Diseases and Conditions > Cudurrada Caruurta

Progeria

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Yaxya:
Definition
Progeria is a progressive genetic disorder that causes children to age rapidly, beginning in their first two years of life. The condition is rare; since the first child with progeria was described in 1886, only about 100 cases of progeria have been documented in the scientific literature.
Children with progeria, also known as Hutchison-Gilford progeria syndrome (HGPS), generally appear normal at birth. By 6 to 12 months, signs and symptoms, such as skin changes and hair loss, begin to appear. The average life expectancy for a child with progeria is 13, but some with the disease die younger and some live longer — up to the age of 30. For at least 90 percent of children with progeria, the cause of death is a complication of the progressive stiffening of the arteries that lead to the heart and brain.
There's no cure for progeria, but ongoing research shows some promise for treatment.


Symptoms
Usually within the first year of life, growth of a child with progeria slows markedly so that height and weight fall below average for his or her age, and weight falls low for height. A child with progeria develops an appearance typical of those with the disorder, including a narrowed face and beaked nose, which makes the child look old.
Motor development and mental development remain normal. Other signs and symptoms of this progressive disorder include:
   Hair loss (alopecia), including eyelashes and eyebrows
   Hardening and tightening of skin on trunk and extremities (scleroderma)
   Loose, aged-looking skin
   Head too large for face
   Prominent scalp veins
   Prominent eyes
   Small lower jaw (micrognathia)
   High-pitched voice
   Delayed and abnormal tooth formation
   Loss of body fat and muscle
   Stiff joints
   Hip dislocation.
                                                                       To be continued…….

Dr.Farabadan:
 well, it's very informative indeed.

This HGPS disease has no known cure, but a reseach suggested a unti-cancer drugs farnesyltransferase inhibitors can cure this kind of disorder. I think it's not confirmed yet.

PrincessMuni:
intersting, never read of this disease, i saw it on tv long time ago
so did u ever see such case?

Dr.Farabadan:

I ve never seen it But I read it many times

Yaxya:
me too i've never seen such case be4

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