粘多糖病是一种基因水平的遗传性疾病。在正常情况下,粘多糖在分解代谢过程中需要溶酶体水解酶,由于先天酶的缺陷,使粘多糖不能正常地分解代谢,而大量的蓄积于体内,累及于多种组织器官,如骨、脑、心、肝、角膜、皮肤等,引起多种临床症状。本病的病理基础为全身结缔组织及纤 Mucopolysaccharidosis is a genetic disease at the genetic level. Under normal circumstances, mucopolysaccharide requires lysosomal hydrolase in the process of catabolism, due to the defects of the innate enzyme, mucopolysaccharide can not be normally catabolized, while a large amount of accumulation in the body, involving a variety of tissues and organs, such as bone , Brain, heart, liver, cornea, skin, etc., causing a variety of clinical symptoms. The pathological basis of the disease for the whole body connective tissue and fiber