Collier L L, Prieur D J, King E J
Curr Eye Res. 1984 Oct;3(10):1241-51. doi: 10.3109/02713688409000828.
The Chediak-Higashi syndrome (CHS) is a hereditary disorder of man, with the homologous condition reported in five animal species. Multiple defects, including oculocutaneous hypopigmentation, are present in individuals with this syndrome. Giant cytoplasmic granules, including melanosomes and lysosomes, are characteristic. In this study, eyes from CHS affected and control cats, cattle, mink, and mice were examined histologically to determine: 1) degree of pigmentation; 2) structure and distribution of melanin granules; and 3) morphology of cells and tissues containing melanin. The CHS cattle were found to be the most ocularly hypopigmented species, whereas CHS mouse eyes contained considerably more melanin than those of the other species. Melanin granules of abnormal sizes and shapes were present in neuroepithelial and uveal tissues of CHS animals of all four species. Depigmentation apparently had occurred in the CHS eyes, since less melanin was present in eyes of old CHS animals of each species than was present in eyes of young animals. In addition, melanin containing macrophages were common in CHS eyes, and the numbers of melanocytes and pigmented epithelial cells were decreased in older CHS eyes.
切迪阿克-希加什综合征（CHS）是一种人类遗传性疾病，在五种动物物种中也报道了类似病症。患有这种综合征的个体存在多种缺陷，包括眼皮肤色素减退。巨大的细胞质颗粒，包括黑素小体和溶酶体，是其特征。在本研究中，对患有CHS的猫、牛、水貂和小鼠以及对照动物的眼睛进行了组织学检查，以确定：1）色素沉着程度；2）黑色素颗粒的结构和分布；3）含黑色素的细胞和组织的形态。发现CHS牛是眼部色素减退最严重的物种，而CHS小鼠眼睛中的黑色素比其他物种的眼睛多得多。在所有四个物种的CHS动物的神经上皮和葡萄膜组织中都存在大小和形状异常的黑色素颗粒。CHS眼睛显然发生了色素脱失，因为每个物种的老年CHS动物眼睛中的黑色素比幼年动物眼睛中的少。此外，含黑色素的巨噬细胞在CHS眼睛中很常见，并且在老年CHS眼睛中黑素细胞和色素上皮细胞的数量减少。
