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Coronary artery blockage
Coronary artery blockage


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.

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:

Signs and tests:

The signs include:

  • Skin changes similar to that seen in scleroderma (the connective tissue becomes tough and hardened)
  • Insulin-resistant diabetes (diabetes that does not respond readily to insulin injections)

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

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



Treatment:

There is presently no treatment for progeria.



Support Groups:

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



Expectations (prognosis):

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.



Complications:

Calling your health care provider:

Call for an appointment with your health care provider if you child does not appear to be growing or developing normally.



Prevention:

There is no known prevention.




Review Date: 7/2/2007
Reviewed By: Brian Kirmse, MD, Mount Sinai School of Medicine, Department of Human Genetics, New York, NY. 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 medical professional 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. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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Wentworth-Douglass Hospital
789 Central Avenue, Dover, NH 03820
Phone: (603) 742-5252
Toll free: 1 (877) 201-7100