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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. 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
  • 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
  • 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
  • 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.