Hutchinson-Gilford+Progeria+Syndrome

**__FACTS__ by Nigel and Adam**
Progeria means premature aging in Greek and there were only 45 cases in 22 countries as of 2006. The chances of having this disorder are only 1:4-8 million. Progeria is not seen in siblings but boys and girls have the same chances of developing the disease. Children with Progeria are already thin and balding at a young age (2-5 years). They also have withered, shriveled narrow faces, old looking skin, loss of body fat, joint dislocation, and poor blood flow to the heart.

The children with Progeria usually die of heart failure due to enlarged hearts and high blood pressure, or stroke. Childern can also experience chest pain called angina. In the first year of life the chidren have growth failure, resulting in a small body and a big head.

__**CAUSES**__
Progeria is a result of a defect in the Lamin A gene. The product of this gene is Lamin A protein which important for the stablity of the nuclear membrane. Why this mutation causes rapid aging is unknown. These mutations arise sporadically (de novo) and are not passed through the parents because the childern die before reaching reproductive age.

__**HISTORY**__
Hutchinson-Gilford Progeria Syndrome was named after the two Engish doctors who discovered it independently. It was described by Dr. Jonathan Hutchinson in 1886 and Dr. Hastings Gilford in 1897. However, the Progeria gene itself was not found until 2003.

**__TREATMENT__**
The Progeria Research Program has a Diagnostics Testing Program. This program enables people who research Progeria and patients to look at the genetic mutation in their genes. There are no complete cures but researchers are exploring the use of drugs like farnesyltransferase inhibitors (FTIs) which have been used to treat cancer but might work for Progeria patients.

__DISCUSSION QUESTIONS__
What are the symptomes of Progeria?

What are the causes Progeria?

How is it treated?

What does Progeria mean? Who was it named after?

What are the chances of getting Progeria?



Work Cited
http://www.progeriaresearch.org/ UNknow Author Tile:Progeria Research Foundation http://www.nlm.nih.gov/medlineplus/ency/article/001657.htm. Unknown Author Tile: Medine Plus http://www.mayoclinic.com/health/progeria/DS00936/DSECTION=2 Unknow Author Tile: Progeria Signes and Symtpomes