An autosomal recessive inherited disorder of mucopolysaccharide metabolism. it is the most severe form of mucopolysaccharidosis type i. it is characterized by deficiency of the enzyme alpha-l-iduronidase resulting in the accumulation of mucopolysaccharides in the tissues. check for "https://www.cancer.gov/about-cancer/treatment/clinical-trials/intervention/c61261" active clinical trials using this agent. ("http://ncit.nci.nih.gov/ncitbrowser/conceptreport.jsp?dictionary=nci%20thesaurus&code=c61261" nci thesaurus)

Hurler syndrome, also known as mucopolysaccharidosis Type IH (MPS-IH), Hurler's disease, and formerly gargoylism, is a genetic disorder that results in the buildup of large sugar molecules called glycosaminoglycans (GAGs) in lysosomes. The inability to break down these molecules results in a wide variety of symptoms caused by damage to several different organ systems, including but not limited to the nervous system, skeletal system, eyes, and heart. The underlying mechanism is a deficiency of alpha-L iduronidase, an enzyme responsible for breaking down GAGs.: 544  Without this enzyme, a buildup of dermatan sulfate and heparan sulfate occurs in the body...