Hurler Syndromeの角膜組織所見について

Histo-pathological Study on the Cornea of Hurler Syndrome

I．緒言

　Hurler syndromeは酸性ムコ多糖類の代謝異常によると考えられ，この疾患に角膜の混濁は必発の症状といわれている。われわれはこの疾患の兄弟例を経験し，その1例に角膜表層移植を行ない，得られた角膜を組織学的に検索した。

A brother case of Hurler syndrome was reported. The elder's corneas obtained by the la-mellar keratoplasty were histologically exami-ned.

Light microscopic observation showed meta-chromasia positive granules in keratocytes.Electron microscopy revealed that almost all the cellular elements such as keratocytes, epi-thelial cells and infiltrating cells in the Bow-man's membrane, were vacuolated, and that in these vacuoles there were substances which were variable in shape and in electron density.