尼曼匹克氏病一例报告

• 作 者: 郭植材 庄礼昭
• 出 处: 广西医学院学报
• 发表日期: 2004年期
• 关键词: 尼曼 克氏 脂类代谢障碍 网状内皮细胞 网状内皮系统 鞘磷脂 族性 神经系统 血红蛋白测定 立方毫米

尼曼匹克氏病为一种先天性、家族性脂类代谢障碍病,主要在于网状内皮系统、神经系统等处有广泛的鞘磷脂(Sphingomyelin)沉着,此病分别于1914年为尼曼(Nieman)和1922年为匹克(Pick)二氏首先描述。近年我国称之为神经网状内皮细胞病。据文献记载国内于1959年始有报告,今例数未越20例,现将我科所见1例报告如下,且略加讨论。
Niemann-Pick disease is a congenital, familial disorder of lipid metabolism, mainly due to the extensive sphingomyelin deposition at the reticuloendothelial system, the nervous system, etc. The disease was diagnosed in 1914 as Niemann Nieman) and 1922 for Pick II first described. In recent years, our country called neural reticuloendothelial disease. According to the records in the country, there were reports in the country beginning in 1959, and the number of cases is less than 20 in this case. The report of 1 case we have seen in our department is as follows, with a slight discussion.