Progeria

A rare genetic disease in which a person ages prematurely and dies young.


A condition of premature ageing.


Progeria is a rare genetic disorder characterized by an appearance of accelerated aging. Its name is derived from the Greek, meaning prematurely old. The classic type is the Hutchinson-Gilford Progeria syndrome, which was first described in England in 1886 by Dr. Jonathan Hutchinson and again in 1886 and 1904 by Dr. Hastings Gilford. Some progeroid syndromes manifest symptoms of premature aging at birth, such as Bamatter-Francescetti syndrome, Berardinelli-Seip syndrome, De Barsy syndrome, Hallerman-Streiff syndrome, Von Lohuizen syndrome, and Wiedemann-Rautenstrauch syndrome. Others become clinically apparent later in life, such as Cockayne syndrome, Hutchinson-Gilford progeria syndrome, and Werner syndrome. Progeroid syndromes occur in about 1 per 4 million births. They affect both sexes in equal proportion and have been observed in all races.


Rare, abnormal condition characterized by premature aging and the appearance of gray hair, wrinkled skin, and the posture of an aged person in a child or adolescent. The cause is unknown.


A rare genetic disease that causes premature aging in young children. Symptoms include severe growth retardation in infancy, baldness, a widened face, large head for face size, small jaw, loss of eyebrows and eyelashes, limited range of motion, skeletal abnormalities such as osteoporosis, and thin, dry skin. Intelligence is unaffected. There is no treatment for progeria, and death usually occurs in the early teenage years because of severe arteriosclerosis of the coronary arteries, a disorder that causes thickening and loss of elasticity in the heart’s arterial walls.


A very rare condition in which all the signs of old age appear and progress in a child, so that ‘senility’ is reached before puberty.


The syndrome of premature aging, which may be an inherited disorder that is transmitted as an autosomal dominant trait. The incidence appears higher in children of older fathers. Onset is from birth to 18 months of age and the average age at death is 12 to 13 years.


Accelerated aging. There are two types of progeria, both of which are exceedingly uncommon.


Hutchinson-Gilford syndrome initiates premature aging around the age of four, with numerous signs of old age like grey hair, balding, skin sagging, and atherosclerosis (accumulation of fatty deposits in arteries) emerging between ten to twelve years of age. Typically, individuals with this condition succumb to it during puberty. Werner’s syndrome, known as adult progeria, commences during adolescence or early adulthood and follows a similar trajectory.


Hutchinson-Gilford syndrome is attributed to a gene mutation, whereas the cause of progeria remains unidentified.


An aged appearance at a young age, occasionally linked to pituitary dwarfism.


 


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