Lysosomes are small bodies containing a series of acidic hydrolytic enzymes enclosed by a single lipoprotein membrane in the cell plasma. Lysosomes contain many types of hydrolytic enzymes and are capable of breaking down many kinds of substances, and they are likened to an intracellular enzyme warehouse digestive system. The enzymes in lysosomes are all hydrolytic enzymes, and generally the optimum pH is 5, so they are all acidic hydrolytic enzymes. The enzymes in the lysosomes, if released, will digest the entire cell. They are generally not released into the internal environment and are mainly digested intracellularly. Congenital lysosomal diseases are a class of metabolic genetic diseases caused by the congenital lack of a certain lysosomal enzyme due to mutations in certain genes on chromosomes. Mucopolysaccharide storage disease is a group of diseases that occur due to the inability to degrade acidic mucopolysaccharide molecules (aminoglucan) caused by lysosomal enzyme defects, resulting in massive mucopolysaccharide deposition in tissues and increased mucopolysaccharide excretion in urine. According to the clinical manifestations and enzyme defects, MPS can be divided into 6 types such as Ⅰ~Ⅶ, among which type I is divided into type ⅠH and type ⅠS, and type V has been renamed as type ⅠH/S. Except for type II, which is sex-linked recessive, the rest are autosomal recessive disorders. As with other lysosomal accumulation diseases, most of the MPS types develop around 1 year of age, and the disease course is progressive and involves multiple systems with similar clinical symptoms, but the severity of each type varies and has its own characteristics, among which type IH is the most typical and has the worst prognosis, with children often dying before 10 years of age; type IS has the mildest disease. The lesions mainly involve the skeleton, but also the central nervous system, cardiovascular system, liver, spleen, joints, tendons, skin, etc.