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Progeria
Definition:
Progeria is a disease that produces rapid aging, beginning in childhood. Alternative Names:
Hutchinson-Gilford syndrome Text Continues Below
Causes, incidence, and risk factors:
Progeria is a rare condition that is remarkable because its symptoms strongly resemble normal human aging, but occur in young children. In addition, the appearance of several affected children in movies and on television have brought progeria to public attention. Ninety percent of children with progeria have a mutation on the gene that encodes the protein lamin A. Progeria usually occurs sporadically, and is not seen in siblings of affected children, although -- in very rare circumstances -- more than one child in a family can have progeria. Progeria results in rapid aging of a child, beginning with growth failure in the first year of life. This results in a disproportionately small body given the size of the head. Progeria children (male and female) are thin and balding, with wizened narrow faces and old-appearing skin. Children with progeria develop early atherosclerosis. The average patient survives to the early teens. However, some patients can live up to 30 years. The cause of death is usually related to the heart or a stroke as a result of the progressive atherosclerosis.
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