Progeria

 

Progeria, or Hutchinson Gilford Progeria syndrome, is an extremely rare, fatal genetic condition of childhood with striking features resembling premature aging. The word Progeria is derived from the Greek meaning “prematurely old”. Hutchinson Gilford Progeria accelerates the process of aging to about eight times the normal rate. Because of this accelerated aging, a child with Hutchinson Gilford Progeria that is ten years old will have similar respiratory, cardiovascular, and arthritic conditions of that of an eighty year old.

Hutchinson Gilford Progeria has a reported incidence of about 1 in 8 million newborns. It affects both sexes equally and all races. In the past 15 years, children with Progeria have been reported all over the world, including in Algeria, Argentina, Australia, Austria, Canada, China, Cuba, England, France, Germany, Israel, Italy, Mexico, the Netherlands, Poland, Puerto Rico, South Africa, South America, South Korea, Switzerland, Turkey, the US, Venezuela, Vietnam, and Yugoslavia. Hutchinson Gilford Progeria is one of the world’s rarest diseases. There are only about 40 known cases throughout the world to this date. There has been only about 100 reported cases since the first diagnosis by Jonathan Hutchinson in 1886 and Hastings Gilford in 1904.

Children with Progeria usually have a normal appearance in early infancy. At about 9-24 months of age, children with Progeria begin to experience profound growth delays, resulting in short stature and low weight. These children also develop a distinctive facial appearance characterized by a disproportionately small face in comparison to the head, an undeveloped jaw, and malformation and crowding of the teeth, abnormally prominent eyes, a small nose and a subtle blueness around the mouth. By the second year of life, children with Progeria lose their hair from the scalp, lose their eyebrows and eye lashes. Small, downy, white or blonde hairs may replace the scalp hair. Hutchinson Gilford Progeria is extremely difficult to diagnose in newborns, but certain suspicious findings may be present at birth, such as unusually taut, shiny, hardened skin over the buttocks, upper legs and lower abdomen, and bluish discolouration of the skin around the mouth. Profound, progressive growth retardation usually becomes evident by approximately 9-24 months of age. According to reports in the medical literature, affected children who are 10 years of age typically have a height of that of an average 3 year old.

Although the time of onset of more definitive symptoms varies from case to case, generally by the end of the first year there is noticeable growth retardation and the appearance of physical features commonly associated with Hutchinson Gilford Progeria.

Progeria is a life limiting disease that results in the Progeria children dying of heart disease and other ageing related problems at an average age of 13, with a range of about 8-21 years of age.

 Posted by at 9:17 am

  5 Responses to “Progeria”

  1. I am trying to bring an awareness to all I meet and my friends friends …about Progeria.It has become sso important to me. it all started with Hayley Okines and TV shows and videos I have watched about her life. She stirred something inside me and touched my heart. i now in my small way am trying to help spread the word about these awesome kids..and help find a cure with any way i know how. God bless All of Them !!

  2. i love your daughter, she’s so inspiring.. i would love to meet her! but i live in the u.s.! which is far. i think she’s the most beautiful girl in the world. your so lucky, your blessed with an angel! i hope you could email me back sometime!(: god bless your daughter!

  3. I pray and when i do this time my prayers will go to all new born and all children in all parts of the world for Christ Jesus to take each
    one under his care and leave them with his blessing ,health and that a cure he can find for those who need.I ask from this day forward and with prayers from all who have faith , that our Lord will take our prayers to God.in Jesus name.

  4. chloe i pray for her and every one and that she gets well!

  5. I’m only 12 years old and i want to know more about this and i just want you kids with Progeria that i think you are the most beautiful thing i have ever layed eyes on 🙂

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