Chii chinonzi Prion Disease? Ngationei kuti chii chaizvo!

Wakambonzwa nezve 'Prion Disease' iyi here? Pamwe zita iri inyaya itsva kwauri. Chikonzero ndechekuti iri iboka rezvirwere zvisingawanzoitiki pasi rose. Zvisinei, zvakakosha kuziva nezvechirwere ichi, sezvo chinokanganisa zvakananga uropi hwedu uye zviratidzo zvinokurumidza kukura. Chinogona kuitika kuvanhu pamwe nemhuka.

Chii chinonzi Prion Disease?

Zvichitaurwa zviri nyore, zvirwere zve prion iboka rezvirwere zvinokonzerwa nepuroteni yakajairika muuropi hwedu inoshanduka kuita mapuroteni anokuvadza anonzi "prions." Fungidzira sero rakanaka mumuviri wedu richingoerekana rashanduka kuita sero rakaipa, rakachinja.

Kana mapuroteni aya asina kujairika eprion akaungana muuropi, anotanga kukuvadza masero europi. Kutaura chokwadi, ichi chirwere chinokanganisa mashandiro etsinga. Nekufamba kwenguva, kukuvara uku kunogona kutungamira kudzikira zvishoma nezvishoma kwebasa reuropi, zvichikonzera mamiriro akaita sedementia . Izvi zvinoreva kuti unorasikirwa nendangariro dzako, unonetseka kufunga, uye haugone kuita basa rako. Chinhu chine njodzi zvikuru nezvechirwere ichi ndechekuti zviratidzo izvi zvinotanga kamwe kamwe uye zvinokurumidza kukura. Chirwere ichi chinobata vanhu vanosvika miriyoni imwe chete pasi rose. Izvi zvinoreva kuti hachiwanzoitiki.

Zvinosuwisa kuti zvirwere zvePrion zvirwere zvinouraya . Izvi zvinoreva kuti kana chirwere changotanga, zvinonetsa kurapa. Vanachiremba vanoedza kudzora zviratidzo uye kuita kuti murwere anzwe akasununguka sezvinobvira. Vanobatsirawo murwere kuti atsungirire kukanganiswa kwechirwere ichi muhupenyu hwake, mhuri yake, uye avo vanomutarisira.

Zvirwere izvi zve prion zvinokura sei?

Kune nzira nhatu huru dzinoita kuti munhu awane chirwere cheprion.

1. Nekugara nhaka yekuchinja kwemajini (izvi zvinonzi chirwere chemhuri chinonzi prion).

2. Nekutapukirwa (izvi zvinonzi chirwere cheprion chinowanikwa).

3. Zvisinei, kazhinji, chirwere ichi chinoitika pasina chikonzero chemajini kana hutachiona hwekunze . Vaongorori nevanachiremba vanodana chirwere ichi kuti 'chirwere cheprion chinongoerekana chaitika'.

Zvino ngatitarisei imwe neimwe yenzira idzi zvakadzama.

Zvirwere zvePrion Zvishoma Nezvishoma

Ichi ndicho chirwere cheprion chinowanzoonekwa. Chinoitika pano ndechekuti, pasina chikonzero chakajeka, mapuroteni akajairwa muuropi anongoerekana ava 'prions' akaipa ataurwa pamusoro apa. Chikonzero chaicho chinoita kuti izvi zviitike hachisati chazivikanwa.

Zvirwere zvikuru zviri muchikamu ichi ndezvi:

• Chirwere cheCreutzfeldt-Jakob (CJD) : Ichi ndicho chinokonzera 85% yezvirwere zveprion zvisingawanzoitiki. Ndiro rudzi rwakajairika.
• Kushaya hope kunouraya nguva nenguva: Izvi hazviwanzoitiki. Zvishoma kupfuura kushaya hope kunouraya kunoberekwa nhaka.
• Variable protease-sensitive prionopathy : Ichi chirwere cheprion chisingawanzoitiki.

