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Progeria

Hutchinson-Gilford progeria syndrome; HGPS

 

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

Causes

 

Progeria is a rare condition. It is remarkable because its symptoms strongly resemble normal human aging, but it occurs in young children. In most cases, it is not passed down through families. It is rarely seen in more than 1 child in a family.

 

Symptoms

 

Symptoms include:

  • Growth failure during the first year of life
  • Narrow, shrunken or wrinkled face
  • Baldness
  • Loss of eyebrows and eyelashes
  • Short stature
  • Large head for size of face (macrocephaly)
  • Open soft spot (fontanelle)
  • Small jaw (micrognathia)
  • Dry, scaly, thin skin
  • Limited range of motion
  • Teeth - delayed or absent formation

 

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. Aspirin and statin medicines 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. People with the condition most often only live to their teenage years (average lifespan of 13 years). However, some can live into their early 20s. The cause of death is very often related to the heart or a stroke.

 

Possible Complications

 

Complications may include:

  • Heart attack (myocardial infarction)
  • Stroke

 

When to Contact a Medical Professional

 

Call your provider if your child does not appear to be growing or developing normally.

 

 

References

Gordon LB, Brown WT, Collins FS. Hutchinson-Gilford progeria syndrome. Gene Reviews. Seattle, WA: University of Washington: 2015:1. PMID: 20301300 www.ncbi.nlm.nih.gov/pubmed/20301300. Accessed August 1, 2015.

 
  • Coronary artery blockage

    Coronary artery blockage - illustration

    Atherosclerosis is a common disorder of the arteries. Fat, cholesterol, and other substances collect in the walls of arteries. Larger accumulations are called atheromas or plaque and can damage artery walls and block blood flow. Severely restricted blood flow in the heart muscle leads to symptoms such as chest pain.

    Coronary artery blockage

    illustration

    • Coronary artery blockage

      Coronary artery blockage - illustration

      Atherosclerosis is a common disorder of the arteries. Fat, cholesterol, and other substances collect in the walls of arteries. Larger accumulations are called atheromas or plaque and can damage artery walls and block blood flow. Severely restricted blood flow in the heart muscle leads to symptoms such as chest pain.

      Coronary artery blockage

      illustration


     

    Review Date: 8/1/2015

    Reviewed By: Chad Haldeman-Englert, MD, FACMG, Fullerton Genetics Center, Asheville, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, 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. 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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