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An Introduction

BIOL. 2402 - ANATOMY  & PHYSIOLOGY
RICHLAND COLLEGE
SPRING 2001
Class Project
Web project members:
  • Dina Hunter
  • Katie Morgan


  •       Progeria is a rare genetic disease resulting in accelerated aging. The name stems from the Greek, and means "prematurely old" .  Progeria occurs in two forms. Hutchinson -Gilford progeria syndrome was first described by English surgeons Jonathan Hutchinson in 1886 and by Hastings Gilford in 1904. It is known as the progeria of childhood and is usually diagnosed in the first or second year of life. Werner syndrome is commonly referred to as "progeria of the adult." It was first described in 1904 by general practitioner Otto Werner. Both conditions result in the appearance of symptoms, which normally occur in people who are seventy years of age or more("The Progeria Research Foundation").

        There are symptomatic similarities between both forms of progeria but there are demographic differences. Hutchinson- Gilford syndrome occurs in the first decade of life with an average life span of twelve years, although some have lived past twenty. There are a reported 1 in 8 million incidences and an estimated 1 in 4 million incidences worldwide. Since its discovery over a century ago, there have been over 100 cases reported. There are 30 to 40 known cases at the present time (MacMichael). Werner syndrome is more common with estimated cases between 1 in 100,000 and 1 in 1,000,000. Werner's appears at the onset of puberty and the average life span is 20-30 years. All forms of progeria show no discrepancy between sex or race. In this decade there have been cases reported in Algeria, Australia, Austria, Canada, Chile, China, Egypt, England, France, Germany, Holland, Libya, Mexico, The Netherlands, Puerto Rico, South Africa, South America, Switzerland, Turkey, the United States, Venezuela, and Vietnam ("The Progeria Research Foundation") .

        Many Americans may recall seeing the children affected with Hutchinson-Gilford on television series, such as the Maury Povich Show, or perhaps, they may recall the news media reports in 1981 of the first meeting of two affected children. Wherever you may have seen these wonderful people it is hard not to notice their small stature, baldness, and aged looking skin. Despite their outward appearance, these children are happy, self-confident, and possess a spirit that serves as an example to all.

        The self-esteem and mental strength these children have is largely due to those who have dedicated their lives to research and to those who reach out to lend a helping hand. THE PROGERIA RESEARCH FOUNDATION  is involved in raising money and doing research into a definitive cause, treatment, and cure. Another institution that deserves recognition is the  SUNSHINE FOUNDATION  The Sunshine Foundation makes dreams come true for children with diseases such as progeria. They organize Disney trips for the children and sponsor annual reunions for those with progeria and their families. With the help of these organizations, the patients with this condition have support and hope for the future.

    CELLULAR MECHANISMS

    HUTCHINSON-GILFORD SYNDROME

    WERNER SYNDROME

    GENETIC INHERITANCE OF THESE DISEASES

    CAUSES OF DEATH

    THERAPY

    References:
    Wynbrandt, James and Mark D. Ludman.  The Encyclopedia of Genetic Disorders and Birth Defects 1991. Facts on File, Inc.: New York

    MacMichael, Ryan.  "Hutchinson-Gilford Progeria Syndrome Resource Center."     1997.  World Wide Web.  8 March 2001  www.laze.net/progeria/

    "National Organization of Rare Disorders, Inc."  World Wide Web. 20 February 2001. www.stepstn.com

    "The Scientific American."  1997. World Wide Web. 8 March 2001.
     www.sciam.com/askexpert/medicine/medicine13.html

    "Progeria_uk."  World Wide Web.  20 February 2001.http://privat.schlund.de/p/progeria_uk.html

    "The Progeria Research Foundation, Inc." World Wide Web. 8 March 2001.
     www.progeriaresearch.org

    "The Sunshine Foundation."  2001. World Wide Web. 2 February 2001.
     www.sunshinefoundation.org



    For feedback:
    mailJackie Reynolds (Professor of Biology, Richland College)

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