What is Progeria? – Causes, Symptoms

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History of progeria
It is indeed a strange phenomenon that one starts to age very early. This health condition is called “progeria”, which is common among children. This syndrome is highlighted in many of the documentaries, talk’s shows as well as American serials.

What is progeria?
This term has been derived from the Greek word, which is “prematurely old.” The persons who discovered this syndrome in the year 1886 are Hastings Gilford and Jonathan Hutchinson. Subsequently about 100 cases have been identified.

It is also known as Hutchinson-Gilford progeria. It is genetically a rare condition as ageing occurs in about one among eight million newborn babies. Most of these babies do not survive once they enter the adolescent age. The maximum period of survival age is not above 30 years, in which case heart stoke is the major cause of death.

Causes of Progeria
It happens to be an autosomal recessive disease. In other words about 90% of the progeria- afflicted children tend to have mutation in the single gene, which does not show kind of symptoms.

It has been also been discovered that about 90% of the progeria afflicted children have a gene which encodes Lamin A protein (LNMA), which is a fibrous protein that happens to be involved in the nuclear membrane structure.

Symptoms of Progeria
The child does not develop in first year of his or her birth. To start with the child looks normal. The actual symptoms become evident when the child is about 6 to 12 months of age, when there is no weight gain. As a result of which the body grows in the form of a dwarf in actual contrast to the head size. The face happens to be small in size with one’s nose pinched. As the ailment progresses, it causes wrinkles, cardiovascular problems as well as arteriosclerosis.

Some common symptoms

  • Face and Head – It indicates a narrowed face, prominent scalp and baldness, prominent scalp veins as well as eyes.
  • Bones – The limbs happen to be fragile and consist of stiff joints and reflect short stature and dislocation.
  • Cardio vascular disease – nearly 97 % of children affected happen to be of Caucasian origin. All the children have the same appearance despite their racial as well as ethnic backgrounds. The children suffer arteriosclerosis at an early age. This is a health condition that cause irregular heartbeats. At rare times, there is a blockage of arteries.
  • Skin – There are changes in the skin. It shows signs of scleroderma. The connective tissues become very tough as well hardened.

There happens to be no permanent cure for progeria.

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