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Progeria

Definition:

Progeria is a disease that produces rapid aging, beginning in childhood.



Alternative Names:
Hutchinson-Gilford syndrome

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.




Review Date: 4/20/2005
Reviewed By: Neal Sondheimer, M.D., PhD., Division of Genetics and Metabolism, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2004 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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