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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.

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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Morristown Medical Center

100 Madison Avenue
Morristown, NJ 07960
973-971-5000

Overlook Medical Center

99 Beauvoir Avenue
Summit, NJ 07901
908-522-2000

Newton Medical Center

175 High Street
Newton, NJ 07860
973-383-2121

Chilton Medical Center

97 West Parkway
Pompton Plains, NJ 07444
973-831-5000

Goryeb Children's Hospital

100 Madison Avenue
Morristown, NJ 07960
973-971-5000

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Atlantic Medical Group

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