Unoziva nezvechirwere cheAlexander here? Ngatitaurei nezvechirwere ichi chisingawanzoitiki!

Wakambonzwa nezvechirwere cheAlexander here? Pamwe hausati wambonzwa nezvacho. Chikonzero ndechekuti chirwere chetsinga chisingawanzoitiki pasi rose. Asi, zvakakosha kuva neruzivo nezvezvirwere zvakaita seizvi, handiti? Kunyanya sezvo tichigara tichinetseka nezvehutano nekukura kwevana vedu, zvakakosha kuziva zvinhu zvakaita seizvi.

Chii chinonzi chirwere cheAlexander? Ngatichinzwisise zviri nyore!

Zvakanaka, ngationei kuti chirwere cheAlexander chii. Zvichitaurwa zviri nyore, chirwere chinokanganisa sisitimu yedu yetsinga, sisitimu inotakura mameseji mumuviri wese . Zvikuru sei, chinokanganisa "chinhu chichena" chiri muuropi hwedu. Chinhu chichena ichi chikamu cheuropi chakaumbwa netsinga dzakawanda.

Chirwere ichi ndecheboka rezvirwere zvinonzi leukodystrophy . Kunyange zvazvo izwi iri ringanzwika serakaoma, rinoreva zvirwere zvinokanganisa uropi.

Muchirwere cheAlexander, kukuvara kukuru kunowanikwa pachikamu chinonzi myelin . Myelin inodzivirira tsinga dzedu. Funga nezvayo sepurasitiki yakakomberedza waya yemagetsi. Iyi myelin sheath ndiyo inobvumira mameseji etsinga kufamba zvakanaka uye nekukurumidza. Saka, kana iyi myelin ikakuvara, maitiro ekutumira mameseji etsinga anokanganiswa.

Nekuda kweizvozvo, munhu ane Alexander syndrome anogona kusangana nematambudziko akasiyana-siyana, akadai sekunonoka kukura uye pfari . Zvinosuwisa kuti chirwere ichi chirwere chinoenderera mberi, dzimwe nguva chinoisa hupenyu panjodzi. Parizvino hapana mushonga wacho.

Chirwere ichi chakajairika zvakadii?

Chirwere cheAlexander chirwere chisingawanzoitiki . Zvinofungidzirwa kuti chirwere ichi chinoitika muvana vanozvarwa vanosvika miriyoni imwe chete . Chirwere ichi chakatanga kuzivikanwa muna 1949 naDr. W. Stewart Alexander , chiremba wekuAustralia. Ndosaka chichinzi chirwere cheAlexander.

Pane mhando dzechirwere cheAlexander here?

Ehe, vanachiremba vanoisa chirwere ichi muzvikamu zvichienderana nezera rinotanga kuoneka zviratidzo. Kune mhando dzakawanda huru:

• Rudzi rwemwana achangoberekwa: Izvi hazviwanzoitiki. Zviratidzo zvinoonekwa mukati memwedzi wekutanga wehupenyu.
• Rudzi rwevacheche: Rudzi urwu runoumba 80% yezvirwere zveAlexander syndrome. Zviratidzo zvinowanzotanga vasati vasvitsa makore maviri .
• Rudzi rwevana: Zviratidzo zvinogona kuonekwa pakati pemakore maviri negumi nematatu. Zvisinei, zvinonyanya kuonekwa pakati pemakore mana negumi. Inenge 14% yezvirwere zveseNdezverudzi urwu.
• Rudzi rwemunhu mukuru: Izvi hazviwanzoitika. Zviratidzo zvinowanzova zvishoma. Chirwere ichi chinogona kuitika chero pazera ripi zvaro mushure mekuyaruka . Inenge 6% yezvirwere zvinowira murudzi urwu.

Chii chinokonzera chirwere cheAlexander?

Mu95% yezviitiko zvechirwere cheAlexander, shanduko inoitika mujini . Jini iri rinobatsira kugadzira puroteni inonzi Glial Fibrillary Acid Protein (GFAP) . Zvinosuwisa kuti chikonzero che5% yasara yezviitiko hachisati chazivikanwa.

Kazhinji, mapuroteni aya eGFAP anobatana kuti aumbe cheni refu (filaments). Cheni idzi dzinopa simba nerutsigiro kumasero etsinga dzedu.

Zvisinei, muvanhu vane chirwere cheAlexander, puroteni iyi yeGFAP haigadzirwe nemazvo. Pane kudaro, mapuroteni asina kujairika anonzi Rosenthal fibers anoungana munzvimbo dziri mukati memasero paasingafaniri kuva. Aya maRosenthal fibers ndiwo anokuvadza myelin yambotaurwa.

Ndiani ari panjodzi huru yekubatwa nechirwere ichi?

