Quote:
Originally posted by hoosier
In the new Rainbow, page 13, is the Albion College/Epsilon report.
This amazing chapter, left with only four members (due to unfortunate circumstances, it says) got six pledges and won Greek Week.
They also raised money for a local boy suffering from "Hurler's syndrome."
Do you think somenoe is trying to pull a joke on us?
Could Hurler's syndrome be caused by hitting the bottle a little too much?
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Hoosier,
I've never heard of that disease either, so I did a search and it is real -- and very serious:
What is Hurlers disease?
Hurler Disease and Scheie Disease (MUCOPOLYSACCHARIDOSIS TYPE I)
Hurler disease (MPS IH) is a progressive disorder. Development and growth are usually normal in the first year,
but with ensuing regression it is the most severe mucopolysaccharidosis. The disease is characterised by
severe mental retardation, coarse facies, corneal clouding, stiff joints including claw hands, dwarfism,
dorso-lumbar gibbus usually in the second or third year, cardiovascular manifestations such as cardiac murmurs
and angina pectoris, hepatosplenomegaly, often papilloedema due to communicating hydrocephalus, inguinal
and umbilical hernias, glaucoma, rhinorrhea and deafness. Radiologically, after about 18 months the lateral view
of the spine shows characteristic beaking of the lower dorsal and upper lumbar vertebrae. The skull may show
evidence of hydrocephalus and an enlarged J-shaped sella turcica. Death occurs usually by the end of the first
decade. At the milder end of the MPS I clinical spectrum, Scheie disease (MPS IS) is characterised by stiff
joints, especially of the hands, aortic incompetence, corneal clouding and normal or high intelligence. Between
the two extremes of Hurler's and Scheie's diseases there is a continuum of intermediate phenotypes, including
the Hurler/Scheie compound type (MPS IH/S) in which patients suffer the severe somatic problems of Hurler's
disease but have normal intelligence.
How is it treated?
Bone marrow transplantation (BMT) is often the preferred method of treatment for a variety of blood disorders including certain leukemias, aplastic anemia, and inborn errors of metabolism (such as thalassemia major, Hurler's Syndrome, and severe Gaucher's disease). Generally, BMT consists of administering high doses of chemotherapy and/or irradiation to destroy abnormal cells in the patient's bone marrow and then transfusing pluripotent blood stem cells (often referred to as pluripotent hematopoietic stem cells, or PHSC) from a tissue- (HLA-) matched donor, usually a sibling, in order to replace the normal blood elements. Because most patients do not have an acceptable matched, related donor, the National Marrow Donor Program has been established to match volunteer bone marrow donors with potential recipients who require BMT. The registry is currently composed primarily of Caucasians of Western European descent, making it extremely difficult to find potential donors for ethnic minorities.
Recently, several investigators have shown that umbilical cord blood is a rich source of pluripotent stem cells and can be used in place of bone marrow in transplantation. To date, umbilical cord blood has been used in sibling and other related donor transplants. For more information on Cord Blood Transplants click on Duke Website.
"Hurler's syndrome"
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