Yintoni isifo sePron?

Umxholo

• Yintoni isifo seprion?
• Iintlobo zezifo ze-prion
• Izifo ezenziwa ngabantu
• Izifo zezilwanyana zezilwanyana
• Zeziphi izinto eziphambili zomngcipheko wesifo se-prion?
• Zithini iimpawu zesifo seprion?
• Sichongwa njani isifo se-prion?
• Sinyangwa njani isifo seprion?
• Ngaba isifo seprion sinokuthintelwa?
• Iindlela zokuthatha eziphambili

Izifo ze-Prion liqela leengxaki zokuphazamiseka kwengqondo ezinokuchaphazela abantu kunye nezilwanyana.

Zibangelwa kukususwa kweeproteni ezisongelwe ngokungaqhelekanga kwingqondo, ezinokubangela utshintsho kwi:

• imemori
• indlela yokuziphatha
• intshukumo

Izifo ze-Prion zinqabile kakhulu. Phantse ama-350 amatyala amatsha esifo se-prion axelwa rhoqo ngonyaka e-United States.

Abaphandi basasebenza ukuqonda okungakumbi ngezi zifo kunye nokufumana unyango olusebenzayo. Okwangoku, izifo ze-prion zihlala zibulala.

Zithini iintlobo ezahlukeneyo zesifo seprion? Ungazihlakulela njani? Kwaye ngaba ikhona indlela yokubathintela?

Qhubeka ufunda ukuze ufumane iimpendulo zale mibuzo nangaphezulu.

Yintoni isifo seprion?

Izifo ze-Prion zibangela ukwehla okuqhubekayo kokusebenza kwengqondo ngenxa yokuphambuka kweeproteni kwingqondo- ngakumbi ukungafakwanga kakuhle kweeprotein ezibizwa ngokuba ziiprotein prion (PrP).

Umsebenzi oqhelekileyo wezi proteni awaziwa okwangoku.

Kubantu abanesifo se-prion, i-PrP ephosakeleyo inokubopha kwi-PrP esempilweni, ebangela ukuba iprotheyini esempilweni iphinde isonge ngokungaqhelekanga.

I-PrP eyonelisekileyo iqala ukuqokelela kunye nokwenza iifom ngaphakathi kwengqondo, ukonakalisa nokubulala iiseli zemithambo-luvo.

Lo monakalo ubangela imingxunya emincinci ukuba yenze izicwili zobuchopho, isenza ukuba sibonakale sifana nesiponji phantsi kwemicroscope. Ngapha koko, usenokubona nezifo zeprion ezibizwa ngokuba yi "spongiform encephalopathies."

Unokuphuhlisa isifo se-prion ngeendlela ezahlukeneyo, ezinokubandakanya:

• Ifunyenwe. Ukubonakaliswa kwi-PrP engaqhelekanga evela kumthombo ongaphandle kunokwenzeka ngokutya okungcolileyo okanye izixhobo zonyango.
• Ilifa. Utshintsho olukhoyo kuhlobo lweekhowudi ze-PrP lukhokelela kwimveliso ye-PrP eneempazamo.
• Ngamaxesha athile. I-PrP eyoneleyo inokukhula ngaphandle kwesizathu esaziwayo.

Iintlobo zezifo ze-prion

Isifo se-Prion sinokubakho ebantwini nasezilwanyaneni. Apha ngezantsi kukho iintlobo ezahlukeneyo zezifo ze-prion. Olunye ulwazi malunga nesifo ngasinye lulandela itafile.

Izifo ezenziwa ngabantu

• Isifo seCreutzfeldt-Jakob (CJD). Ukuchazwa okokuqala kwi-1920, i-CJD inokufunyanwa, ifunyenwe njengelifa, okanye yenzeke kancinci. ye-CJD ayifane yenzeke.
• Isifo esahlukileyo seCreutzfeldt-Jakob isifo (vCJD). Le fomu ye-CJD inokufunyanwa ngokutya inyama engcolileyo yenkomo.
• Ukuphulukana nokufa okungaziwayo (FFI). I-FFI ichaphazela i-thalamus, eyinxalenye yengqondo yakho elawula ukujikeleza kunye nokuvuka kwemijikelezo. Olunye lweempawu eziphambili zale meko kukuqaqamba kobuthongo. Utshintsho luzuzwa ngendlela ebalaseleyo, oko kuthetha ukuba umntu ochaphazelekayo unethuba lokulidlulisela kubantwana babo ngama-50 eepesenti.
• Isifo sikaGerstmann-Straussler-Scheinker (GSS). I-GSS nayo izuzwe njengelifa. Njenge-FFI, idluliselwa ngendlela ebalaseleyo. Ichaphazela i-cerebellum, eyinxalenye yengqondo elawula ibhalansi, ulungelelwaniso kunye nokulingana.
• Kuru. UKuru wachongwa kwiqela labantu abavela eNew Guinea. Esi sifo sasidluliselwa ngendlela ethile yokutya abantu apho izidlo zezalamane eziswelekileyo zazisetyenziswa.

