What is Progeria? What is the possible treatment of it?

Progeria is a rare disease in world. It is also called Hutchinson-Gilford Progeria Syndrome. The word ‘progeria’ comes from
Greek which means ‘prematurely old’. Progeria is an accelerated aging disease which develops in children from infancy. This accelerating age process usually seven times more than normal aging. A child of ten years, having progeria looks like a 70 years
old person. S/he may also have the same respiration, joint aches and heart system of 70 years old person. Progeria usually affects 1 in 8 million children.

Causes

There are not any exact causes found for progeria. Usually it is said that it is a genetic disease which comes from parents genes. In progeria, gene which is called
“Lemin A” holds unstable mutation.  For each parent gene, there are two copies of “Lemin A”: one from each parent. This disease is believed to be the result of a dominant mutation.
It occurs when one parent gene is abnormal copy and one is normal copy. At the time of mutation, neither of parents carries the mutation. Each parent is considered to represent
sporadic new mutation at the time of conception.

Symptoms

Children having progeria are born healthy. The first symptoms of this disease start appearing at the age of 12 to 24 months. The children’s age accelerating features starts to appear in their infancy period.  Other progeria symptoms include: loss of hair,
thrive failure, wrinkled and old-aged looking skin, pinched nose, stiffness of joints and hip dislocation. Children having this disease, almost have similar appearances even if they belong to a different background.

Treatment

Children having this disease normally live from 20 to 22 years at the higher possibilities. A kind of anticancer drug,
Farnesyltransferase inhibitors (FTIs), which is currently using for cancer treatment, might overturn the genes structure abnormalities that are believed to cause Progeria. However, it should always keep in mind that there is no effective treatment
for this disease. All potential drugs can be used in pre-clinical stages of progeria.

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Posted by Zeeshan Gohar on Mar 21 2012. Filed under Health. You can follow any responses to this entry through the RSS 2.0. You can leave a response or trackback to this entry