Usui T, Sawaguchi S, Abe H, Iwata K, Oyanagi K
Department of Ophthalmology, Niigata University School of Medicine, Japan.
Br J Ophthalmol. 1993 Mar;77(3):165-7. doi: 10.1136/bjo.77.3.165.
Conjunctival biopsy was performed in two siblings with adult-form galactosialidosis. Electron microscopically, several types of intracytoplasmic inclusion were observed in the fibroblasts in conjunctival stroma, lymphatic capillary endothelial cells, Schwann cells, and epithelial cells. Membrane-bound vesicles with fibrillogranular content were frequently observed, and occasional lamellar structures were noted in these inclusions. Dense granular inclusions and oil droplets were also seen. Dense granular inclusions have not been reported in this disease previously.
对两名患有成人型半乳糖唾液酸贮积症的兄弟姐妹进行了结膜活检。电子显微镜下,在结膜基质中的成纤维细胞、淋巴管内皮细胞、施万细胞和上皮细胞中观察到几种类型的胞质内包涵体。经常观察到含有纤维颗粒内容物的膜结合囊泡,并且在这些包涵体中偶尔会注意到层状结构。还可见致密颗粒状包涵体和油滴。致密颗粒状包涵体在该疾病中以前尚未见报道。