Zvirwere zvePrion zvemhuri

Rudzi urwu rwechirwere cheprion runokonzerwa nekuchinja kwemajini kunonzi `PRNP` mumajini edu. Chirwere ichi chinogona kuitika kunyangwe kuchinja uku kuchitorwa kubva kuna amai kana baba (izvi zvinonzi `autosomal dominant inheritance`). Kuchinja kwakasiyana-siyana kunopfuura makumi mashanu mujini re`PRNP` kwawanikwa, uye kuchinja kwega kwega kunogona kukonzera mhando dzakasiyana dzechirwere cheprion chinogara nhaka.

Zvimwe zvirwere zviri muchikamu ichi:

• Chirwere cheCreutzfeldt-Jakob chemhuri (CJD)
• Chirwere cheGerstmann-Sträussler-Scheinker (GSS) : Ichi chirwere chisingawanzoitiki. Chinoitika mumunhu mumwe chete kusvika kugumi muvanhu vane mamiriyoni zana pasi rose.
• Kushaya hope mumhuri kunouraya : Izvi hazviwanzoitiki kupfuura chirwere cheGSS. Mhuri 50 kusvika 70 chete pasi rose dzine genetic mutation inokonzera chirwere ichi.
• Zvimwe zvirwere zve prion zvinokonzerwa nekuchinja kwemajini ePRNP zvakashumwawo. Semuenzaniso, nhengo gumi neimwe dzemhuri yekuBritain dzakaratidza zviratidzo zvakaita semanyoka, kunzwa kwetsinga dzeropa, pfari, uye dementia. Dambudziko iri, rinonzi autonomic neuropathy, rinokanganisa zvinhu zvakaita seBP yako, kurova kwemoyo, uye kusazvidzora kwemudumbu nedumbu.

Chirwere chePrion chakawanikwa

Iyi ndiyo nzira isingawanzoitiki yekuwana zvirwere zveprion. Pano, munhu anogona kuwana chirwere ichi nekudya chikafu chakasvibiswa nemaprion (semuenzaniso, nyama yemombe ine 'chirwere chemombe chinopenga') kana nekushandisa michina yekurapa ine utachiona (semuenzaniso, panguva yekuvhiyiwa).

• Kuru ndiyo nyaya yekutanga yechirwere ichi chepfungwa chinotapukira kuonekwa nekuongororwa. Chakawanikwa pakati pevanhu vekuPapua New Guinea. Zvinofungidzirwa kuti chakapararira kuburikidza nemimwe mitambo yavo (yakadai sekudya nyama yevanhu).

Nerombo rakanaka, nzira dzemazuva ano dzekuchenesa michina yekurapa uye kungwarira kuchengetedzeka kwechikafu zvakaderedza zvakanyanya kupararira kwezvirwere zveprion nenzira iyi.

Ndezvipi zvirwere zve prion zvine njodzi zvikuru?

Kutaura zvazviri, zvirwere zvese zve prion zvinouraya . Kureva kuti, kana ukabatwa nechirwere ichi, uchafa zvechokwadi. Kazhinji, murwere anofa mukati memwedzi mishoma kusvika makore matatu mushure mekutanga kwezviratidzo zvechirwere che prion.

Ndezvipi zviratidzo zvezvirwere zve prion?

Zviratidzo zvechirwere cheprion zvinogona kusiyana, zvichienderana nerudzi rwechirwere cheprion uye kuti chikamu chipi cheuropi chinobatwa nacho. Asi kazhinji, vanhu vane chirwere cheprion vanowana matambudziko netsinga dzavo izvo zvinozowedzera zvishoma nezvishoma.

