Progeria – “accelerated aging disease”
Progeria – “accelerated aging disease”
Imagine for a second that you are 10 years old. Your friends call you up to go out and play but you can't because you have a painful case of arthritis that hurts with every step you take. Or, your chronic heart disease disables you from being able to breathe easily. These conditions are just a few that a child with Progeria may suffer from. Progeria is also known as Hutchinson-Gilford Syndrome. It is an extremely rare aging disease that causes a child to age up to eight times faster than normal. It is also know as the “accelerated aging diseases. I first became interested in Progeria when I have read it from an article in Nature.The disease affects between 1 in 4 million and 1 in 8 million newborns. Currently, there are 51 known cases in the world. Most people with progeria die at around 13 years of age.
The condition was first identified in 1886 by Jonathan Hutchinson and in 1987 by Hastings Gilford. The condition was later named Hutchinson-Gilford Progeria syndrome (HGPS). However until now scientist have yet found a medicine for this condition. In the beginning, Progeria was studied as a disease that included the process of normal aging, just at an incredible rate. This is no longer be true because Progeria does not affect all of the body's organs. For instance, there is no evidence of aging in the central nervous system. A few are mentally retarded, but most have normal intelligence and may even be advanced. A child with Progeria is just a normal seven-year-old in a 70-year-old body!
The cause of this rare disorder is not yet fully understood. In 2003, researchers announced the discovery of a single gene mutation responsible for Hutchison-Gilford progeria syndrome. The gene is known as lamin A (LMNA), which makes a protein necessary to holding the center (nucleus) of a cell together. Researchers believe the genetic mutation renders cells unstable, which appears to lead to progeria's characteristic aging process. Unlike many genetic mutations, Hutchison-Gilford progeria isn't passed down in families. Rather, the gene change is a chance occurrence that researchers believe affects a single sperm or egg just before conception. Neither parent is a carrier, so the mutations in the children's genes are new (de novo).
Individuals with Progeria appear normal at birth. It is not until about the age of one that signs become evident. Growth is extremely stunted, and they may only reach the height of a normal two or three-year-old. It is very unlikely that they will grow taller than a normal five-year-old will, even though they have the physical characteristics of a 60-year-old.
According to the Hutchinson-Gilford entry of the Electronic Textbook of Internal Medicine, there are many signs to detect the presence of Progeria. These signs include: (visual aide) baldness, prominent scalp veins, beaked nose, receding chin, prominent eyes, a narrow chest, a protruding abdomen, an enlargement of the spleen, and the absence of eyebrows and lashes. The sex organs also remain small and underdeveloped, and they are not able to reproduce. They often have relatively large heads with small faces and crowded irregular teeth. Individuals with Progeria often develop diseases related to aging, such as arthritis and heart disease. Another common cause of death is angina pectoris, which is a condition in which there is a sudden decrease of blood in the heart muscle. Progeria is extremely rare. In my speech, I have informed you of the extremely rare aging disease known as Progeria.
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