Izifo zezilwanyana zezilwanyana

• I-encephalopathy ye-Bovine spongiform (BSE). Ngokuqhelekileyo kuthiwa "sisifo seenkomo ezihlanyayo," olu hlobo lwesifo se-prion sichaphazela iinkomo. Abantu abatya inyama yeenkomo nge-BSE banokuba semngciphekweni we-vCJD.
• Ukuchithwa kwesifo esinganyangekiyo (CWD). I-CWD ichaphazela izilwanyana ezinjengexhama, i-moose, kunye ne-elk. Ifumana igama layo ekunciphiseni ubunzima bomzimba obubonwa kwizilwanyana ezigulayo.
• Iscrapie. I-Scrapie lolona hlobo ludala lwesifo se-prion, esele ichaziwe njengakwi-1700s. Ichaphazela izilwanyana ezifana neegusha neebhokhwe.
• I-Feline spongiform encephalopathy (FSE). I-FSE ichaphazela iikati zasekhaya kunye neekati zasendle ekuthinjweni. Uninzi lweziganeko ze-FSE zenzekile e-United Kingdom naseYurophu.
• Ukudluliselwa kwemink encephalopathy (TME). Olu hlobo lunqabileyo lwesifo se-prion luchaphazela i-mink. I-mink sisilwanyana esincancisayo esincinci esidla ngokukhuliselwa ukuveliswa koboya.
• Yenza i-encephalopathy yesiponiform. Esi sifo sinqabile kwaye sichaphazela izilwanyana ezingaqhelekanga ezinxulumene neenkomo.

Zeziphi izinto eziphambili zomngcipheko wesifo se-prion?

Zininzi izinto ezinokukubeka emngciphekweni wokuhlaselwa sisifo seprion. Oku kubandakanya:

• Imfuza. Ukuba umntu osapho lwakho unesifo esivela kwilifa, usemngciphekweni wokufumana utshintsho.
• Ubudala. Izifo ezixhaphakileyo ze-prion zihlala zikhula kubantu abadala.
• Iimveliso zezilwanyana. Ukutya iimveliso zezilwanyana ezingcoliswe yiprion kunokuhambisa isifo se-prion kuwe.
• Iinkqubo zonyango. Izifo ze-Prion zinokuhanjiswa ngezixhobo zonyango ezingcolileyo kunye nezicubu zemithambo-luvo. Iimeko apho oku kwenzekileyo kubandakanya ukuhanjiswa ngokudluliselwa kwe-cornea engcolileyo okanye i-graf mater graf.

Zithini iimpawu zesifo seprion?

Izifo ze-Prion zinexesha elide lokufukama, zihlala ngokulandelelana kweminyaka emininzi. Xa iimpawu zikhula, ziqhubeka zisiba mandundu, ngamanye amaxesha ngokukhawuleza.

Iimpawu eziqhelekileyo zesifo se-prion zibandakanya:

• ubunzima bokucinga, inkumbulo kunye nokugweba
• utshintsho kubuntu njengokungakhathali, ukuphazamiseka, kunye noxinzelelo
• ukudideka okanye ukudideka
• ukuphazamiseka kwemisipha okungazibandakanyi (myoclonus)
• ukulahleka kokulungelelanisa (ataxia)
• ingxaki yokulala (ukulala)
• intetho enzima okanye engacacanga
• ukungaboni kakuhle okanye ukungaboni kakuhle

Sichongwa njani isifo se-prion?

Kuba izifo ze-prion zinokubonisa iimpawu ezifanayo kwezinye iingxaki ze-neurodeergenerative, kunokuba nzima ukufumanisa isifo.

Ekuphela kwendlela yokuqinisekisa ukuxilongwa kwesifo se-prion kukusebenzisa i-biopsy yengqondo emva kokufa.

Nangona kunjalo, umboneleli wezempilo unokusebenzisa iimpawu zakho, imbali yezonyango, kunye novavanyo oluninzi ukunceda ukufumanisa isifo se-prion.

