Progeria

881 Words Dec 28th, 2013 4 Pages
Melanie Amento
Professor Post
SC-108-1
April 29, 2013
Progeria
Progeria, also known as Hutchinson Gilford Progeria Syndrome, and Progeria syndrome, is an extremely rare genetic disease wherein symptoms resembling aspects of aging are manifested at a very early age. The Progeria come from the Greek words “pro” meaning “before” and “gēras” meaning “old age”. The disorder has a very low incident rate, occurring in an estimated 1 per 8 million live births. Those born with Progeria typically live to their mid teens and early twenties. It is a genetic condition that occurs as a new mutation, and is rarely inherited. Although the term Progeria applies strictly to all diseases characterized by premature aging symptoms, and is often used as
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Children who suffer from Progeria are genetically susceptible to premature, progressive heart disease. Nearly all Progeria patients die from heart disease. Heart disease is also one of the leading causes of death globally. Children with Progeria commonly experience cardiovascular events, such as hypertension (high blood pressure), stroke, angina, enlarged heart and heart failure-conditions linked to aging. Experts say that any research into finding a cure for Progeria would probably have results which would benefit adults with diseases linked to aging. 90% of children with Progeria have a mutation on the gene that encodes Lamin A, a protein that holds the nucleus of the cell together. It is believed that the defective Lamin A protein makes the nucleus unstable. This instability seems to lead to the process of premature aging among Progeria patients.
Progeria appears to occur without cause - it is not seen in siblings of affected children. In extremely rare cases more than one child in the same family may have the condition. Experts do not believe that Progera is hereditary. They say it is due to a rare gene change, which happens purely by chance. A non-twin sibling runs the same risk of having Progera as any other child from another family. In about 1 in every 100 cases of HGPS the syndrome is passed down to the next generation within the same family. The health care professional will possibly suspect Progeria if the signs and

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