progeria


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pro·ge·ri·a

(prō-jē'rē-ă), [MIM*176670]
A condition of precocious aging with onset at birth or early childhood; characterized by growth retardation, a senile appearance with dry wrinkled skin, total alopecia, and birdlike facies; early occurrence of atherosclerosis in blood vessels and premature death due to coronary artery disease; genetics unclear.
[pro- + G. gēras, old age]

progeria

/pro·ge·ria/ (-jēr´e-ah) premature old age, a condition occurring in childhood marked by small stature, absence of facial and pubic hair, wrinkled skin, gray hair, and eventual development of atherosclerosis.

progeria

(prō-jîr′ē-ə)
n.
A rare genetic disorder of childhood that is characterized by rapid onset of the physical changes typical of old age, usually resulting in death before the age of 20. Also called Hutchinson-Gilford progeria syndrome.

progeria

[prōjir′ē·ə]
Etymology: Gk, pro + geras, old age
an abnormal congenital condition characterized by premature aging, appearance in childhood of gray hair and wrinkled skin, small stature, absence of pubic and facial hair, and posture and habitus of an aged person. Death usually occurs before 20 years of age. Also called Hutchinson-Gelford syndrome. Compare infantilism.
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Progeria

progeria

Pediatrics A condition characterized by rapid premature aging of childhood onset, in which morbidities usually seen in the elderly–eg, ASHD, CAD, cataracts, wrinkled skin, appear during puberty and death from 'old age' occurs by age 20 from heart disease or strokes Clinical Growth failure in 1st yr of life; affected children are small and thin with disproportionately large appearing heads, baldness, wizened narrow faces, and aged skin; congenital progerias–eg, Cockayne syndrome, Hutchinson-Gilford syndrome.

pro·ge·ri·a

(prō-jēr'ē-ă)
A condition in which normal development in the first year is followed by gross retardation of growth, with a senile appearance characterized by dry, wrinkled skin, total alopecia, and birdlike facies; genetics unclear.
Synonym(s): Hutchinson-Gilford disease.
[pro- + G. gēras, old age]

progeria

Premature ageing. There are two types: Hutchinson-Gilford syndrome and Werner's syndrome. In the former, a child of 10 may show all the characteristics of old age-baldness, grey hair, wrinkled skin, loss of body fat and degenerative diseases of the arteries. In the latter, the disease starts in adult life and runs a rapid course over about 10 years. Both types are now believed to result from single spontaneous gene mutations. In HG syndrome the mutation is in the gene coding for lamin A, an important structural protein. In Werner's syndrome the mutation is in a helicase-a gene that unwinds double-strand DNA into two single strands. From the Greek pro , before and geras , old age.

Hutchinson,

Sir Jonathan, English surgeon and pathologist, 1828-1913.
Hutchinson crescentic notch - the semilunar notch on the incisal edge of Hutchinson teeth, encountered in congenital syphilis.
Hutchinson disease - age-related guttate choroiditis.
Hutchinson facies - the peculiar facial expression produced by drooping eyelids and motionless eyes in external ophthalmoplegia.
Hutchinson freckle - a brown or black mottled, irregularly outlined, slowly enlarging lesion. Synonym(s): lentigo maligna
Hutchinson mask - the sensation experienced in tabetic neurosyphilis as if the face were covered with a mask or with cobwebs.
Hutchinson patch - interstitial or parenchymatous keratitis giving rise to neovascularization of the cornea. Synonym(s): salmon patch
Hutchinson pupil - dilation of the pupil on the side of the lesion as part of a third nerve palsy.
Hutchinson teeth - the teeth of congenital syphilis in which the incisal edge is notched and narrower than the cervical area. Synonym(s): notched teeth; screwdriver teeth; syphilitic teeth
Hutchinson triad - parenchymatous keratitis, labyrinthine disease, and Hutchinson teeth, significant of congenital syphilis.
Hutchinson-Gilford disease - a condition in which normal development in the first year is followed by gross retardation of growth, with dry wrinkled skin, total alopecia, and birdlike facies. Synonym(s): Hutchinson-Gilford syndrome; progeria
Hutchinson-Gilford syndrome - Synonym(s): Hutchinson-Gilford disease

pro·ge·ri·a

(prō-jēr'ē-ă) [MIM*176670]
A condition of precocious aging with onset at birth or early childhood.
[pro- + G. gēras, old age]

progeria (prōjir´ēə),

References in periodicals archive ?
Prof Hutchinson added: "We are using a careful approach that will look at patients with Progeria to see if there is a model that can be used for wider medicine.
Ted Brown, the nation's leading progeria researcher and chairman of the Department of Human Genetics at the New York State Institute for Basic Research on Staten Island.
Their team examined a stem/progenitor cell population derived from the muscle of progeria mice and found that compared to those from normal rodents, the cells were fewer in number, did not replicate as often, didn't differentiate as readily into specialized cells and were impaired in their ability to regenerate damaged muscle.
As previously demonstrated by Nicolas Levy's team - which originally identified the gene in 2003 - progeria is due to the accumulation in the cells of a truncated protein, progerin, whose toxicity is linked to the presence of a fatty acid which remains fixed to the protein (whilst it is eliminated in normal cells).
Progeria is an aggressive disease that ages sufferers eight years for every year of their life.
This delightful little girl suffers from progeria, which makes her age prematurely but she is a real little ray of sunshine.
Then there's the courage and grace of 16-year-old Denny Sim, a student at Monroe High School in North Hills, bravely dealing with progeria, an accelerated aging disease.
Collins' own research laboratory has discovered a number of important genes, including those responsible for cystic fibrosis, neurofibromatosis, Huntington's disease, a familial endocrine cancer syndrome, and most recently, genes for adult onset (type 2) diabetes and the gene that causes Hutchinson-Gilford progeria syndrome.
She was diagnosed with the genetic condition progeria as a toddler and doctors warned she was unlikely to live past the age of 13.