The on-site manufacture of nitroglycerin was thus required for the remaining hard-rock drilling and blasting required for the completion of the First Transcontinental Railroad in North America.In June 1869, two one-ton wagons loaded with nitroglycerin, then known locally as Powder-Oil, exploded in the road at the North Wales village of Cwm-y-glo. The explosion led to the loss of six lives, many injuries and much damage to the village. Little trace was found of the two horses. The UK Government was so alarmed at the damage caused and what could have happened in a city location (these two tons were part of a larger load coming from Germany via Liverpool) that they soon passed The Nitro-Glycerine Act of 1869. Liquid nitroglycerin was widely banned elsewhere, as well, and these legal restrictions led to Alfred Nobel and his companys developing dynamite in 1867. This was made by mixing nitroglycerin with diatomaceous earth ("Kieselguhr" in German) found in the Krümmel hills. Similar mixtures, such as "dualine" (1867), "lithofracteur" (1869), and "gelignite" (1875), were formed by mixing nitroglycerin with other inert absorbents, and many combinations were tried by other companies in attempts to get around Nobels tightly held patents for dynamite. Dynamite mixtures containing nitrocellulose, which increases the viscosity of the mix, are commonly known as "gelatins". Following the discovery that amyl nitrite helped alleviate chest pain, the physician William Murrell experimented with the use of nitroglycerin to alleviate angina pectoris and to reduce the blood pressure.
MPS-IH (Hurler syndrome) is the most severe of the MPS I subtypes. The other two types are MPS-IS (Scheie syndrome) and MPS-IHS (Hurler-Scheie syndrome).Because of the substantial overlap between Hurler syndrome, Hurler-Scheie syndrome, and Scheie syndrome, some sources consider these terms to be outdated. Instead, MPS I may be divided into "severe" and "attenuated" forms. Treatment
There is currently no cure for Hurler Syndrome. Enzyme replacement therapy with iduronidase (Aldurazyme) may improve pulmonary function and mobility. It can reduce the amount of carbohydrates being improperly stored in organs. Surgical correction of hand and foot deformities may be necessary. Corneal surgery may help alleviate vision problems.Bone marrow transplantation (BMT) and umbilical cord blood transplantation (UCBT) can be used as treatments for MPS I. BMT from siblings with identical HLA genes and from relatives with similar HLA genes can significantly improve survival, cognitive function, and physical symptoms. Patients can develop graft versus host disease; this is more likely in non-sibling donors. In a 1998 study, children with HLA-identical sibling donors had a 5-year survival of 75%; children with non-sibling donors had a 5-year survival of 53%.Children often lack access to a suitable bone marrow donor. In these cases, UCBT from unrelated donors can increase survival, decrease physical signs of the disease, and improve cognition. Complications from this treatment may include graft versus host disease. Prognosis
A British study from 2008 found a median estimated life expectancy of 8.7 years for patients with Hurler syndrome. In comparison, the median life expectancy for all forms of MPS type I was 11.6 years.