Progeria
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What would you do if at the age of one years old you started to look like a decaying human being? One in eight million children face this problem. The disease is called Progeria (also known as Hutchinson-Gilford Progeria Syndrome.) Progeria is a very rare terminal condition. Progeria, affects both males and females of all ethnic backgrounds. The term Progeria comes from the Greeks and it means “prematurely old”. In the following paragraphs I will be discussing symptoms of Progeria, treatments, and other facts about this rare but serious disease.
Progeria is named after Jonathan Hutchinson, who first described the disease in 1886 and Hastings Gilford who was the first to refer to the disease as progeria
Progeria is a rare, fatal genetic condition characterized by an appearance of accelerated aging in children It is caused by a mutation in the gene called LMNA. The LMNA gene produces the Lamin A protein, which is the structural scaffolding that holds the nucleus of a cell together. The defective Lamin A protein makes the nucleus unstable, the instability appears to lead to the process of premature aging in Progeria.
Babies with Progeria are born looking healthy, then at 18-24 months the symptoms start to occur. Symptoms of Progeria include growth failure, loss of body fat and hair, aged-looking skin, stiffness of joints, hip dislocation, generalized atherosclerosis, cardiovascular (heart) disease and stroke. Children with Progeria die of atherosclerosis (heart disease) between the ages of 8-21. There has not been a cure for this disease yet.