Progeria - Premature Aging Syndrome

Progeria (ancient Greek προσ - over, γέρων - old man) is one of the rarest genetic diseases that causes premature aging of the body.

Child progeria (Hutchinson's (Hutchinson's) -Gilford syndrome) and adult progeria (Werner's syndrome) are classified.

Experts do not classify Progeria as a hereditary syndrome, since it is the result of a completely random and very rare change in genes. In the world, no more than 80 cases of progeria have been recorded, and among the patients there was only one representative of the Negroid race.

Newborn babies with Progeria appear healthy. When they reach the age of 10-24 months, they begin to show signs of accelerated aging. The average life expectancy in childhood progeria is 13 years. Most sources indicate the age of death from 7 to 27 years old, while cases of reaching the age of majority are very rare. There is only one known case when a patient from Japan, sick with protegeria, lived for 45 years.

In adults, the disease manifests itself during puberty. Slow growth, symptoms of hypogonadism are noted. Usually, in the third decade of life, the patient turns gray and hair falls out, cataracts develop, the skin gradually becomes thinner and the subcutaneous tissue on the face and limbs atrophies, as a result of which the arms and especially the legs become thin.

The cause of death of patients with protegeria, as a rule, is such senile ailments as diseases of the cardiovascular system and brain tissue.

The severe form of human progeria, Hutchinson-Guildford syndrome, has been found to be associated with molecular changes that are characteristic of normal aging, such as genomic instability, decreased telomere length, and impaired stem cell homeostasis. Unfortunately, at the moment, the disease is poorly understood and a cure for it has not yet been found.