Scheie syndrome is considered the mild form of MPS I. Individuals with Scheie syndrome usually have normal intelligence
Arne Scheie was born on January 7, 1944.
Arne Scheie was born on January 7, 1944.
Scheie Eye Institute was created in 1972.
Common physical problems include corneal clouding, heart abnormalities, and orthopedic difficulties involving the hands and back
Common physical problems include corneal clouding, heart abnormalities, and orthopedic difficulties involving the hands and back
Arne Scheie is 67 years old (birthdate: January 7, 1944).
Mutations in the alpha-L-iduronidase (IDUA) gene located on chromosome 4 cause the MPS I disorders (Hurler, Hurler-Scheie, and Scheie syndromes
DefinitionScheie syndrome is an inherited disease of metabolism in which the body cannot properly break down long chains of sugar molecules called glycosaminoglycans (formerly called mucopolysaccharides).The syndrome belongs to a group of diseases called mucopolysaccharidoses (MPS). Specifically, it is known as MPS I S.See also:MPS I H (Hurler syndrome)MPS II, Hunter syndromeMPS IV Morquio syndromeMPS III (Sanfilippo syndrome)Alternative NamesMucopolysaccharidosis type I S; MPS I SCauses, incidence, and risk factorsPersons with Scheie syndrome are missing a substance called lysosomal alpha-L-iduronidase. This substance, called an enzyme, helps break down long chains of sugar molecules called glycosaminoglycans (formerly called mucopolysaccharides). These molecules are found throughout the body, often in mucus and in fluid around the joints.Without the enzyme, glycosaminoglycans build up. This excess material is stored in body tissues and can damage organs, including the heart. Symptoms can range from mild to severe.Scheie syndrome is the mildest form of mucopolysaccharidosis type 1. The syndrome is transmitted as an autosomal recessive trait. That means that both parents must pass along the defective gene for the offspring to have the disease.SymptomsSymptoms may not appear until age 4 or 5, and may include:Broad mouth with full lipsClaw hands and deformed feetCloudy corneaand progressive loss of vision, resulting in blindnessCoarsened facial featuresIncreased body hair (hirsutism)PrognathismStiff jointsSigns and testsA physical exam may show signs of:Aortic regurgitationHearing lossHernias -- inguinal hernia, umbilical herniaLiver swellingPrognathismAn eye exam will show cloudy corneas and retinal pigmentation.Urine tests will be done. Persons with Scheie syndrome have increased amounts of dermatan and heparan sulfate in their urine. See: Urine dermatan sulfateOther tests may include:Blood cultureCulture of skinfibroblastsEchocardiogramX-ray of the skeletonTreatmentEnzyme replacement therapy for patients with a defect in the enzyme a-L-iduronidase is now possible. This includes individuals with Scheie syndrome, but also Hurler and Hurler-Scheie syndromes.Early recognition and treatment of spinal cord compression can prevent permanent nerve damage. Treatment is also given for heart problems caused by leaky valves.Support GroupsThe National MPS Society -- www.mpssociety.orgExpectations (prognosis)Scheie syndrome is compatible with an almost normal lifespan. However, some disabilities such as limitation of joints, blindness, or deafness are likely to occur later in life.ComplicationsCompression of the spinal cord, which can lead to loss of nerve functionExtremity deformitiesHearing lossand deafnessProblems with the aortic valveVision problemsCalling your health care providerCall your health care provider if you have symptoms of this disorder.PreventionGenetic counseling is recommended for prospective parents with a family history of Scheie syndrome. Counseling is also recommended for families who have a child with Scheie syndrome, to help them understand the condition and possible treatments.
Hurler (MPS I H), Hurler-Scheie (MPS I H/S), Scheie (MPS I S), Hunter (MPS II), Sanfilippo (MPS III), Morquio (MPS IV), Maroteaux-Lamy (MPS VI),
Arne Scheie has: Played Kommentator (1990-) in "Sportsrevyen" in 1990. Played Sorrow man in "U" in 1991. Played himself in "Myggen" in 1996. Played himself in "Gylne tider" in 2002. Played Himself - TV sports commentator in "United" in 2003. Played himself in "Showman" in 2009.
The cast of Myggen - 1996 includes: Erik Mykland as himself Egil Olsen as himself Arne Scheie as himself
Notable is the correct spelling.