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粘多糖沉积病(MPS)是遗传性酶代谢障碍的结缔组织病,目前尚无根治方法,但认识本病的类型及临床表现,预防和治疗其并发症对预后的判断或延长生存期有一定价值。MPS的临床分型MPS是属于溶酶体贮积病的一大类疾病,溶酶体中存在多种能在酸性环境下水解糖链的糖苷酶,而MPS则是以各种糖苷酶缺乏,造成粘多糖在各种组织中沉积的疾病。MPS患者有面容粗犷,角膜混
Mucopolysaccharidosis (MPS) is a hereditary disorder of enzyme metabolism of connective tissue disease, there is no cure, but the type and clinical manifestations of this disease, prevention and treatment of complications to determine the prognosis or prolong survival of a certain value. MPS clinical classification MPS belongs to a major disease of lysosomal storage diseases, lysosomal presence of a variety of glycosidases can hydrolyze sugar chains in acidic environment, and MPS is a variety of glycosidase deficiency, Diseases that cause mucopolysaccharides to deposit in various tissues. MPS patients have rough face, corneal mixed