Wednesday, March 18, 2009

PROGERIA


Progeria is an extremely rare, genetic condition (1 in 4,000,000) which causes exceptionally rapid aging. Children who are born with this ailment usually die by the time they are 13 years old.

Though the disease is genetic, it is not usually inherited. It is a mutation which causes a change to LMNA, a gene on chromosome 1 that is crucial in producing the membrane which surrounds cell walls. It is this change to the cellular membrane that causes the rapid aging seen in progeria patients.

There is no treatment for Progeria. Instead, doctors focus on solving the problems that the disease causes, such as heart disease, through surgery and profilactic measures.

90% of progeria cases die from cardiovascular causes such as stroke or heart attack.

Progeria was first discovered in 1886 by Jonathan Hutchinson. It was also independently documented in 1897 by Hastingls Gilford. The disease's full name is: Hutchinson-Gilford Progeria syndrome (HGPS).

This is a truly devestating illness with 100% mortality over a relatively long period of time.
Its physical manifestations are disturbing and painful to the victim.

So, this sick gets a:
9 on my lethality scale (1-10)
and a 9 on my disturbing scale (1-10)

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