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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

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

Exams 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)
  • Generally normal cholesterol and triglyceride levels

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

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

Treatment

There is no specific treatment for progeria, but aspirin and statin medications may be used to protect against a heart attack or stroke.

Support Groups

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

Outlook (Prognosis)

Progeria causes early death. Patients usually only live to their teenage years (average lifespan of 13 years). However, some patients can live into their early 20s. The cause of death is usually related to the heart or a stroke.

Possible Complications

When to Contact a Medical Professional

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

References

Gordon LB, Brown WT, Collins FS. Hutchinson-Gilford Progeria Syndrome. 2003 Dec 12 [Updated 2011 Jan 6]. In: Pagon RA, Adam MP, Bird TD, et al., editors. GeneReviews™ [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2013.


Review Date: 9/8/2013
Reviewed By: Chad Haldeman-Englert, MD, FACMG, 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, Bethanne Black, and the A.D.A.M. Editorial team.
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