Confocal laser-scanning microscopy allows differentiation between Fabry disease and amiodarone-induced keratopathy
Confocal laser-scanning microscopy allows differentiation between Fabry disease and amiodarone-induced keratopathy
复制标题
DOI：
10.1007/s00417-011-1726-5
复制
发表时间：
2011-11-01
影响因子：
2.7
通讯作者：
Pitz, Susanne
中科院分区：
文献类型：
--
作者：
Wasielica-Poslednik, Joanna;Pfeiffer, Norbert;Pitz, Susanne
应用共聚焦激光扫描显微镜（CLSM）对10例Fabry病患者的10只眼、6例胺碘酮性角膜病变患者的6只眼和8例健康对照者的眼轮状角膜进行了显微结构的观察。1例法布里病患者接受胺碘酮治疗。所有Fabry患者均接受半乳糖苷酶α的酶替代治疗，10例Fabry患者中有7例和所有接受胺碘酮治疗的患者在裂隙灯检查中显示典型的角膜轮状。CLSM显示，在所有患有角膜轮状的法布里病患者和1例没有裂隙灯可检测到的旋涡性角膜病变的法布里病患者以及所有患有胺碘酮角膜病变的眼睛的基底上皮细胞中存在高反射细胞内包涵体。胺碘酮沉积物反射性更强，大小明显不同。7例法布里病患者和所有胺碘酮患者有间质微粒。2例胺碘酮患者在内皮中显示胺碘酮包涵体。法布里病患者的CLSM变化的数量与裂隙灯可检测到的角膜轮状细胞没有相关性，而法布里引起的角膜轮状细胞和胺碘酮角膜病变不能通过传统的裂隙灯显微镜区分，CLSM允许在大多数患者中区分两种病因。CLSM显示角膜变化之前，裂隙灯显微镜检测角膜轮状，因此可能有助于法布里病的早期诊断。CLSM不允许定量监测酶替代疗法下法布里病患者的角膜变化。
The aim of this work is to compare the microstructure of cornea verticillata in Fabry disease with amiodarone-induced keratopathy by means of in vivo confocal laser-scanning microscopy (CLSM).Ten eyes of ten patients suffering from Fabry disease, six eyes of six patients with amiodarone-induced keratopathy and eight eyes of healthy control subjects were examined by conventional slit-lamp microscopy and CLSM. One Fabry patient received amiodarone therapy. All Fabry patients were under enzyme replacement therapy with agalsidase alfa.Seven out of ten Fabry patients and all patients receiving amiodarone showed typical cornea verticillata on slit-lamp examination. CLSM revealed hyper-reflective intracellular inclusions in basal epithelial cells of all Fabry patients with cornea verticillata and in one Fabry patient without slit-lamp-detectable vortex keratopathy, as well as in all eyes featuring amiodarone keratopathy. Amiodarone deposits were more reflective and of grossly different size. Seven Fabry patients and all amiodarone patients had stromal microdots. Two amiodarone patients showed amiodarone inclusions in the endothelium. The number of CLSM changes in Fabry patients did not correlate with that of slit-lamp detectable cornea verticillata.While Fabry-induced cornea verticillata and amiodarone keratopathy cannot be distinguished by conventional slit-lamp microscopy, CLSM allows the differentiation between both etiologies in the majority of patients. CLSM appears to reveal corneal changes prior to the detection of cornea verticillata on slit-lamp microscopy and may thus be helpful in the early diagnosis of Fabry disease. CLSM does not allow quantitative monitoring of corneal changes in Fabry patients under enzyme-replacement therapy.