The 80 Year Old Children: Progeria

Mighty_Emperor

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On tonight:

Bodyshock: The 80-Year-Old Children

Channel 4, 13 February 9pm

In March 2003 doctors at Calcutta's Institute of Child Health made an astonishing and ground-breaking medical discovery. Quite by accident they had unearthed the only family in the world known to be struck by more than one case of progeria, a rare and incurable genetic disease. There are currently only 48 known cases worldwide, all of them isolated and seemingly random. But the doctors in Calcutta were faced with a mother who had given birth to five affected children.

Progeria is caused by a single tiny defect in a child's genetic code, but it has devastating and life-changing consequences. On average, a child born with this disease will be dead by the age of 13. As they see their bodies fast forward through the normal process of ageing they develop striking physical symptoms, often including premature baldness, heart disease, thinning bones and arthritis.

The 80-Year-Old Children follows Dr Chattopadhyay who diagnosed the Khan children in 2003 as he returns to Calcutta to treat them. This is the first time that the family have been filmed for a documentary. Ikramul, 17, and Rehanna, 19, the two eldest progeria children, see this film as their opportunity to tell the world their story before they die.

The 80-Year-Old Children describes the race to solve this scientific riddle and ultimately cure this devastating disease. It also offers a moving testament to human dignity under tragic circumstances.

www.channel4.com/health/microsites/0-9/ ... _children/

There is also a section with help and information links:

www.channel4.com/health/microsites/0-9/ ... _info.html

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See also this thread about something that is possibly the opposite of progeria (where children don't age):

forteantimes.com/forum/viewtopic.php?t=22237
Link is obsolete. The current link is:
https://forums.forteana.org/index.php?threads/children-who-never-age-grow-up.22237/


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Wikipedia:
http://en.wikipedia.org/wiki/Progeria
 
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Justin_Anstey

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I wonder if there is a genetic disease that slows the ageing process.
 

Mighty_Emperor

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Justin_Anstey said:
I wonder if there is a genetic disease that slows the ageing process.

I get the impression that so much research has gone into such a rare disease because of the important light it could throw on increasing longevity.

See also the other thread I mentioned for more stuff along those lines:

forteantimes.com/forum/viewtopic.php?t=22237
Link is obsolete. The current link is:
https://forums.forteana.org/index.php?threads/children-who-never-age-grow-up.22237/
 
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RainyOcean

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Damn it, I have to post to subscribe to this thread, because despite the fact that I am logged in, there is no "watch this topic" thing. I apologize for wasting anyone's time.
 

EnolaGaia

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The US FDA has approved the first treatment capable of extending a progeria patient's prospective lifespan (if only fractionally).
The FDA has approved the first drug to treat the rapid-aging disease progeria

The U.S. Food and Drug Administration has approved a treatment that could give children with a rare genetic illness that causes premature aging more time to live.

Children with the disease, known as Hutchinson-Gilford progeria syndrome, or progeria for short, often die of heart failure, heart attack or stroke as teenagers. Most children with the disorder die before they reach age 15. The newly approved drug, called Zokinvy, is the first and only approved treatment for progeria and certain related syndromes, the FDA announced November 20.

In clinical trials of 62 children receiving the drug, Zokinvy increased life span by about 3 months on average during the first three years of treatment, compared with another 81 kids who did not take the drug from a separate study that collected their health data. Following children who continued to receive Zokinvy for up to 11 years showed that, on average, kids’ life spans were lengthened by about 2.5 years.

“This is not a cure,” cautions Monica Kleinman, a pediatric critical care doctor at Boston Children’s Hospital who was involved with the clinical trials. “We’ve hopefully extended the life span that [the children] have by slowing the pace of the disease,” but, she says, the drug doesn’t give kids a normal length of life. ...

An estimated 350 to 400 kids across the world have progeria. For these children, a single mutation in their genetic code upends their health (SN: 2/7/13). That mutation interferes with the gene responsible for making the protein lamin A, which helps hold cells’ nuclei together. Children with progeria end up with higher amounts of a defective protein called progerin, which is similar to lamin A but with an extra piece attached. This protein gets stuck in cells’ membranes and can’t be recycled for fresh proteins, causing the cells to prematurely age and making blood vessels and connective tissue stiffer, Kleinman says. ...

FULL STORY: https://www.sciencenews.org/article/fda-approved-first-drug-treat-rapid-aging-disease-progeria
 

Comfortably Numb

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Once upon a time, in a faraway, distant... I wrote an article highlighting my research, at that point, into the 'alien autopsy' footage.

Back then, I was a CompuServe subscriber and frequented its UFO related forum.

Someone else who did so was Ray Santilli, promoter of the footage and I was party to a rare, first-hand insight regarding the background story.

Subsequently, I published a lengthy article entitled, 'The Manikin Who Fell to Earth' and which incorporated the following.

This is revealed with the sole intention of featuring a herein Fortean connection regarding Progeria and which may be of interest.

No discussions otherwise regarding the footage are pertinent.

Consequently, for information solely, this is the relevant extract:

An Earthly Cause

One person who claims to have the answer is Dr Thomas Jansen of the prestigious Ludwig-Maximilians-University, Munich. Der Spiegel reported that when Jansen saw the footage, he recognised it as depicting a "textbook case" of progeria, an exceedingly rare premature ageing disorder. Jansen believed he saw "all the typical identifying characteristics", a diagnosis reportedly endorsed by his colleagues at the University. Such was the conviction of the "one hundred percent watertight" case, that it was to be published in the Munich Weekly Medical Journal.

Jansen points out that in cases of progeria, only the skull bones grow normally, the growth of the other bones is retarded and the head is disproportionate.

A "beak-like" nose, stunted ears and mouth are all characteristics, the genitals are "not mature" and the stomach is swollen due to the manner in which the intestines form.

Polydactylism - extra digits - is also a feature of such rare deformities.

Jansen even has an explanation for the apparent absence of a navel. Because the subdermal tissue shrinks, the skin is pulled tight and the navel is not visible.

Whilst this appears to offer a possible explanation, there are a number of obvious problems with Jansen's theory.

As Theresa Carlson notes, "The reflected skin reveals no subcutaneous fat layer. While some diseases will diminish body fat considerably, the reflected skin shows no fat whatsoever. Also missing from this body are veins and arteries".

Furthermore, neither the internal organs or the brain are human and Jansen offers no explanation for the extensive leg damage, partially severed hand, the dark eye coverings, or the need for protective clothing".
[End of Extract]
 

Kondoru

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My question is, if its just a simple genetic fault, why is it so very rare?
 

Xanatic*

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Not all genetic faults are equally likely.
In a previous place that I lived, one of the neighbourhood kids looked like he might have it.
 

WeeScottishLassie

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I've seen a few documentaries about them and it's just horrifying but equally fascinating at the same time.
 
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