Progeria

Hutchinson–Gilford syndrome or progeria (derived from pro, before, and geras, old age) is characterized by premature ageing (Fig. 18.1) and the early onset of age-related symptoms such as joint restriction and cerebral and myocardial infarction. Progeria occurs in about one in eight million people (DeBusk, 1972), but the true incidence may be somewhat higher (Sarkar and Shinton, 2001).

Hutchinson-Gilford progeria is a syndrome commonly characterized by accelerated aging. Children affected by progeria have a life expectancy of approximately 13 years. Progeria results from damage to the LMNA gene that codes for the protein lamin A.

Hutchinson Gilford progeria syndrome (progeria) is a rare childhood disease, affecting 1 in 4 million births worldwide (Hennekam, 2006) and recapitulates certain aspects of the normal aging process at an accelerated rate.