Forums

Rare syndromes part 1: The hutchinson-Gilford progeria syndrome

Sort:
Poweranony

The purpose of this thread is merely informational.
Did you know that there is a genetic disorder called the progeria syndrome? It's a syndrome where the people who suffer from it age much faster than a normal person, estimated to be between 5 to 10 times faster. 
In average, persons who suffer this syndrome live between 8 to 21 years, and they usually pass out because of hearth diseases that normally appear in elder people. 

Here's an image of how people with this syndrome can look like:

This image corresponds to a popular person with this syndrome called Sam berns. He passed away at the age of 17 (rest in peace). 

Poweranony

The reason persons with this syndrome age faster is because they have a mutation on the LMNA gene, which causes the production of a permanently farnesylated mutant lamin A called progerin, and this contributes to premature aging. On normal proteins, this farnesyl group would be removed, but people with progeria are not able to remove it and therefore they have alterations.

DaTrueSliverwolf
Ah
GoatedPawn12
That is sad
Thekinginvasor09

I've just looked for some images, and i'm really sad for the people who have it. The have a much more limited lifetime than us

Polygonatic2_26

That syndrome is very rare. According to cleveland clinic statistics, it happens approximately 1 time per every 4 million births worldwide.
There is a case of a woman with this syndrome that managed to be still alive, she's a bit older than 40 years if im correct

DaTrueSliverwolf
Wow
Poweranony

That is true #7.
the name of that woman is tiffany wedekind. Though, it's believed her progeria may differ from other patients with that disease making it less severe. It's still astonishing that she has survived with that syndrome, around three times more than the average patient of it. 

Poweranony

Here is an image of tiffany wedekind, the oldest patient with progeria syndrome, at the age 41.

Thekinginvasor09

At least tiffany wedekind is still alive i guess