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Progeria

Contents of this page:

Illustrations

Coronary artery blockage
Coronary artery blockage

Alternative Names    Return to top

Hutchinson-Gilford syndrome

Definition    Return to top

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

Causes    Return to top

Progeria is a rare condition that is remarkable because its symptoms strongly resemble normal human aging, but occur in young children.

Ninety percent of children with progeria have a mutation on the gene that encodes the protein lamin A. Progeria usually occurs without cause. It is not seen in siblings of affected children, although -- in very rare circumstances -- more than one child in a family can have the condition.

Symptoms    Return to top

Exams and Tests    Return to top

The signs include:

Cardiac stress testing may reveal signs of early atherosclerosis of blood vessels.

Genetic testing can detect mutations in lamin A that cause progeria.

Treatment    Return to top

There is presently no treatment for progeria.

Support Groups    Return to top

Progeria Research Foundation, Inc. -- www.progeriaresearch.org

Outlook (Prognosis)    Return to top

Progeria is associated with a short lifespan. 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.

Possible Complications    Return to top

When to Contact a Medical Professional    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 known prevention.

Update Date: 7/2/2007

Updated by: Brian Kirmse, MD, Mount Sinai School of Medicine, Department of Human Genetics, New York, NY. Review provided by VeriMed Healthcare Network.

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