At 3 or 4, the hair is almost totally gone, and the skin becomes thin and wrinkled. A 'stooped' appearance develops, and the head appears large and veiny, with 'pinched' facial features. Circulatory disorders, such as heart disease and high blood pressure become characteristic around this age. The median age of death is at roughly 13 years of age. A. Cells taken from affected individuals show only a few generations of division before death, whereas the count for a healthy person is around 50 or so.
Nobody knows just what causes Progeria.
Many theories have been produced, but there is none that completely blankets all instances of the disease. In somatic cells, telomeres shorten every time they go through miotic division. When telomeres in dividing cells become too short, the cells enter a senescence stage, and initiate apoptosis or stop producing. Following that is a crisis stage, and the cells die. Shorter telomeres might be responsible for the decreased amount of divisions cells from a Progeria-affected individual can go through.