What is Progeria?
The word Progeria comes from the Greek progeros meaning 'prematurely old'. The Greek word pro means 'before', while the word geras means 'old age'. HGPS (Hutchinson-Gilford Progeria Syndrome) or Progeria is an extremely rare, fatal genetic condition. Progeria affects children and gives them an appearance of accelerated aging. The classic type of Progeria (there are different forms) is Hutchinson-Gilford Progeria Syndrome (HGPS). Progeria was first described in an academic journal by Dr. Jonathan Hutchinson in 1886, and Dr. Hastings Gilford in 1897 - both in England.
What are the characteristics of Progeria?
Children with Progeria are born looking healthy. When they are about 10 to 24 months old, features of accelerated aging start to appear. Signs of Progeria may include : -
- Growth failure
- Loss of body fat
- Loss of hair
- Skin starts to look aged
- Stiffness in the joints
- Hip dislocation
- Generalized atherosclerosis (cardio and heart disease)
- Stroke
What causes Progeria?
90% of children with Progeria have a mutation on the gene that encodes Lamin A, a protein that holds the nucleus of the cell together. It is believed that the defective Lamin A protein makes the nucleus unstable. This instability seems to lead to the process of premature aging among Progeria patients.
Progeria appears to occur without cause - it is not seen in siblings of affected children. In extremely rare cases more than one child in the same family may have the condition.
Is Progeria hereditary?
Experts do not believe that Progeria is hereditary. They say it is due to a rare gene change which happens purely by chance. A non-twin sibling runs the same risk of having Progeria as any other child from another family. In about 1 in every 100 cases of HGPS the syndrome is passed down to the next generation within the same family.
Diagnosis of Progeria
The health care professional will possibly suspect Progeria if the signs and symptoms are there - aging skin, loss of hair, stiffness of joints, etc. This can then be confirmed through a genetic test. The Progeria Research Foundation has created a Diagnostic Testing Program.
So until scientists unable to control this disease which is no doubt very few numbers of patients in the world and as per Progeria Research Foundation stats right now in the World there are fifty four cases of Progeria in the world in thirty countries,as per some other estimates number can be around one hundred and fifty but whatever the figures may be,sufferer of this disease can tell us that life is not worth living being a Progeria Patient.
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