What is Progeria ?

Progeria is an extremely rare autosomal dominant genetic disorder in which symptoms resembling aspects of aging are manifested at a very early age. Progeria is one of several progeroid syndromes.

Those born with progeria typically live to their mid-teens to early twenties. It is a genetic condition that occurs as a new mutation, and is rarely inherited, as carriers usually do not live to reproduce children. It is believed to affect 1 in every 4 million newborns of both sexes and all ethnicities. The disease can lead to fatal heart complications and a heightened risk of stroke.Progeria is incurable, but symptoms can be managed.A drug called Ionafarnib can extend the average 14 year life expectancy by 1.6 years.

Progeria was first described in 1886 by Jonathan Hutchinson.It was also described independently in 1897 by Hastings Gilford.The condition was later named Hutchinson-Gilford progeria syndrome.Scientists are interested in progeria partly because it might reveal clues about the normal process of aging.

Other similar syndromes

There are other progeroid syndromes that do run in families. These inherited syndromes cause rapid aging and a shortened life span:

• Wiedemann-Rautenstrauch syndrome, also known as neonatal progeroid syndrome, starts in the womb, with signs and symptoms of aging apparent at birth.
• Werner syndrome, also known as adult progeria, begins in the teen years or early adulthood, causing premature aging and conditions typical of old age, such as cataracts and diabetes.