Progeria is also known as Hutchinson-Gilford Progeria syndrome. According to the Progeria Research Foundation, the condition is characterized by the following signs:
• Accelerated ageing from around 18 months of age
• Growth failure
• Loss of hair and body fat
• Skin that appears aged
• Stiffness of joints
• Hip dislocation
• Cardiovascular problems and strokes
• Small jaw
• Dental problems
What Causes Progeria
Progeria is extremely rare with an estimated 40 cases worldwide. The cause has been pinpointed to the mutation of a gene called LMNA. This gene produces a protein by the name of Lamin A which is described as the structural scaffolding that holds the nucleus of a cell together. The mutant form of Lamin A is called progerin and this builds up in the nucleus of cells. The nucleus becomes deformed and growth slows and stops. It particularly affects cells in blood vessels.
How does Progeria Resemble the Natural Process of Ageing
The most common cause of death in children with Progeria is atherosclerosis. This can be defined as a disease where plaque builds up and causes hardening and narrowing in arteries. The restricted blood flow may lead to death through heart attacks or strokes. Most children with Progeria do not live past their early teens.
Heart attacks and atherosclerosis are also leading causes of death in the elderly and researchers believe that finding a cure for Progeria may benefit millions of other people. Dr Leslie Gordon, The Progeria Research Foundation’s Medical Director was senior author of the study, Arteriosclerosis, Thrombosis, and Vascular Biology. When this was electronically published in August 2010, he made this comment: “By examining one of the rarest diseases in the world, we are gaining crucial insight into a disease that affects millions of people worldwide. Ongoing research has the potential to have a significant impact on our understanding of heart disease and aging.”
Is there a Cure for Progeria
At present there is no cure for Progeria. The condition is managed symptomatically but with limited success. The Mayo Clinic recommends the following treatments:
• A low dose of aspirin can help prevent heart attacks and stroke
• Physical and occupational therapy can help with mobility and joint stiffness
• High-calorie dietary supplements can ensure good nutrition and prevent weight loss
• Extraction of baby teeth before permanent teeth erupt can reduce dental problems
• Bypass surgery and angioplasty are sometimes used to slow the progression of cardiovascular disease
Progeria or Hutchinson-Gilford Progeria syndrome is extremely rare and causes premature ageing in children. Atherosclerosis is a common result of Progeria and many afflicted children die in their early teens or before. While there is no cure, research is advancing and the condition can be treated symptomatically with limited success.
Progeria Research Foundation