Kutaura zvazviri, chero munhu anogona kurwara nechirwere ichi. Kazhinji, kuchinja kwemajini kunoitika zvisina tsarukano, pasina chikonzero chakajeka. Kashoma kuti mwana agare nhaka kuchinja kwemajini uku kubva kuvabereki vake. Izvi zvinoreva kuti vanhu vazhinji havana nhoroondo yemhuri yechirwere ichi.

Ndezvipi zviratidzo zvechirwere cheAlexander?

Zviratidzo zvechirwere ichi zvinogona kusiyana kubva pamunhu kusvika pamunhu. Zvakare, zviratidzo zvinosiyana zvichienderana nezera (rudzi) iro chirwere chinotanga.

Zviratidzo zvevana vacheche:

Zviratidzo izvi zvinoonekwa mukati memwedzi wekutanga wehupenyu.

• Hydrocephalus: Uku kuunganidzwa kwemvura muuropi hwemwana. Izvi zvinogona kukonzera kuti musoro ukure.
• Megalencephaly: Kukura kusingajairika kweuropi nemusoro.
• Kubatwa nepfari/Epilepsy: Zvirwere zvinowanzoita sepfari.
• Kuomarara: Kuomarara kwetsandanyama, kudzikira kwekuchinjika.
• Kunonoka kukura: Kukundikana kwemwana kukura zvine mwero unoenderana nezera rake. Semuenzaniso, kusimba kwemutsipa, kutenderera, uye kugara zvakanaka zvinogona kunonoka.
• Kukundikana kukura zvakanaka kana kuwedzera uremu zvakanaka.

Zviratidzo zvevana vacheche:

Zviratidzo izvi zvinowanzotanga mukati memwedzi mitanhatu yekutanga, asi dzimwe nguva zvinogona kutanga kusvika pamwedzi makumi maviri nemana, kana makore maviri. Zviratidzo zvacho zvakafanana zvikuru nezvinoonekwa panguva yekuzvarwa.

Zviratidzo zvekutanga kwevana:

Zviratidzo izvi zvinowanzoonekwa pakati pemakore mana negumi.

• Kuoma kumedza nekutaura.
• Kusagona kugadzikana kwepfungwa (ataxia):Izvi zvinoreva matambudziko ekuenzanisa, kubatana, uye kufamba, zvakaita sekutadza kufamba kana kunetseka kubata zvinhu.
• Matambudziko emhasuru: zvinhu zvakaita sekuoma kwemhasuru (spasticity), kurwadziwa kwemhasuru, kugwinha kwemhasuru.
• Kurutsa kakawanda.

Zviratidzo zvekutanga kwechirwere chepfungwa chevakuru:

Zviratidzo izvi zvinogona kuonekwa chero nguva munhu akura. Kazhinji zvakafanana nezvakaonekwa achiri mudiki, asi kudedera kunogonawo kuitika. Dzimwe nguva zviratidzo izvi zvinogona kutevedzera zvimwe zvirwere, zvakaita sechirwere cheParkinson kana multiple sclerosis , saka zvakakosha kuti munhu awane chirwere chakarurama.

Chirwere cheAlexander chinoonekwa sei?

Chiremba wako achatanga anyatsoongorora zviratidzo zvako kana zvemwana wako. Pamusoro pezvo, vanogonawo kuita bvunzo dzakadai se:

• Kuongororwa kweMRI (MRI - Magnetic Resonance Imaging): Izvi zvinogona kutarisa chero shanduko dziri muuropi. Kunyanya, MRI inogona kuratidza shanduko muzvinhu zvichena, zvakaita sezviratidzo zveRosenthal fibers.
• Kuongororwa kwemajini: Uku kuongororwa kweropa kunogona kusimbisa kana paine shanduko mujini reGFAP inokonzera chirwere cheAlexander.

Chirwere ichi chinorapwa sei? Chinorapwa sei?

Zvinosuwisa kuti chirwere chaAlexander hachina mushonga . Mishonga yazvino inovavarira kudzora zviratidzo uye kuderedza kufambira mberi kwechirwere .

Zvisinei, masayendisiti ari kuramba achiita miedzo yekiriniki kuti vawane mishonga mitsva yechirwere ichi. Unogona kutaura nachiremba wako nezvekuti rudzi urwu rwemuedzo rwakakunakira iwe kana mwana wako here.

Zvichienderana nezviratidzo, nzira dzinotevera dzekurapa dzinogona kushandiswa:

• Mishonga yekudzivirira pfari.
• Kurapa muviri, kurapwa nebasa, uye kurapwa nekutaura. Izvi zvinobatsira kuvandudza kufamba kwemwana, kugona kwake kuita mabasa ezuva nezuva, uye kutaura.
• Panyaya yehydrocephalus, chirwere chinosanganisira mvura yakawanda muuropi, kuvhiyiwa kweshunt kunogona kuitwa. Izvi zvinobvisa mvura yakawanda muuropi.

