Werner kuti chirwere: tsananguro uye zviratidzo

Vasachembera chokwadi, hakudzivisiki vose, asi zvishoma nezvishoma uye anononoka, saka munhu ane nguva kuchinjira kuchinja kuitika muviri wake. Pari, kunzwisisa chakavanzika kuna sayenzi yemazuva ano rava chirwere kuti yekudyidzana Inowedzera pezvakaitwa zvose kukwegura mumuviri - Werner urwere Morrison.

The chirwere apo neapo, saka masayendisiti vasingakwanisi kuwana nzira inobudirira mishonga. Zviri ane anomaly evanhu vemazuva ano mhando. Kokutanga chokwadi urwere kubva chirwere ichi hwakanyorwa muna 1904 ne musayendisiti wokuGermany. Kusvikira zvino, mukuru mupfungwa vanhu vari kuedza kuongorora chinoshamisa chichiitwa, izvo chinoita kukurumidza kuchembera womunhu muviri, uye vari kutsvaka nzira zvinobudirira kurwisa chirwere.

chirwere ichi akaita kupi?

Kazhinji, chirwere nhaka. Varwere kubva vabereki imwe zvemamiriro Gene, iyo iri rechisere chromosome. Nokuda nemagene mutation kukundikana kunoitika, ipapo mwana nhaka magene izvi. Kuchinja zvinoratidzwa basa muviri wose, kusanganisira pasi kunze.

Muna nhasi, takazoziva chete chii vaWerner chirwere - ane autosomal recessive chirwere. Asi kuramba kana vachisimbisa chirwere ichi magene kuongororwa hazvibviri.

Zviratidzo zviri chirwere

Anoratidza isingawanzowanikwi tsika, zviratidzo chirwere ichi pakutanga anogona kuonekwa mushure nemakore gumi. Yokutanga worrisome simba kukura retardation. Asi zvikuru zviratidzo zvose zvakajeka kunogona kuonekwa chete mumakore 20.

Nyanzvi kudoma zvinoverengeka moyo zviratidzo:

• Mumashoko kumbonyanya pfupi nguva munhu akatendeuka bvudzi uye nokukurumidza anorasikirwa ose bvudzi romusoro wake.
• The ganda anotanga zera nokukurumidza, uye rinoratidza hyperpigmentation, wrinkles, zvinova akacheneruka uye yakaoma.

Pamwe kusingarapiki azvaiitwa muvanhu pane rakakura yakawanda nezvimwe pathologies:

• Atherosclerosis.
• Tsanga.
• Kuvhiringidza pamwoyo.
• Osteoporosis.
• Benign uye nyanyisa neoplasms.

Werner kuti chirwere kunokanganisa kuti kwemakemikari anobudiswa ezvinhu, saka munhu anogona kushaya yechipiri pabonde maitiro: kumwedzi hakuitiki, pane akanyanya kukwirira inzwi, zvirwere zviri thyroid shwanu, kuva chirwere cheshuga (insulin irambe). Zvose izvi inozivikanwa vanhu vane ichi urwere inotyisa.

chitarisiko

Varwere nechirwere ichi vanogona kudzidza kubva kure, nekuti vanhu ava vane akasiyana chaizvo chiso zviri kubva dzose. Kazhinji, ivo wana acuteness, mhino yake akafanana muromo kweshiri, uye chirebvu iri kwazvo nyasha, muromo duku, denderedzwa chiso anova kirisendi serin'i chimiro. Also atrophied chose adipose rinenge uye tsandanyama. Naizvozvo, maoko nemakumbo zvisinganzwisisiki dzakaonda - izvi zvose kunokanganisa chakaipa mufambo. Human sangano zvakaoma, anoneta nyore uye haagoni kumira rinonetesa muviri.

Pane mumwe, chikuru aizoonekawo kunoratidzwa Werner syndrome. Photos of vanhu vanotambura nezvirwere ichi, achirova: kuti nemakore 40 vanotarira kaviri sezvo kare. Nhamba dzinoratidza kuti varwere vakanga vakaidzwa chirwere ichi, regai kurarama kusvika makore 50. Kana muviri rinokanganisa kenza, kana rufu kunoitika nokuda mwoyo kurwisa kana sitiroko.