Izvi ndizvo zvimwe zvezviratidzo zvinonyanya kuzivikanwa:

• Kutadza kufamba zvakanaka, kudzedzereka (`Ataxia`)
• Kuoma kutaura, kusanzwisisa mazwi (`Aphasia`)
• Kuvhiringidzika, kuvhiringidzika
• Kushaya hope
• Kurasikirwa nendangariro, kunetseka kufunga uye kuita zvisarudzo
• Kufamba zvishoma nezvishoma kana kuomarara kwetsandanyama (`Hypokinetic movement disorders`)
• Kungoerekana wazununguka, kudedera (`Myoclonus`)
• Kuchinja kwehunhu (semuenzaniso, kushatirwa, kushatirwa)
• Matambudziko epfungwa (semuenzaniso kuzvidya mwoyo, kuora mwoyo, uye dzimwe nguva kunyange kuona zvinhu zvisipo)

Fungidzira, mumwe munhu ari pedyo newe anotanga kuchinja kamwe kamwe. Anokanganwa zvinhu, haagoni kutaura sezvaaimboita, anonetseka nekufamba. Zvinoshungurudza sei kuona zviratidzo izvi?

Ndezvipi zvinetso zvezvirwere zve prion?

Matambudziko ezvirwere zveprion anogona kubatanidzwa, zvichigadzira kuwanda kwematambudziko anogona kukanganisa zvakanyanya kwete murwere chete, asiwo mhuri neshamwari dzinomutarisira.

Matambudziko aya anogona kuitika mukati memwedzi mishoma kana gore mushure mekunge zviratidzo zvatanga:

• Kusakwanisa kuita basa rako wega
• Dementia ( kurasikirwa zvachose kwendangariro)
• Kusagona kutaura ('Mutism')
• Coma (iyi ndiyo danho rekupedzisira)

Kana munhu aine chirwere cheprion, anokurumidza kuvimba nevamwe. Mhuri nevamwe varipo kuti vamutarisire uye vamubatsire. Panguva iyi, kuona mudiwa wake achirasikirwa nendangariro dzake, asingachagoni kutaura, uye hunhu hwake huchichinja zvinoshungurudza zvikuru vanomuchengeta.

Sei zvirwere izvi zve prion zvichiitika?

Iyi inyaya yesainzi, asi ndichataura zviri nyore. Zvirwere zvePrion zvinotanga kana puroteni yeprion yakajairika muuropi hwedu (inonzi `PrPc`) ikashanduka kuita puroteni isina kujairika, yakapetwa zvisina kunaka (inonzi `PrPSc` - ndiyo prion).

Mapuroteni aya asina kujairika (`PrPSc`) anotanga kuungana, kana kubatana nemapuroteni eprion akajairika (`PrPc`), achiachinja kuita mapuroteni asina kujairika. Zvakafanana nekukanda apuro rakaipa mumurwi wemaapuro akanaka, uye mamwe ose anozoipawo. Nekufamba kwenguva, mapuroteni aya asina kujairika anokuvadza kana kuparadza masero etsinga muuropi. Ndipo paunotadza kufamba, kufunga, kana kutaura zvakanaka.

Chirwere cheprion chinoonekwa sei?

Vanachiremba vanogona kushandisa bvunzo dzinotevera kuti vaongorore chirwere cheprion:

• Kuongororwa kweropa uye kubhoorwa kwechiuno : Vanhu vane majini anokonzera zvirwere zveprion zvakagarwa nhaka vanoongororwa zviratidzo zvechirwere kana kukuvara muropa ravo kana cerebrospinal fluid (mvura inotenderedza uropi nemusana).
• Kuongororwa kweMRI yeuropi : Izvi zvinogona kutora mifananidzo yakajeka yeuropi, kuitira kuti vanachiremba vagone kutsvaga zviratidzo zvechirwere cheprion.
• Electroencephalogram (EEG) : Iyi inoyera mashandiro emagetsi europi.
• Kuyedzwa kwekuchinja kwemuviri kunokonzerwa nekubvunda (RT-QuiC) : Muyedzo uyu, vanachiremba vezvirwere vanotsvaga ma prion ari mumvura yemusana. Iyi bvunzo itsva uye yakarurama.

Pane mushonga wezvirwere zveprion here?

