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Alternative names Return to top
Hutchinson-Gilford syndromeDefinition Return to top
Progeria is a disease that produces rapid aging starting in childhood.Causes, incidence, and risk factors Return to top
Progeria is a rare condition but has come into public awareness because its symptoms strongly resemble normal human aging as well as the appearance of several affected children in movies on national television.
Lamin A is the name of the gene recently identified as causing some types of progeria. Lamin A codes for a protein that surrounds the nucleus of cells. Further study of lamin A will hopefully answer why mutations in this gene cause such striking premature aging.
Progeria results in rapid aging of children, beginning with growth failure during the first year of life that results in disproportionately small bodies given the size of their heads. The children are thin with baldness, wizened narrow faces, and old-appearing skin.
Children with progeria develop early atherosclerosis. The average lifespan is 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.
Symptoms Return to top
Signs and tests Return to top
The signs include:Treatment Return to top
There is presently no treatment for progeria. Support groups are available for the families of children with progeria.Support Groups Return to top
Progeria Research Foundation, Inc.
P.O. Box 3453
Peabody, MA 01961-3453
Phone: 978-535-2594
www.progeriaresearch.org
progeria@netzero.net
Expectations (prognosis) Return to top
Progeria is associated with a short lifespan.Complications Return to top
Calling your health care provider Return to top
Call for an appointment with your health care provider if you child does not appear to be growing or developing normally.Prevention Return to top
There is no prevention for progeria per se, but prevention of some of the manifestations of the disorder relies on the same medical approaches for these complications in other disorders. Update Date: 8/27/2003 Updated by: Douglas R. Stewart, M.D., Division of Medical Genetics, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |