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 ?
This instability then appears to pave the way to the process of premature aging in Progeria.
Despite these sour notes, "Salamin" did move and enlighten viewers with a number of effective and insightful scenes, like the one right after Rochelle's 15th birthday, when she was so happy to realize that she was still alive (youths stricken with progeria generally don't make it to their midteens)
Progeria is extremely rare--about 100 patients worldwide have been diagnosed--and always fatal at an early age.
They also plan to use the smooth muscle cells derived from human induced pluripotcnt stem cells to test new treatments for progeria.
Conclusion: Es fundamental para el odontopediatra saber reconocer las manifestaciones clinicas de la Progeria, para asi poder comenzar con la aplicacion de terapeuticas preventivas y tratamientos oportunos.
Together, they positively impacted the lives of people around the world in their quest to find a cure for progeria.
A case report of 6-year-old boy with progeria syndrome, with marked cardiac complications is presented.
ISLAMABAD -- Changes in cholesterol may speed heart disease in children affected by the premature aging disease progeria, researchers report.
Harry, 14, suffers from a rare form of Progeria, a genetic disorder which means that he is ageing five times faster than his contemporaries.
The second possibility is that Ata, the size of a 22-week-old foetus, was suffering from a severe form of a rare rapid ageing disease, progeria, and died in the womb or after premature birth.