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Hutchinson-Gilford Progeria Syndrome

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Hutchinson-Gilford Progeria Syndrome

Progeria is a dominant, rare and fatal genetic condition caused by an appearance of accelerated aging in children. Its name originates from Greek literacy and means "prematurely old". Progeria, also known as Hutchinson-Gilford Progeria Syndrome (HGPS), was named after the doctors who first described it in England in 1886 by Dr. Jonathan Hutchinson and in 1897 by Dr. Hastings Gilford when the first two patients were diagnosed with the disease. Progeria affects about one in every four to eight million newborns and although they are born looking healthy, children with Progeria begin to display many characteristics of accelerated aging around eighteen to twenty-four months of age when the baby fails to gain weight and skin changes occur. Progeria is usually first diagnosed based only on appearance and treatment is given for other conditions associated with the aging process rather than the disease itself.

Affected children age up to seven times faster and unfortunately have an average lifespan of thirteen years. Over time the child begins to age not only appearance but physically as well, some symptoms that are obvious in appearance are alopecia, wrinkled skin accompanied by pigmented age spots, a high pitched voice( caused by underdevelopment) underdeveloped sexual maturation; bone lesions, often resulting in fractures and hip dislocation. Death is more commonly due to cardiovascular problems that usually affect the body at a much greater age and generally includes arthrosclerosis, myocardial infraction and congestive heart failure.

Although there is no cure for Progeria there is an on-going research that has successfully found the cause of Progeria in October 2002 by a group of scientists from the Progeria Research Foundation's Genetics Consortium. "HGPS is caused by a mutation in the gene called LMNA (pronounced, lamin - a). The LMNA gene produces the Lamin A protein, which is the structural scaffolding that holds the nucleus of a cell together. Researchers now believe that the defective Lamin A protein makes the nucleus unstable. That cellular instability appears to lead to the process of premature aging in Progeria." (Progeria Research, Vol. 163, p. 260, April 26, 2003).



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