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Progeria is an extremely rare genetic condition which causes physical changes that resemble greatly accelerated aging in sufferers. It is an autosomal recessive condition that affects around 1 in 8 million newborns. Currently, approximately forty people have been diagnosed with this disease. The average lifespan of a child with Progeria is 8-21 years.
Progeria is not inherited. It develops during cell division in a newly conceived child. It is on the LMNA (Lamin A protein) gene on chromosome 1.
The condition was first identified in 1886 by Jonathan Hutchinson and Hastings Gilford. The condition was later named Hutchinson-Gilford Progeria syndrome; the name was derived from the Greek for "prematurely old". Around 100 cases have been definitely identified since then.
The condition is distinguished by limited growth, alopecia and a characteristic appearance with small face and jaw and pinched nose. Later the condition causes wrinkled skin, atherosclerosis and cardiovascular problems. Mental development is unaffected. Individuals with the condition rarely live more than 16 years; the longest recorded life-span is 26 years. The development of symptoms is comparable to aging at a rate six to eight times faster than normal, although certain age related conditions do not occur.
Compare: Werner syndrome
External links
- [1] (http://asylumeclectica.com/malady/archives/progeria.htm) not a medical site but explains the disease in layman's terms
- Progeria Research Foundation (http://www.progeriaresearch.org/)
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