Ana Progeria: Kodi muli ana mwayi?

Ana Progeria - matenda a khalidwe chibadwa, zikubweretsa inasinthiratu mu ziwalo za dongosolo chifukwa cha ukalamba msanga wa chamoyo wonse. Kwa nthawi yoyamba matendawa wakhala kuzindikiridwa ndi anafotokoza mu 1889 ndi Yohane. Hutchinson ndi payekhapayekha mu 1904 ndi J. Guilford. Ngakhale kuti ana progeria - matenda osowa kwambiri, popeza anapeza ake anafotokoza milandu zoposa 150. Ndipo aliyense wa iwo ankaphunzira nambala yaikulu asayansi.

mmodzi wa 7 miliyoni akhanda madokotala Only oterewa vuto la Hutchinson-Gilford. Ana Progeria kusiyana mofulumira - chabe chaka thupi wodwala pa zaka 5-9. The ana nthawi zina adzapulumuke wazaka 23, akufa ndi matenda a kawirikawiri kwa okalamba. Zizindikiro zoyambirira za "ukalamba, ana" kuwonekera ana ku m'badwo woyambirira (zaka 2-3).

Pamene zimenezi kulibe, kuti, mankhwala amaikidwa yekha zochizira symptomatic matenda mbali. Koma asayansi padziko lonse akuphunzira vutolo ndipo akufunafuna njira zothandiza kuthana nazo.

Yodziwika ana progeria zotsatirazi mawonetseredwe:

• ang'onoang'ono thunthu;
• kuwala (13-22 makilogalamu);
• khungu abwino, amene ziwiya zooneka;
• pang'onopang'ono mfundo za manja ndi mapazi;
• chachikulu mutu, koma pa nthawi yomweyo - munthu wamng'ono;
• okwera mawu.

Zimayambitsa matenda

asayansi amavomereza kuti ana progeria si matenda, ndi akatengela apabanja. dziko zodziwika kokha kwa banja lina, pamene ana onse 3 adzalandira matendawa.

Si kale ankaganiza kuti chifukwa chokha kwa "zaka ana" - ndi kusintha kwa khalidwe lililonse limene kumatanthauza mapangidwe a A. mapuloteni lamin The mtima selo anamanga pamaziko a mapuloteni ndi. Mu ndondomeko ya kusintha zolephera A prelamina okhwima mapuloteni ndi. Choncho, chifukwa lamin A kwambiri isiyana ndi mapuloteni yachibadwa. Zimenezi zimabweretsa zosiyanasiyana kusintha pathological mu thupi la mwanayo, amene amakhala ndi matenda Hutchinson-Gilford. Ana Progeria kuyamba patsogolo kuchititsa ukalamba msanga.

Koma asayansi ena anapeza jini wina mu DNA, ndi kusintha kumene kumakhala kumayambitsa matenda. njira sizingasinthe mu BAF-1 jini -prichina zopindika chitukuko maselo nembanemba. Popeza BAF-1 komanso interacts ndi mapuloteni ena, kusintha mu dongosolo lake amachititsa chisokonezo mapuloteni ena.

Kodi pali mwayi?

Pakali pano, asayansi maso yovuta kumaliza phunziro mwatsatanetsatane za ndondomeko ya kusintha jini. Ndipo ngati bwino, ndiye ana odwala adzapatsidwa mwayi weniweni kupambana ana progeria, ndi madokotala - kuteteza pakuwonekera kwake.

Tikumbukenso odwala kuti ambiri ndi chiyembekezo kukomoka chipulumutso. US asayansi anayamba mayesero matenda a odana ndi matenda. N'zotheka Komabe, kuti si mwana zimatha kukhala nthawi imeneyi. Koma Mulimonsemo, ngati mayesero ali bwino ndi ana progeria ali wogonjetsedwa, zidzakhala kupambana kwa onse amene akuchita zonse kuti apulumutse ana awo matenda zoipazi.