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Progeria

Progeria ( /proʊˈdʒɪəriə/),[1] also called Hutchinson–Gilford progeria syndrome[2][3] and HGPS progeria syndrome[3] is a very rare genetic disorder. Children born with progeria show symptoms which are like aging.[4] This can include skin wrinkles and grey hair or baldness.[1] Progeria is one of several progeroid syndromes.[5] The word progeria comes from the Greek words "pro" (πρό), meaning "before" or "premature", and "gēras" (γῆρας), meaning "old age".[6]

Progeria
Classification and external resources
A young girl with progeria (left). A healthy cell nucleus (right, top) and a progeric cell nucleus (right, bottom).
ICD-10E34.8 (ILDS E34.840)
ICD-9259.8
OMIM176670
DiseasesDB10704
MedlinePlus001657
eMedicinederm/731
MeSHD011371

It is very rare, only 1 child in every 8 million live births.[7] People with progeria usually only live to their mid teens to early twenties.[8][9] It is a genetic condition that occurs as a new mutation. It is rarely inherited, as people with the condition do not usually live long enough to have children.

Scientists are studying progeria because it might reveal clues about the normal process of aging.[10][11][12] Progeria was first described in 1886 by Jonathan Hutchinson.[13] It was also described independently in 1897 by Hastings Gilford.[14] The condition was later named Hutchinson–Gilford progeria syndrome.

Progeria Media

  • Ultrastructural analysis of the nuclear envelope in fibroblasts from a subject with HGPS. Low magnification transmission electron microscopic image of a passage 10 PT001 nucleus showed several herniations (a).

References

  1. ↑ 1.0 1.1 Definition of progeria, Progeria - definition of progeria by The Free Dictionary, accessdate: December 30, 2015
  2. ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology (10th ed.). Saunders. p. 574. ISBN 0-7216-2921-0.
  3. ↑ 3.0 3.1 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
  4. ↑ Sinha, Jitendra Kumar; Ghosh, Shampa; Raghunath, Manchala (May 2014). "Progeria: a rare genetic premature ageing disorder". Indian J Med Res. 139 (5): 667–74. PMC 4140030. PMID 25027075.
  5. ↑ Ramírez, C. L.; Cadiñanos, J.; Varela, I.; Freije, J. M. P.; López-Otín, C. (1 January 2007). "Human progeroid syndromes, aging and cancer: new genetic and epigenetic insights into old questions". Cellular and Molecular Life Sciences: CMLS. 64 (2): 155–170. doi:10.1007/s00018-006-6349-3. PMID 17131053. S2CID 22892146 – via PubMed.
  6. ↑ "ETYMOLOGY OF THE WORD PROGERIA". Archived from the original on 2016-03-04. Retrieved 2015-12-29.
  7. ↑ Progeria Archived 2009-12-04 at the Wayback Machine, Incidence of Progeria and HGPS.
  8. ↑ Ewell Steve Roach & Van S. Miller (2004). Neurocutaneous Disorders. Cambridge University Press. p. 150. ISBN 978-0-521-78153-4.
  9. ↑ Kwang-Jen Hsiao (1998). Advances in Clinical Chemistry:33. Academic Press. p. 10. ISBN 0-12-010333-8.
  10. ↑ McClintock, Dayle; Ratner, Desiree; Lokuge, Meepa; Owens, David M.; Gordon, Leslie B.; Collins, Francis S.; Djabali, Karima (2007-12-05). "The Mutant Form of Lamin A that Causes Hutchinson-Gilford Progeria Is a Biomarker of Cellular Aging in Human Skin". PLOS ONE. 2 (12): e1269. doi:10.1371/journal.pone.0001269. ISSN 1932-6203. PMC 2092390. PMID 18060063.
  11. ↑ Korf B (2008). "Hutchinson-Gilford progeria syndrome, aging, and the nuclear lamina". N. Engl. J. Med. 358 (6): 552–5. doi:10.1056/NEJMp0800071. PMID 18256390.
  12. ↑ Merideth, Melissa A.; Gordon, Leslie B.; Clauss, Sarah; Sachdev, Vandana; Smith, Ann C. M.; Perry, Monique B.; Brewer, Carmen C.; Zalewski, Christopher; Kim, H. Jeffrey; Solomon, Beth; Brooks, Brian P. (2008-02-07). "Phenotype and course of Hutchinson-Gilford progeria syndrome". The New England Journal of Medicine. 358 (6): 592–604. doi:10.1056/NEJMoa0706898. ISSN 1533-4406. PMC 2940940. PMID 18256394.
  13. ↑ Hutchinson J (1886). "Case of congenital absence of hair, with atrophic condition of the skin and its appendages, in a boy whose mother had been almost wholly bald from alopecia areata from the age of six". Lancet. I (3272): 923. doi:10.1016/S0140-6736(02)06582-0.
  14. ↑ Gilford H; Shepherd, RC (1904). "Ateleiosis and progeria: continuous youth and premature old age". Brit. Med. J. 2 (5157): 914–8. doi:10.1136/bmj.2.5157.914. PMC 1990667. PMID 14409225.
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