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Progeria


Definition:

Progeria is a rare genetic condition that produces rapid aging in children.

Alternative Names:

Hutchinson-Gilford progeria syndrome; HGPS

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. It usually is not passed down through families. Rarely is it seen in more than one child in a family.

Symptoms:
Signs and tests:

The health care provider will perform a physical exam and order laboratory tests. This may show:

  • Insulin resistance
  • Skin changes similar to that seen in scleroderma (the connective tissue becomes tough and hardened)

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

Genetic testing can detect changes in the gene that causes progeria.

Treatment:

There is no specific treatment for progeria.

Support Groups:

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

Expectations (prognosis):

Progeria causes early death. Patients usually only live to their teenage years. However, some patients can live into their early 20s. The cause of death is usually related to the heart or a stroke.

Complications:
Calling your health care provider:

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

References:

Brown WT. Progeria. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 90.


Review Date: 8/4/2011
Reviewed By: Chad Haldeman-Englert, MD, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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