组织细胞增生症X是一种病因未明的组织细胞异常增殖及类瘤型病变,韩-薛-柯氏综合症是其中的一型,此型与眼科之关系较为密切。其特征为含有多量胆固醇的泡沫细胞增生,由于病变最常累及垂体区颅底骨、眶骨、上下颌骨等,故又称为头颅黄色瘤。临床上以骨质缺损、眼球突出、尿崩为主征的综合征候群。国内关于这方而资料甚少,我院近25年经眼科、儿科和内科共收治韩-薛-柯氏综合症14例,现报导于下。 Histopathosis X is an etiology of unknown abnormal proliferation of tissue cells and tumor-like lesions, Han - Xue - Koch’s syndrome is one type, this type is closely related with ophthalmology. It is characterized by foam cells containing large amounts of cholesterol hyperplasia, the lesions most commonly involved the pituitary region skull base bone, orbital bone, upper and lower jaw, it is also known as the head of the yellow tumor. Clinically, bone defects, prominent eye, diabetes insipidus sign syndrome. Domestic little information on this side, our hospital nearly 25 years by ophthalmology, pediatrics and internal medicine were treated Han - Xue - Koch’s syndrome in 14 cases, are reported below.