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

(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

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]
References in periodicals archive ?
Unlike many other genetic mutations, Progeria is rarely passed down in families.
A child with progeria is borne normally; dysmorphic features appear with age.
"Neighbours and extended family tormented us for not getting help for the children - they just couldn't understand a disease with no cure." Meanwhile, life was unbearable for Rehana, Ikramul, Gudiya, Rubina and Ali as they grew up with progeria.
It's never going to be fast enough while there are still children dying of progeria."
Mathers Charitable Foundation, the National Institutes of Health (R01HL123755 and 5 DP1 DK113616), The Progeria Research Foundation, The Glenn Foundation, KAUST, The Moxie Foundation, the FundaciEn Dr.
"You can't inherit it from your parents and so it's very unlikely to have two children with progeria. We knew it had happened before but with a twin from the same cell."
Biopharmaceutical company Eiger BioPharmaceuticals Inc (Nasdaq: EIGR) revealed on Wednesday the receipt of the Food and Drug Administration's (FDA) breakthrough therapy designation to expedite the development and review of lonafarnib for the treatment of Hutchinson-Gilford progeria syndrome (HGPS or Progeria) and progeroid laminopathies.
The 75-year-old had earlier donned prosthetic make-up to look like a 12-year-old boy suffering from a rare genetic disorder called Progeria in the 2009 movie 'Paa'.
Lenz Majewski Hyperostotic Dwarfism (LMHD) was first described by Braham RL, in 1969, in a paediatric patient with several clinical features identical to Camurati-Engleman syndrome, another hyperostotic disorder associated with unusual features of progeria, widened ribs, enamel dysplasia, webbed hands and brachysyndactyly.1 Another paediatric patient with strikingly similar characteristics of progeria, enamel hypoplasia, craniodiaphyseal hyperostosis, choanal atresia and symphalangism, was later reported by Lenz and Majewski.2 These malformations were subsequently compiled into a distinct entity, LMHD.
WEDNESDAY, April 25, 2018 (HealthDay News) -- Lonafarnib monotherapy is associated with lower mortality among patients with Hutchinson-Gilford progeria syndrome (HGPS), according to a study published in the April 24 issue of the Journal of the American Medical Association.
Lucy, who was eight and from Ballyward, Co Down, was diagnosed with Hutchinson Gilford Progeria Syndrome - more commonly known as Progeria - shortly after birth.
Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutation in the Lmna gene, and the mouse model of HGPS used in this study is a widely used aging model in basic research studies [13-15].