Progeria


Progeria
A disorder characterized by premature aging. There is more than one type of progeria, and the mechanism behind these disorders is not yet known. Researchers have recently found dramatically lowered levels of antioxidant enzymes, particularly catalase and glutathione peroxidase, in patients with progeria. One theory is that lower levels of these enzymes permit cell damage by toxins and free radicals. There is currently no treatment for progeria, although gene therapy is being investigated.
* * *
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 vessel s and premature death due to coronary artery disease; genetics unclear. SYN: Hutchinson-Gilford disease, Hutchinson-Gilford syndrome, premature senility syndrome. [pro- + G. geras, old age]
- p. with cataract, p. with microphthalmia SYN: dyscephalia mandibulo-oculofacialis.

* * *

pro·ge·ria prō-'jir-ē-ə n a rare genetic disorder of childhood marked by slowed physical growth and characteristic signs (as baldness, wrinkled skin, and atherosclerosis) of rapid aging with death usu. occurring during puberty

* * *

n.
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.

* * *

pro·ger·ia (pro-jērґe-ə) [pro- + ger- + -ia] an autosomal dominant syndrome of precocious senility, with affected children developing growth retardation, thinning skin, and fragile bones as young as 18 months, and death from stroke or coronary artery disease usually occurring by 13 years of age. It is caused by mutation in the LMNA gene (locus: 1q21.2), which encodes lamin A; the mutation causes partial activation of a cryptic splice site, leading to production of a truncated precursor to lamin A, called progerin, which accumulates at the nuclear envelope, dusrupting the nuclear scaffolding. Cf. infantilism. Called also Hutchinson-Gilford syndrome.

Medical dictionary. 2011.

Look at other dictionaries:

  • Progeria — Progéria Enfant atteint de progéria (l image en haut à droite représente un noyau cellulaire normal, celui d en bas, un noyau déformé au cours d une progéria). La progéria, ou Syndrome de Hutchinson Gilford, est une maladie génétique extrêmement… …   Wikipédia en Français

  • Progeria — Niño con Sindrome de Progeria Clasificación y recursos externos CIE 10 …   Wikipedia Español

  • progeria — f. patol. Enfermedad hereditaria poco frecuente que se caracteriza por un envejecimiento precoz y muy acelerado del sujeto afectado. La senilidad se inicia desde muy pronto y enseguida el niño empieza a mostrar los primeros síntomas: piel… …   Diccionario médico

  • Progeria — progeria. f. Med. Síndrome genético que consiste en un envejecimiento prematuro. * * * Progeria (del griego geras, vejez) es una extremadamente rara enfermedad genética de la infancia caracterizada por un dramático envejecimiento prematuro. Se… …   Enciclopedia Universal

  • progeria — 1904, Modern Latin, from Gk. progeros prematurely old; see PRO (Cf. pro ) + GERIATRIC (Cf. geriatric) …   Etymology dictionary

  • progéria — s. f. [Medicina] Doença genética que se caracteriza por envelhecimento precoce e, nas crianças, nanismo.   ‣ Etimologia: grego progeros, on, prematuramente velho + ia …   Dicionário da Língua Portuguesa

  • progeria — f. Med. Síndrome genético que consiste en un envejecimiento prematuro …   Diccionario de la lengua española

  • progeria — [prō jir′ē ə] n. [ModL < Gr progērōs, prematurely old < pro, before + gēras, old age + IA] a rare genetic disorder, esp. of early childhood, characterized by premature aging …   English World dictionary

  • Progeria — Infobox Disease Name = Progeria Caption = DiseasesDB = 10704 ICD10 = ICD10|E|34|8|e|20 ICD9 = ICD9|259.8 ICDO = OMIM = 176670 MedlinePlus = eMedicineSubj = derm eMedicineTopic = 731 MeshID = D011371 Progeria is a condition of early aging [Stedman …   Wikipedia

  • Progéria — Enfant atteint de progéria (l image en haut à droite représente un noyau cellulaire normal, celui d en bas, un noyau déformé au cours d une progéria). La progéria, ou Syndrome de Hutchinson Gilford, est une maladie génétique extrêmement rare …   Wikipédia en Français

  • progeria — /proh jear ee euh/, n. Pathol. a rare congenital abnormality characterized by premature and rapid aging, the affected individual appearing in childhood as an aged person and having a shortened life span. [1900 05; < NL < Gk progér(os) prematurely …   Universalium


Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”

We are using cookies for the best presentation of our site. Continuing to use this site, you agree with this.