青霉胺是目前治疗粘多糖病Ⅰ型(MPS-Ⅰ)遗传性代谢障碍病的唯一有效药物,但因该药奇缺、加上价格昂贵而使很多患者失去了及时治疗的机会,但可通过用中药去实逐瘀汤治疗,疗效较好。患儿男,4岁,因幼儿园体检发现肝脾肿大而就医,通过临床症状及体征,X线摄片骨骼明显改变,尿甲苯胺兰试验弱阳性,粘多糖电泳硫酸皮肤素(DS)升高,硫酸乙酰肝素(HS)升高等特征性变化而被确 Penicillamine is currently the only effective treatment of hereditary metabolic disorders of mucopolysaccharidosis type I (MPS-I), but many patients have lost the chance of being treated promptly due to the lack of the drug and the high cost of the drug By using traditional Chinese medicine to cure stasis soup, the effect is better. Children aged 4 years old, because of kindergarten physical examination found hepatosplenomegaly and medical treatment, through clinical symptoms and signs, X-ray bone was significantly changed, urine toluidine blue test weakly positive, mucopolysaccharide electrophoresis dermatan sulfate (DS) High, heparan sulfate (HS) increased and other characteristic changes were confirmed