Zvinosuwisa kuti vanachiremba havasati vawana mushonga wezvirwere zveprion kana mushonga unogona kudzora kufambira mberi kwechirwere. Zvirwere zveprion zvirwere zvinotyisidzira hupenyu . Vanhu vazhinji vanofa mukati memwedzi kusvika makore mushure mekuonekwa vaine chirwere cheprion.

Kurapa kunonyanya kutarisisa kurapwa kwekurwadziwa kwemusana . Izvi zvinoreva kudzora zviratidzo uye kuita kuti murwere anzwe akasununguka uye asanetseke sezvinobvira. Semuenzaniso, vanachiremba vanogona kunyora mishonga yezvinhu zvakaita se:

• Mishonga yekuderedza pfari kana mishonga inozorodza tsandanyama yemyoclonus.
• Maopioid ekurwadziwa.

Munhu ane chirwere cheprion ane remangwana rerudzii?

Panguva ino, hapana mushonga kana mushonga wezvirwere zveprion. Zvisinei, vaongorori vari kuferefeta izvi nenzira mbiri, izvo zvinogona kutungamira mukuonekwa kwechirwere ichi nekukurumidza uye kurapwa mune ramangwana.

Bvunzo ye `RT-QuiC` yambotaurwa yakabatsira kuona chirwere che prion uropi husati hwakuvara zvisingagadzirisiki. Vaongorori vari kudzidzawo nezve prion kuti vawane nzira dzekumisa kuumbwa kwemapuroteni aya asina kujairika uye kudzivirira mapuroteni asina kujairika kuti asave asina kujairika.

Chii chandinofanira kuita kana ndine chirwere cheprion?

Nekuti chirwere cheprion chinokurumidza kukura, zvinogona kukuomera kuti uite basa rako wega. Kana uine chirwere ichi, zvakanaka kuti unyore "mirairo yekurapa nguva isati yasvika" .

Mirayiridzo yepamberi magwaro epamutemo. Mienzaniso:

• Zvinyorwa zvenhaka zvekurarama : Kana uine chirwere chinouraya senge chirwere cheprion, gwaro iri rinogona kutsanangura zvaunoda kuti zviitike kwauri (semuenzaniso kana uchida kurapwa kwakati kana kuti kwete).
• Simba rekuchengetedza hutano (DPA): Gwaro iri rinogona kudoma kuti ndiani achaita sarudzo dzako dzehutano kana usingachakwanisi kutaura wega.

Nekuronga zvinhu zvakadai pachine nguva, unogona kuderedza kushushikana kwauchasangana nako iwe nemhuri yako gare gare.

Ko kana mumwe munhu mumhuri aine chirwere cheprion chakagarwa nhaka?

Dzimwe nguva, vanhu vanogara nhaka kushanduka kwemajini kunowedzera njodzi yavo yekubatwa nechirwere cheprion. Kuongororwa kwemajini kunogona kuona kana uine chimwe kana kupfuura kushanduka kwemajini kunokonzera chirwere cheprion. Kuongororwa uku kunogonawo kuratidza njodzi yako chaiyo.

Kuongororwa majini echirwere chinouraya chisarudzo chemunhu oga. Vamwe vanhu havadi kuziva kana vari panjodzi. Kana uchida kuongororwa majini, kumbira chiremba kuti akutumire kune mumwe munhu. Semuenzaniso, muUnited States, kune masangano akaita se "National Prion Disease Pathology Surveillance Center" yeizvi. Kunewo vanachiremba vane hunyanzvi muSri Lanka vanogona kupa mazano panyaya iyi.

Unotarisira sei nhengo yemhuri ine chirwere cheprion?

