Progeria - Hutchinson-Gilford Progeria Syndrome (quot;Progeriaquot;, or quot;HGPSquot;) is a rare, fatal genetic condition characterized by an appearance of accelerated aging in children. Its name is derived from the Greek and means quot;prematurely old.quot; While there are different forms of Progeria*, the classic type is Hutchinson-Gilford Progeria Syndrome, which was named after the doctors who first described it in England; in 1886 by Dr. Jonathan Hutchinson and in 1897 by Dr. Hastings Gilford. 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. Although they are born looking healthy, children with Progeria begin to display many characteristics of accelerated aging at around 18-24 months of age. Progeria signs include growth failure, loss of body fat and hair, aged-looking skin, stiffness of joints, hip dislocation, generalized atherosclerosis, cardiovascular (heart) disease and stroke. The children have a remarkably similar appearance, despite differing ethnic background. Children with Progeria die of atherosclerosis (heart disease) at an average age of thirteen years (with a range of about 8 - 21 years). * Other progeroid syndromes include Werner s syndrome, also known as quot;adult progeriaquot; which does not have an onset until the late teen years, with a life span into the 40 s and 50 s.
Sunday, April 25, 2010
What is the disease of children that makes them grow older 4x faster? -
What s that disease called? It s like the one Robin Williams had in his movie quot;Jackquot;. Only it s not like the person will grow taller and will look like an adult. The person will grow older physiologically, as in, he ll get wrinkles and he will get diseases, like hypertension,etc, which commonly afflicts adults, people with congenital diseases, or those who don t have a heathly lifestyle.
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