Cohen Kenneth L, Bouldin Thomas W
Department of Ophthalmology, University of North Carolina at Chapel Hill, School of Medicine, Chapel Hill, NC 27599-7040, USA.
Cornea. 2002 Nov;21(8):774-7. doi: 10.1097/00003226-200211000-00008.
To describe the histopathology of familial, band-shaped, spheroidal keratopathy in ethnic Chinese siblings. New histopathologic findings are presented that may shed light on the etiology of this rare and unique condition.
Three ethnic Chinese siblings in Urumqi, People's Republic of China, were examined. One brother and sister had spheroidal, amber-colored droplets in the superficial, central corneal stroma. Each had a diffuse gray haze and thickening of the peripheral corneal stroma. A histopathologic study of penetrating keratoplasty specimens was performed. Another sister had only thickening and haze of the corneal stroma.
Typical spheroidal deposits in the superficial stroma were demonstrated. One cornea had vascularization and amyloid deposition. One cornea had loss of endothelial cells and a thickened Descemet's membrane.
Neovascularization and amyloid deposition are likely secondary phenomena in this familial disease. A new clinical finding is the hazy, thickened stroma. New pathologic findings are the loss of endothelial cells and the marked thickening of Descemet's membrane. An endothelial cell dystrophy may accompany familial, band-shaped, spheroidal keratopathy.
描述中国汉族同胞家族性带状球形角膜病变的组织病理学特征。本文呈现了新的组织病理学发现，这可能有助于阐明这种罕见且独特病症的病因。
对中华人民共和国乌鲁木齐市的三名中国汉族同胞进行了检查。一名兄弟和一名姐妹在角膜中央基质浅层有球形琥珀色液滴。两人均有弥漫性灰色混浊及周边角膜基质增厚。对穿透性角膜移植标本进行了组织病理学研究。另一名姐妹仅有角膜基质增厚和混浊。
在浅层基质中发现了典型的球形沉积物。一只角膜有血管化和淀粉样沉积。一只角膜有内皮细胞丢失和后弹力层增厚。
在这种家族性疾病中，新生血管形成和淀粉样沉积可能是继发现象。一个新的临床发现是基质混浊、增厚。新的病理学发现是内皮细胞丢失和后弹力层明显增厚。内皮细胞营养不良可能与家族性带状球形角膜病变伴发。
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