If either the person just having made a not most obvious statement in Flemish, or some listener sneezes, often one of the listeneners will say "It is beniesd", literally "Its sneezed upon", as if a proof of truth – usually self-ironically recalling this old superstitious habit, without either suggesting doubt or intending an actual confirmation, but making any apology by the sneezer for the interruption superfluous as the remark is received by smiles.In Europe, principally around the early Middle Ages, it was believed that ones life was in fact tied to ones breath – a belief reflected in the word "expire" (originally meaning "to exhale") gaining the additional meaning of "to come to an end" or "to die". This connection, coupled with the significant amount of breath expelled from the body during a sneeze, had likely led people to believe that sneezing could easily be fatal. Such a theory could explain the reasoning behind the traditional English phrase, "God bless you", in response to a sneeze, the origins of which are not entirely clear (see "Traditional Responses To A Sneeze" below for alternative explanations). Sir Raymond Henry Payne Crawfurd, for instance, the registrar of the Royal College of Physicians, in his 1909 book, "The Last Days of Charles II", states that, when the controversial monarch was on his deathbed, his medical attendants administered a concoction of cowslips and extract of ammonia to promote sneezing.
A person born with one normal copy and one defective copy is called a carrier. They will produce less α-L-iduronidase than an individual with two normal copies of the gene. The reduced production of the enzyme in carriers, however, remains sufficient for normal function; the person should not show any symptoms of the disease. Mechanisms
The IDUA gene is responsible for encoding an enzyme called alpha-L-iduronidase. Through hydrolysis, alpha-L-iduronidase is responsible for breaking down a molecule called unsulfated alpha-L-iduronic acid. This is a uronic acid found in the GAGs dermatan sulfate and heparan sulfate. The alpha-L-iduronidase enzyme is located in lysosomes. Without sufficient enzymatic function, these GAGs cannot be digested properly. Diagnosis
Diagnosis often can be made through clinical examination and urine tests (excess mucopolysaccharides are excreted in the urine). Enzyme assays (testing a variety of cells or body fluids in culture for enzyme deficiency) are also used to provide definitive diagnosis of one of the mucopolysaccharidoses. Prenatal diagnosis using amniocentesis and chorionic villus sampling can verify if a fetus either carries a copy of the defective gene or is affected with the disorder. Genetic counseling can help parents who have a family history of the mucopolysaccharidoses determine if they are carrying the mutated gene that causes the disorders. Classification
All members of the mucopolysaccharidosis family are also lysosomal storage diseases. Mucopolysaccharidosis type I (MPS I) is divided into three subtypes based on severity of symptoms. All three types result the absence or decreased functioning of the same enzyme.