Ndiani pangozi?

Kazhinji, hosha rikaitika hono hafu yevagari. Werner Syndrome pachena (zviratidzo) pashure pakuyaruka. The chirwere hwapedyo panguva samolecular vanochiva mutated Gene, riri basa DNA yeEncoding. Masayendisiti haana afunga chii ukama chirwere ichi nevamwe zvirwere yakafanana anokonzera nokukurumidza zvokukwegura. Naivowo chokwadi kuti iripo zvachose.

Kunyange zvazvo chirwere ichi - husiri nyore kuwana, kune vanhu vakadaro, uye nzanga dzemazuva ano vanofanira kuvabata zvakawanda akavimbika, nokuti hapana munhu ane kusiya ichi. Vanhu vari watarisana chirwere ichi, tinofanira chaizvo. Kuwedzera inotyisa chitarisiko, pane mamwe chokuita nezvirwere kuti havabvumirwi kuvapo zvakanaka. The kwokutanga chikuva achiperekedzwa zvokunze kuchinja, uye unozotevera vanotsamwisa chirwere miviri zvose.

Werner chirwere ari kusimukira nokukurumidza kuitira kuti murwere ari nemakore 40 rurefu mukana kunogona kukanganisa kenza, nekuti malignancies - Kazhinji mune chirwere ichi.

Diagnostics

Anenge zvirwere zvose kazhinji rwakavapo vakura, uye kunyange akwegura, asi chete kana ichi asiri Werner urwere, kukurumidza kuchembera syndrome. Kazhinji, kana zvakazara wezvokurapa kuongorora muna varwere osteoporosis akaona tsoka uye makumbo ezasi, vakadhakwa sechivako nemarunda nemarunda. Kazhinji, murwere anonyunyuta varombo chiratidzo, nekuti kana hosha iri rinenge nguva dzose cataract. Vakawanda vakaona hadzina kufambiswa zvakanaka mubasa uropi, izvo pamwe akurumidze kuderera chepfungwa mano. Kazhinji varwere vanobva kunyunyuta kurwadziwa mumwoyo.

Kuti chirwere, "Werner Syndrome", gadzai chiremba kuongorora zvizere. Kana chiremba haarambi zvinogona mutsika kuona mano fibroblasts zvichiberekana.

mabatiro

Werner kwakaita chirwere - dambudziko XXI remakore. Masayendisiti ari kuedza kudzidza chirwere ichi, kutsvaga chingapedza dambudziko iri uye vari kushanda kuti aponese vanhu ano kubvira anomalies mhando iyi. Tinogona kutaura chinhu chimwe chete - uyu ndiye mumwe zvisingawanzoitiki zviitiko vaberekwe vakaremara.

Zvinosuruvarisa, pasinei nokuti mushonga mumakumi emakore achangopfuura akaita chaizvo mberi kukura nhasi, masayendisiti haagoni kurwisa chirwere ichi. Zvinoita kurapa here? It yasara iri chakavanzika. Kuti tirambe mamiriro murwere vakatakura kurapa chaiyo inobatsira kuti kumisa kufambira kukwegura uye chinonditadzisa nhego chirwere. Kwava kuitwa kuitira kuderedza kudzivirira zviratidzo kuti varipo mune chirwere ichi. Asi kusvikira pakuguma kumisa iyi, vanachiremba haagoni asi.

Ko mapurasitiki kubatsira?

Chokwadi, unogona kutsvaka rubatsiro yakanaka epurasitiki vanovhiya uye kuvandudza chitarisiko. Asi munhu mazera ari kusimba kutsanya, saka rubatsiro rwakadai ichava pfupi-pfupi. Mumwe bedzi kuti munguva yemberi iri pedyo nevatsvakurudzi kuva vachabudirira uye pane mushonga chinopa zvinozoita. Munguva ichangopfuura, mumwe mujenya tariro hunopiwa zvokurapa, iro vobatanidzwa mukurapa kupfuura apererwa zvirwere vachishandisa hunde masero. Tinogona chete kutenda kuti nokukurumidza tichibatsirwa nzira iyi zvichiita kurapa Verner Johnson syndrome.