Progeria - Hutchinson - Gilford syndrome

Progeria (also known as "Hutchinson–Gilford progeria syndrome" and "Hutchinson–Gilford syndrome") is an extremely rare, severe, genetic condition wherein symptoms resembling aspects of aging are manifested at an early age. The disorder has a very low incidence and occurs in one per eight million live births. Those born with progeria typically live about thirteen years, although many have been known to live into their late teens and early twenties and rare individuals may even reach their forties. It is a genetic condition that occurs as a new mutation and is not usually inherited, although there is a uniquely heritable form. This is in contrast to another rare but similar premature aging syndrome, dyskeratosis congenita (DKC), which is inheritable and will often be expressed multiple times in a family line.

Scientists are particularly interested in progeria because it might reveal clues about the normal process of aging. Progeria was first described in 1886 by Jonathan Hutchinson and also described independently in 1897 by Hastings Gilford. The condition was later named Hutchinson-Gilford Progeria syndrome (HGPS).

Symptoms
The earliest symptoms include failure to thrive and a localized scleroderma-like skin condition. As a child ages past infancy, additional conditions become apparent. Limited growth, alopecia, and a distinctive appearance (small face and jaw, pinched nose) are all characteristic of progeria. People diagnosed with this disorder usually have small, fragile bodies, like those of elderly people. Later, the condition causes wrinkled skin, atherosclerosis, and cardiovascular problems.


Cause
Hutchinson-Gilford progeria syndrome (HGPS) is a childhood disorder caused by a point mutation in position 1824 of the LMNA gene, replacing cytosine with thymine, creating an unusable form of the protein Lamin A. Lamin A is part of the building blocks of the nuclear envelope.
Unlike most other "accelerated aging diseases" (such as Werner's syndrome, Cockayne's syndrome, or xeroderma pigmentosum), progeria is not caused by defective DNA repair. Because these diseases display different aspects of aging but never every aspect, they are often called "segmental progerias".


Diagnosis
Diagnosis is suspected according to signs and symptoms, such as skin changes, abnormal growth, and loss of hair. It can be confirmed through a genetic test.

A young girl with progeria (left). 

A healthy cell nucleus (right, top) 

and a progeric cell nucleus (right, bottom).

Treatment
No treatments have been proven effective. Most treatment focuses on reducing complications (such as cardiovascular disease) with heart bypass surgery or low-dose aspirin. Children may also benefit from a high-calorie diet.
Growth hormone treatment has been attempted.
A type of anticancer drug, the farnesyltransferase inhibitors (FTIs), has been proposed, but their use has been mostly limited to animal models. A Phase II clinical trial using the FTI Lonafarnib began in May 2007.


Prognosis
There is no known cure. Few people with progeria exceed 13 years of age. At least 90% of patients die from complications of atherosclerosis, such as heart attack or stroke.
Mental development is not affected. The development of symptoms is comparable to aging at a rate eight to ten times faster than normal, although certain age-related conditions do not occur. Specifically, patients show no neurodegeneration or cancer predisposition. They do not develop physically mediated "wear and tear" conditions commonly associated with aging, like cataracts (caused by UV exposure) and osteoarthritis (caused by mechanical wear).
Although there may not be any successful treatments for Progeria itself, there are treatments for the problems it causes, such as arthritic, respiratory, and cardiovascular problems.


Epidemiology
A study from the Netherlands has shown an incidence of 1 in 4 million births. Currently, there are between 35 and 45 known cases in the world. Approximately 100 cases have been formally identified in medical history.

Classical Hutchinson-Gilford Progeria Syndrome is almost never passed on from parent to child. It is usually caused by a new (sporadic) mutation during the early division of the cells in the child. It is usually genetically dominant; therefore, parents who are healthy will normally not pass it on to their children. Affected children rarely live long enough to have children themselves.

There have been only two known cases in which it became evident that a healthy parent can carry the LMNA mutation that causes progeria. A family from India has five children with progeria; they were the subject of a 2005 Bodyshock documentary entitled The 80 Year Old Children.

Nabi Hussain Khan, 50, (left) with his wife Razia, 46, (right) and their children. Sanjeeda, now aged 20, (back, centre) does not have Progeria

In the other case, a family from Belgium has two children with progeria. …

Popular culture

• The 1922 short story "The Curious Case of Benjamin Button" by F. Scott Fitzgerald (and later released as a feature film in 2008) may have been inspired by progeria. The main character, Benjamin Button, is born as a seventy-year-old man and ages backwards.
• The Hindi film Paa, released in December 2009, has its story line around progeria (starring Amitabh Bachchan playing a thirteen year old boy Auro).
  
