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涎酸累积症(sialidosis)系近年来由粘脂病中独立出来的一组疾病。根据Spra-nger(1970)的定义,粘脂病是包括内脏及间质细胞含有大量酸性粘多糖、神经鞘脂(sphingolipid)和/或糖脂累积的遗传代谢性疾病。曾被分为8类,即粘脂病Ⅰ、Ⅱ、Ⅲ、Ⅳ型,甘露糖、岩藻糖累积症,单涎酸四己糖神经节苷脂(GM_1)及粘硫脂(mucosulfatide)累积症。患者症状有相似之处,具有Hurler 病的表型,但无粘多糖尿。随近代分子生化诊断水平的日
Sialidosis (sialidosis) is a group of diseases independent of viscous disease in recent years. According to Spra-nger (1970), mucoidosis is a genetically metabolic disease that includes visceral and mesenchymal cells that contain large amounts of acidic mucopolysaccharides, sphingolipids and / or glycolipids. It has been divided into 8 categories, namely, viscous disease type Ⅰ, Ⅱ, Ⅲ, Ⅳ, mannose, fucose accumulation, monosomyosin tetrahexose ganglioside (GM_1) and mucosulfatide accumulation disease. Symptoms in patients are similar, with a Hurler disease phenotype, but no mucosal glycosuria. With the modern molecular biochemical diagnosis of the day