施奈德角膜营养不良脂质沉积的定量分析。
Quantitative analysis of lipid deposits from Schnyder's corneal dystrophy.
作者信息
Yamada M, Mochizuki H, Kamata Y, Nakamura Y, Mashima Y
机构信息
Department of Ophthalmology, Keio University School of Medicine, Tokyo, Japan.
出版信息
Br J Ophthalmol. 1998 Apr;82(4):444-7. doi: 10.1136/bjo.82.4.444.
Abstract
AIM
To report the quantitation of the lipid composition of a corneal button from a Japanese woman in her 60s with clinically and histopathologically proved Schnyder's corneal dystrophy.
METHODS
Total lipids extracted from the corneal button of the patient were analysed by the method of thin layer chromatography flame ionisation detection. Two different solvent systems were used for neutral lipid analysis and phospholipid analysis. Results were compared with three age matched corneal buttons obtained from cadaveric eyes.
RESULTS
The lipids that accumulated in the cornea in Schnyder's dystrophy consisted mainly of unesterified cholesterol and phospholipids. The analysis of phospholipids showed sphingomyelin to be the predominant phospholipid in the patient's cornea.
CONCLUSION
Findings suggest that this disorder involves a disturbance of the metabolism of cholesterol and/or sphingomyelin metabolism that is limited to the cornea.
摘要
目的
报告一名60多岁日本女性角膜植片脂质成分的定量分析结果，该患者临床及组织病理学诊断为施奈德角膜营养不良。
方法
采用薄层色谱火焰离子化检测法分析从患者角膜植片中提取的总脂质。使用两种不同的溶剂系统分别进行中性脂质分析和磷脂分析。将结果与从尸体眼睛获取的三个年龄匹配的角膜植片进行比较。
结果
施奈德营养不良症中角膜蓄积的脂质主要由未酯化胆固醇和磷脂组成。磷脂分析显示，鞘磷脂是患者角膜中的主要磷脂。
结论
研究结果表明，这种疾病涉及仅限于角膜的胆固醇代谢和/或鞘磷脂代谢紊乱。
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Panstromal Schnyder corneal dystrophy. A clinical pathologic report with quantitative analysis of corneal lipid composition.全基质性施奈德角膜营养不良。一份角膜脂质成分定量分析的临床病理报告。
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Crystalline stromal dystrophy: histochemistry and ultrastructure of the cornea.结晶性角膜基质营养不良：角膜的组织化学与超微结构
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