• Progeria is also a central theme in the animated film Renaissance in which one of the characters finds the much sought cure.
• Orlando Gardiner of the science fiction book series Otherland suffers from this disease, and ultimately succumbs to it at the age of 14.
• On the episode "Designate This" from the series "Dark Angel", Max's "younger version" has the disease.
• Rabbi Harold Kushner, author of the well known book, "When Bad Things Happen to Good People," had a son who suffered from this disease, and likely his son's tragic illness was the impetus for this book on suffering.
• The character J.F. Sebastian in the science fiction film Blade Runner states that he suffers from "Methuselah Syndrome," clearly a form of progeria.
• Progeria was featured in the X-Files episode "Young at Heart," where a scientist studying the disease found a way to reverse the aging process.
• A disease similar to progeria, called Werner syndrome, was featured in the movie Jack (film), starring Robin Williams, in which his character aged 4 times faster than normal.
• Leon Botha, who appeared in a video for the South African conceptual rave-rap group Die Antwoord, has Progeria Syndrome.
• The Chuck Palahniuk novel Haunted includes one character, Brandon Whittier, who is dying of progeria at 13.

Source(s): wikipedia

Tran Van Hay aka The Man with the LONGEST HAIR ​

Tran Van Hay died of natural causes, at the age of 79, at his home in Kien Giang province on Fri, Feb 25, 2010., after dedicating his life to curing people, for free. His wife, Nguyen Thi Hoa, says Tran’s personality changed completely after he stopped cutting his hair – he became inspired by the local Buddhist monks  in nearby pagodas to learn traditional medicine to help the poor, and lived a content life, as a herbalist.
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He was 79 and unfortunately died before receiving formal acknowledgement of his record.
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He had stopped cutting his hair 50 years ago and his hair was 6.8m (22ft.) long and weighed 10.5kg (23 lbs). The world record is now in the name of a Chinese woman whose hair is 5.6m long.
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According to his wife Nguyen Thi Hoa, Tran hadn’t gotten a haircut in over 50 years, after getting seriously sick after cutting his hair, when he was 25 years old. And, in half a century, he only washed it a few times, the last time being 11 years ago.
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But his hair had become too much of a burden in his day-to-day life; he could not work as a farmer because of it.
At the time of his death, Tran Van Hay’s hair was over 6.8 meters long and weighed around 10.5 kilograms. As impressive as that sounds, the hair was a real problem for Tran, who couldn’t even catch a motorcycle taxi or work as a farmer, because of the giant pile of hair he had to balance on top his head.
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The herbalist was providing free treatment to poor patients in Chau Thanh District until his death.

The RED-HAIRED MUMMIES of EGYPT

Professor P. F. Ceccaldi, with a research team, studied some hairs from the mummy's scalp. Ramesses II was thought to be 87 years-old when he died, and his hair had turned white. Ceccaldi determined that the reddish-yellow color of the hair was due to a dye with a dilute henna solution. Many Egyptians dyed their hair, and this personal habit was preserved by the embalmers.

Red-haired Ramesses II

However, traces of the hair's original color remained in the roots. Microscopic examinations showed that the hair roots contained natural red pigments, and that therefore, during his younger days, Ramesses II had been a red head. Analysis concluded that these red pigments did not result from the hair somehow fading, or otherwise being altered after death, but did represent Ramesses' natural hair color. Ceccaldi also studied the cross-section of the hairs, and determined from their oval shape, that Ramesses had been "cymotrich" (wavy-haired). Finally, he stated that such a combination of features showed that Ramesses had been a "leucoderm" (white-skinned person).


THE RED HAIRED RAMSES II - LAST SIGNIFICANT WHITE PHARAOH

Egypt's last display of national vigor came with the red haired Pharaoh Ramses II (1292 - 1225 BC). Ramses II managed to re-establish the already decaying Egyptian Empire by recapturing much land in Nubia.

He also fought a series of battles against invading Indo-Europeans, the Hittites. This was culminated with the battle of Kadesh in northern Syria. Ramses signed a treaty with the Hittites in 1258 BC, which ended the war. In terms of the treaty, Ramses took as his wife an Indo-European Hittite princess. His other achievements included the building of the rock-hewn temple of Abu Simbel, the great hall in the Temple of Amon at Karnak, and the mortuary temple at Thebes.

After this king, Egypt entered into a steady period of decay, caused directly by the elimination of the original Egyptians, and their replacement with a mixed population made up of Black, Semitic and the remnant White population. This racially divergent nation was never again to reach the heights achieved by the First, Second or the first part of the Third Kingdoms. In these later years there were competing claimants to the pharaohs throne, many of whom, racially speaking, bore no resemblance to the original pharaohs at all.

The mummy of the wife of King Tutankhamen has auburn hair.

A mummy with red hair, red mustache and red beard was found by the pyramids at Saqqara.

Red-haired mummies were found in the crocodile-caverns of Aboufaida.

The book HISTORY OF EGYPTIAN MUMMIES mentions a mummy with reddish-brown hair.

The mummies of Rameses II and Prince Yuaa have fine silky yellow hair. The
mummy of another pharaoh, Thothmes II, has light chestnut-colored hair.

An article in a leading British anthropological journal states that many mummies have dark reddish-brownhair. Professor Vacher De Lapouge described a blond mummy found at Al Amrah, which he says has the face and skull measurements of a typical Gaul or Saxon.

A blond mummy was found at Kawamil along with many chestnut-colored ones.

Chestnut-haired mummies have been found at Silsileh.

The mummy of Queen Tiy has "wavy brown hair."