Uvavanyo abanokuzisebenzisa lubandakanya:

• Imagnetic resonance imaging (iMRI). I-MRI inokwenza umfanekiso ocacileyo wengqondo yakho. Oku kunokunceda ababoneleli ngezempilo ukuba babone utshintsho kulwakhiwo lobuchopho olunxulunyaniswa nesifo se-prion.
• Uvavanyo lweCerebrospinal fluid (CSF). I-CSF inokuqokelelwa kwaye ivavanyelwe ukumakishwa okunxulunyaniswa ne-neurodegeneration. Ngo-2015, uvavanyo lwaphuhliswa ukufumanisa ngokukodwa iimpawu zesifo somntu.
• Electroencephalography (I-EEG). Olu vavanyo lurekhoda umsebenzi wombane kwingqondo yakho.

Sinyangwa njani isifo seprion?

Okwangoku akukho lonyango lwesifo se-prion. Nangona kunjalo, unyango lujolise ekunikezeleni ngononophelo lwenkxaso.

Imizekelo yolu hlobo lokhathalelo ibandakanya:

• Amayeza. Amanye amayeza anokumiselwa ukunceda ukunyanga iimpawu. Imizekelo ibandakanya:
  - ukunciphisa iimpawu zengqondo kunye nokudambisa okanye ukuthomalalisa
  -Ukubonelela ngesiqabu seentlungu usebenzisa amayeza e-opiate
  -Ukunciphisa izihlunu zemisipha kunye neziyobisi ezinjenge-sodium valproate kunye ne-clonazepam
• Uncedo. Njengoko esi sifo sihambela phambili, abantu abaninzi bafuna uncedo lokuzinakekela nokwenza imisebenzi yemihla ngemihla.
• Ukubonelela ngamanzi kunye nezondlo. Kumanqanaba aphezulu esi sifo, i-IV fluid okanye ityhubhu yokondla inokufuneka.

Iinzululwazi ziyaqhubeka nokusebenza ukufumana unyango olusebenzayo kwizifo ze-prion.

Ezinye zeendlela ezinokubakho zonyango eziphandwayo zibandakanya ukusetyenziswa kwee-anti-prion antibodies kunye "" ezithintela ukuphindaphinda kwe-PrP engaqhelekanga.

Ngaba isifo seprion sinokuthintelwa?

Kuthathwe amanyathelo aliqela ukukhusela ukuhanjiswa kwezifo ezifumaneka kwi-prion. Ngenxa yala manyathelo asebenzayo, ukufumana isifo se-prion kukutya okanye kwindawo yezonyango ngoku kunqabile kakhulu.

Amanye amanyathelo okhuseleko athathiweyo abandakanya:

• ukumisela imigaqo eqinileyo yokungenisa iinkomo kumazwe apho iBSE yenzeka khona
• ukuthintela iinxalenye zenkomo ezinje ngengqondo kunye nentambo yomqolo ekubeni isetyenziswe ekutyeni kwabantu okanye izilwanyana
• Ukuthintela abo banembali okanye abasemngciphekweni wokuchaphazeleka kwisifo se-prion ngokunikela ngegazi okanye ezinye izicwili
• usebenzisa amanyathelo okunyanzelisa inzalo kwisixhobo sonyango esiye sanxibelelana nethishu yovalo yomntu onesifo esikrokrelwayo se-prion
• ukutshabalalisa izixhobo zonyango ezilahlwayo

Okwangoku akukho ndlela yokuthintela iintlobo ezizuzwe njengelifa okanye ezinqabileyo zesifo seprion.

Ukuba umntu osapho lwakho unesifo se-prion esizuzwe njengelifa, unokucinga ngokubonisana nomcebisi ngemfuza ukuxoxa ngomngcipheko wokuba nesi sifo.

Iindlela zokuthatha eziphambili

Izifo ze-Prion liqela elinqabileyo leengxaki ze-neurodegenerative ezibangelwa yiprotheyini esongelwe ngokungaqhelekanga kwingqondo yakho.

Iiprotheyini ezingasetyenziswanga kakuhle zenza iiclumps ezonakalisa iiseli zemithambo-luvo, zikhokelela ekwehleni okuqhubekayo kokusebenza kwengqondo.

Ezinye izifo ze-prion zihanjiswa ngofuzo, ngelixa ezinye zinokufunyanwa ngokutya okungcolileyo okanye izixhobo zonyango. Ezinye izifo ze-prion zikhula ngaphandle kwesizathu esaziwayo.

Okwangoku akukho lunyango lwezifo ze-prion. Endaweni yoko, unyango lujolise ekunikezeleni ngononophelo lwenkxaso kunye nokunciphisa iimpawu.

Abaphandi bayaqhubeka nokusebenza ukufumana okuninzi ngezi zifo kunye nokuphuhlisa unyango.