The incidence of Hunter syndrome is estimated to be 1 in 100,000 to 150,000 male births. MPS III
MPS III, Sanfilippo syndrome, is marked by severe neurological symptoms. These include progressive dementia, aggressive behavior, hyperactivity, seizures, some deafness and loss of vision, and an inability to sleep for more than a few hours at a time. This disorder tends to have three main stages. During the first stage, early mental and motor skill development may be somewhat delayed. Affected children show a marked decline in learning between ages 2 and 6, followed by eventual loss of language skills and loss of some or all hearing. Some children may never learn to speak. In the syndromes second stage, aggressive behavior, hyperactivity, profound dementia, and irregular sleep may make children difficult to manage, particularly those who retain normal physical strength. In the syndromes last stage, children become increasingly unsteady on their feet and most are unable to walk by age 10. Thickened skin and mild changes in facial features, bone, and skeletal structures become noticeable with age. Growth in height usually stops by age 10. Other problems may include narrowing of the airway passage in the throat and enlargement of the tonsils and adenoids, making it difficult to eat or swallow. Recurring respiratory infections are common. There are four distinct types of Sanfilippo syndrome, each caused by alteration of a different enzyme needed to completely break down the heparan sulfate sugar chain.
Circumcision
Lidocaine/prilocaine eutectic mixture has been used during circumcision in newborn boys and has been considered efficacious and safe to lessen pain from circumcision.The European Medicines Agency concludes in its latest (2014) statement on Emla:
"Safety and efficacy for the use of EMLA on genital skin and genital mucosa have not been established in children younger than 12 years. Available paediatric data do not demonstrate adequate efficacy for circumcision. "The Patient Information Leaflet of EMLA in the UK states: "EMLA Cream should not be applied to the genital skin (e.g. penis) and genital mucosa (e.g. in the vagina) of children (below 12 years of age) owing to insufficient data on absorption of active
substances." Tempe
The spray is a combination of local anaesthetics lidocaine and prilocaine in a metered-dose aerosol that is sprayed directly on the penis to numb sensations. It was developed by the same group that invented the erectile dysfunction drug sildenafil. The drug was approved in Europe and was released in the UK market in November 2016 and within the EU will be marketed by, early in 2017 In the United States, the Food and Drug Administration (FDA) is expected to approve the drug in 2018. Compendial status
United States Pharmacopeia 31

See also
Dosage form
Eutectic point
Lidocaine
Prilocaine

References
External links
"Lidocaine mixture with Prilocaine". Drug Information Portal. U.S. National Library of Medicine.