Ndezvipi zvinetso zvechirwere cheAlexander?

Zviratidzo zveAlexander syndrome zvinogona kuwedzera zvishoma nezvishoma nekufamba kwenguva. Saka, murwere angada zvinhu zvakaita se:

• Zvishandiso zvinobatsira pakufamba, zvakaita semawiricheya kana mawalkers.
• Kuti uwane kudya kwakakwana, kudya kwemwana nepachubhu ( kudya kwemukati ) kunogona kudiwa.

Remangwana revanhu vane chirwere ichi nderei?

Kufanotaura ramangwana revanhu vane chirwere cheAlexander kwakaoma zvishoma, sezvo kuchisiyana kubva pamunhu kuenda kumunhu . Sezvo chirwere ichi chichiwedzera, zviratidzo zvinogona kuwedzera nekufamba kwenguva, kunyanya kuvana. Hunhu uye nguva yezviratidzo zvinosiyana zvichienderana nerudzi rwechirwere:

• Vacheche vane chimiro chekuzvarwa kwemwana vanowanzova vakaremara zvakanyanya, uye vazhinji vanofa vasati vasvitsa makore maviri .
• Vana vane rudzi rwevacheche vanogona kurarama kwemakore angangoita mashanu kusvika gumi .
• Vana vane rudzi rwevana vanogona kurarama kusvika pamakore ekuma30 kana 40 .
• Vamwe vanhu vane chirwere ichi vanotanga kukura vane zviratidzo zvishoma, kana kuti vanogona kunge vasina zviratidzo. Kazhinji, chirwere ichi hachikanganisi hupenyu hwavo.

Zvakajairika kuti unzwe kusuruvara paunonzwa zvinhu izvi. Asi kuziva ruzivo urwu kuchakubatsira kunzwisisa chirwere chacho.

Chirwere cheAlexander chinogona kudzivirirwa here?

Kazhinji, kunyange nyanzvi hadzigoni kutaura kuti sei shanduko yemajini inokonzera chirwere cheAlexander ichiitika. Saka, kazhinji, hapana nzira yekudzivirira chirwere ichi.

Zvisinei, kana mumwe munhu mumhuri yako aine chirwere cheAlexander kana rumwe rudzi rweleukodystrophy, zvakanaka kutaura nenyanzvi yemajini . Izvi zvichakubatsira kunzwisisa njodzi yekuti vana vako vemangwana vagare nhaka chirwere ichi. Ungangodawo kufunga nezvenzira inonzi Preimplantation Genetic Diagnosis (PGD) . Izvi zvinosanganisira kusarudza mazai ane hutano asina GFAP gene mutation inokonzera chirwere cheAlexander uye kuaisa muchibereko kuburikidza ne in vitro fertilization (IVF) .

Ndinofanira kuona chiremba nguvai?

Kana iwe kana mwana wako muine chirwere cheAlexander, shanyirai chiremba nekukasika kana mukaona chimwe chezviratidzo izvi:

• Kuoma kwekufamba kana kuenzana.
• Kuomerwa nekufema, kumedza, kudya, kana kutaura.
• Kusvotwa nekurutsa.
• Kubatwa nepfari.

Mibvunzo ipi yandinofanira kubvunza chiremba wangu?

Unogona kubvunza chiremba mibvunzo yakaita seiyi:

• Nderudzii rwechirwere cheAlexander chandinacho (kana mwana wangu)?
• Ndeipi nzira yakanakisisa yekurapa?
• Zvakanaka here kuti vamwe vemumhuri vawane kuongororwa kwemajini echirwere cheAlexander syndrome?
• Ndezvipi zviratidzo zvematambudziko andinofanira kutarisa?

Pakupedzisira, meseji yekudzokera nayo kumba

Chirwere cheAlexander chirwere chinoenderera mberi, chinoenderera mberi kwehupenyu hwese.Dambudziko rehutano. Izvi hazviwanzoitiki uye dzimwe nguva zvinogona kuitika mumhuri. Kuongororwa kwemajini kunogona kuona kusiyana kwemajini kunokonzera chirwere ichi.

Kunyange hazvo pasina mushonga, kune mishonga inogona kubatsira kuderedza zviratidzo uye kuvandudza hupenyu hwevanhu. Masayendisiti ari kuramba achitsvaga nzira dzekurapa chirwere ichi chisingawanzoitiki.

Ndinovimba kuti kuziva ruzivo urwu kuchakubatsira kunzwisisa chirwere ichi uye kutsvaga mazano echiremba nekukurumidza kana zvichidikanwa. Gara uchiyeuka kuti hausi wega, uye unogona kuwana rubatsiro rwaunoda.

Chirwere chaAlexander , Chirwere chetsinga, Chirwere cheMajini, Myelin, Leukodystrophy, Chirwere cheVana