Chirwere chakadai chinogona kuchinja hupenyu hwako hwezuva nezuva. Paunenge uchinetseka nekusuwa kwekunzwa kuti waunoda ane chirwere chisingarapiki, unofanirawo kuronga kuti uchatarisira sei nhengo yemhuri yako. Heano mamwe mazano:

• Gadzira hurongwa hwekutarisira : Zvichienderana nemamiriro ako ezvinhu, nhengo yemhuri yako ingangoda kutarisirwa munzvimbo yekugara, kana kuti ingangoda kutarisirwa kumba, ichivimba nemhuri, shamwari, uye vabatsiri vehutano. Munhu waunoda angangoda rubatsiro pamabasa ezuva nezuva, akadai sekuenda kuchimbuzi nekudya.
• Ronga kutarisirwa kwevanorwara zvikuru : Chirwere chePrion chirwere chinouraya chinogona kungoerekana chaitika uye chikawedzera nekukurumidza. Kana ukaronga kutarisirwa kwevanorwara zvikuru pachine nguva, mudiwa wako anogona kuchigamuchira kana achichida.
• Gadzira nharaunda yakanyarara : Vanhu vane zvirwere zve prion vanowanzo nzwa zvisingawanzoitiki kana paine zviitiko zvinongoerekana zvaitika (semuenzaniso, kubata mumwe munhu, kunzwa ruzha rukuru, kuva munzvimbo dzakazara vanhu). Vanogona kushatirwa kana kutsamwa. Kutarisira nharaunda yavo kunogona kuvabatsira kuti vagare vakadzikama uye vakasununguka.

Panguva yakaita seiyi, zvakakosha kuti muchengeti wevana azvifungirewo. Iwe unodawo zororo nerutsigiro. Usaedze kutakura mutoro uyu uri wega.

Ndinofanira kuona chiremba rini?

Zviratidzo zvezvirwere zveprion zvinowanzowedzera nekukurumidza. Kana iwe kana mumwe munhu mumhuri yako aine chirwere cheprion, unofanira kushanda navanachiremba vako kuti ugadzirise migumisiro yechirwere.

Zvikuru sei, kana zviratidzo zvambotaurwa (zvakadai sekukanganwa, kunetseka kufamba, kuoma kutaura, kuchinja kwehunhu) zvikatanga kamwe kamwe uye zvikawedzera nekukurumidza, tsvaga rubatsiro rwechiremba nekukasika.

Mibvunzo ipi yandinofanira kubvunza chiremba wangu?

Kuziva kuti iwe kana mumwe munhu mumhuri yako ane chirwere cheprion kunogona kukushamisa zvikuru. Iwe nemhuri yako mune matambudziko mazhinji ikozvino, uye mamwe achamuka sezvo chirwere ichi chichienderera mberi. Heano mimwe mibvunzo yekukubatsira kunzwisisa zvaunotarisira:

• Unoziva sei kuti ndine chirwere cheprion?
• Chirwere chepi cheprion chandinacho?
• Izvozvo zvinondikanganisa sei?
• Chii chandinofanira kuita zvinotevera?
• Ndingaitei kuti nditarisire nhengo yemhuri yangu?
• Mhuri yangu inofanira kupiwa mazano nekuongororwa majini here?

Pakupedzisira, zvinhu zvekurangarira

Zvirwere zvePrion iboka rezvirwere zvisingawanzoitiki uye zvinouraya zvinokanganisa uropi hwako. Zviratidzo zvePrion zvinogona kungoerekana zvaonekwa uye zvikawedzera nekukurumidza. Zvinogona kurwadza kuziva kuti iwe kana mumwe munhu waunoda ane chirwere chisina mushonga kana mushonga wekuchidzora.

Zvisinei, kune mishonga inobatsira kudzora zviratidzo . Uye kune mapurogiramu nemasevhisi ekubatsira avo vanobatwa nezvirwere zveprion nevanovachengeta. Kana iwe kana mumwe munhu waunoda aine chirwere cheprion, usatya kubvunza mibvunzo uye kutsvaga rubatsiro. Hausi wega.

` Chirwere chePrio, Chirwere cheHuropi, Chirwere cheHuropi, Dementia, CJD, Chirwere cheMajini