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Tuesday, May 17, 2011

Children suffering Progeria, Old Before Time

Hair loss and does not grow longer, the blood vessels in the head was clear, wizened skin because there is no fatty tissue, are the traits of progeria patients. worse these symptoms occur in children.

Another characteristic is curved and brittle nails, stiffness in the joints, causing bone loss easily cracked or broken bones, delayed tooth growth, are signs of progeria patients. Though it is a symptom in people who enter old age.


Clinical symptoms that occur in patients with progeria at the top is really heartbreaking. How not, all these distressing symptoms to be experienced by a boy who should be able to grow and play normally.

Progeria is a disease premature aging in children that made ​​her look older than normal age, currently known as the most unique and strange disease that has ever been found.

Derived from the Greek, namely geras meaning old age, disease progeria, also known as HGPS (Hutchinson-Gilford Progeria Syndrome) since it was first discovered by Dr. Jonathan Hutchinson (1886) and Dr. Hastings Gilford (1897).Clinical symptoms that occur in patients with progeria at the top is really heartbreaking. How not, all these distressing symptoms to be experienced by a boy who should be able to grow and play normally.

Progeria is a disease
premature aging in children that made ​​her look older than normal age, currently known as the most unique and strange disease that has ever been found.

Derived from the Greek, namely geras meaning old age, disease progeria, also known as HGPS (Hutchinson-Gilford Progeria Syndrome) since it was first discovered by Dr. Jonathan Hutchinson (1886) and Dr. Hastings Gilford (1897).


Reporting from the Progeria Research, on Thursday (16/07/2009), HGPS is caused due to mutation of a gene called LMNA, which produces protein Lamin A, which can disrupt the stability of the nucleus of the cell (nucleus) in the body. The instability of the cell nucleus suspected by some researchers as the cause of the 'premature aging' in patients with progeria.

Progeria can not be detected as a baby, even while still in the womb was not visible indication. The disease is actually emerged after a 1-2 year old child. No wonder if before the age of 1 year he looks normal and healthy, but just look after the physical signs of aging.


The aging process in progeria patients occurs with a speed 4-7 times the normal aging process. So if a child who has progeria age of 10 years, the appearance will look like the people aged 40-70 years, including respiratory organs, heart, and joints. Really sad.

According to Professor Peter Clayton from Great Ormond Street Hospital, a boy suffering from progeria usually died at the age of 13 years, and until now there is no medicine to cure disease
this.


"This disease is a rare disease and rarely found, but more research is needed to study the aging process and how to discourage it," he said.

There are two newspapers that accompany this disease progeria, which is good news and bad news. The good news, progeria patients is very rare in Indonesia, even in the world. There may be a case that is 1 person of 4-8 million children.


But the bad news is, progeria can happen to anyone, even those whose parents do not have the gene mutation at all. Therefore, the exact cause of this disease can not be known and still studied.

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