Progeria, or Hutchinson-Gilford syndrome (pro-jerry-ya)
General: This disease causes people to age at about seven times the normal rate. This means that a child of ten would not only look like a miniature 70 year old, but would have all of the health problems associated with old age. Average life expectancy of a child with Hutchinson-Gilford Progeria Syndrome (HGPS) is 16 years of age, although one or two have lived to be twenty.
Chromosomal cause: This is an extremely rare disease, and only about 40 children are known to have it worldwide. It is unknown which cromosome this syndrome is located on, but it is believed to be on either chromosome 1 or 8. Chromosome 1 is more likely, but a similar disease (Werner's Syndrome) is located on chromosome 8. This disease is definitely autosomal though (not the X or Y chromosome). We know this because both boys and girls get this in equal numbers. The disease appears to be spontaneous. It is not received because a faulty gene was passed from parent to child, but it is a new mutation. This means also that it is a dominant trait. It is not localized to any ethnic group.
Characteristics: A child born with HGPS will usually start life looking fairly ordinary. They grow rapidly during the first year, but then start falling behind as far as growth is concerned. They then start to take on the appearance of miniature adults. They may lose all their hair, their bones get brittle, their skin starts to wrinkle and sag. This gives them the appearance of having large, wise eyes. Their teeth all come in at first, crowding each other. The teeth may fall out. They start having heart problems and strokes early. They may develop eye problems associated with age. Heart attacks are the most common cause of death.
Progeria, or Hutchinson-Gilford syndrome (pro-jerry-ya)
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