEK surgery such as hemi- or quarter DMEK with individualized and smaller grafts or Descemet membrane stripping only (DSO). In the future, clinical imaging of the FL as a particularly affected endothelial area could be important for FECD progression assessment and planning of surgical interventions. This article provides an overview of the current state of research on the clinical aspects, pathogenesis, fibrillar layer and individualized treatment of FECD.

摘要

富克斯内皮性角膜营养不良(FECD)是一种与遗传和年龄相关的角膜疾病,其特征是角膜内皮细胞加速丧失以及细胞外基质(ECM)在内皮细胞下的沉积增加。临床上,晚期疾病会导致角膜水肿,进而导致视力下降。在大多数晚期FECD患者中,角膜后表面会出现一个纤维层(FL)。这个FL大多位于角膜颞下象限,标志着内皮细胞密度显著降低且角膜因水肿而厚度增加的区域,并且由于反向散射增加,可使用Scheimpflug反向散射分析进行可视化和测量。FECD目前是全球角膜移植最常见的适应症,通常采用Descemet膜内皮角膜移植术(DMEK)的形式。新的治疗方法包括DMEK手术的变体,如半量或四分之一量DMEK,采用个体化且更小的移植物,或仅进行Descemet膜剥离(DSO)。未来,作为特别受影响的内皮区域的FL的临床成像对于FECD进展评估和手术干预规划可能很重要。本文概述了FECD在临床方面、发病机制、纤维层和个体化治疗的研究现状。

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