Medical Marvels: Progeria Children

Progeria (also known as "Hutchinson-Gilford progeria syndrome") is an extremely rare genetic condition where symptoms resembling aspects of aging are manifested at an early age. About 1 in 8 million babies are born with this condition, and most affected children have a life span of around 13 years, although many have been known to live into their late teens and early twenties. It is a genetic condition, but occurs as a new mutation (sporadically) and is not usually inherited, although there is one unique form of the condition identified in only one family in the world that is inherited from parents who carry the protein genetically. In this case, five children in the family had the condition.

This is in contrast to another rare but similar premature aging syndrome, dyskeratosis congenita (DKC), which is inheritable and will often be expressed multiple times in a family line. Scientists are particularly interested in progeria because it might reveal clues about the normal process of aging.

The earliest symptoms include failure to thrive and a localized scleroderma-like skin condition.

As the child ages past infancy, additional conditions become apparent. Limited growth, alopecia, and a distinctive appearance (small face and jaw, pinched nose) are all characteristic of progeria. The people diagnosed with this disease usually have small, fragile bodies, like those of elderly people. Later, the condition causes wrinkled skin, atherosclerosis, and cardiovascular problems.