Siyini isifo se-Prion?

-Delile

• Siyini isifo se-prion?
• Izinhlobo zezifo ze-prion
• Izifo ze-prion zabantu
• Izifo ze-prion yezilwane
• Yiziphi izimbangela eziyinhloko zesifo se-prion?
• Ziyini izimpawu zesifo se-prion?
• Sitholakala kanjani isifo se-prion?
• Selashwa kanjani isifo se-prion?
• Ngabe isifo se-prion singavinjelwa?
• Ukuthatha ukhiye

Izifo ze-Prion ziyiqembu lezinkinga ze-neurodegenerative ezingathinta abantu nezilwane.

Zibangelwa ukufakwa kwamaprotheni agoqwe ngokungajwayelekile ebuchosheni, okungadala ushintsho ku:

• inkumbulo
• ukuziphatha
• ukunyakaza

Izifo ze-Prion azivamile kakhulu. Cishe amacala amasha we-350 wesifo se-prion abikwa minyaka yonke e-United States.

Abaphenyi basasebenza ukuqonda okuningi ngalezi zifo nokuthola ukwelashwa okusebenzayo. Njengamanje, izifo ze-prion zihlala zibulala ekugcineni.

Yiziphi izinhlobo ezahlukahlukene zesifo se-prion? Ungazihlakulela kanjani? Futhi ingabe ikhona indlela yokuzivimbela?

Qhubeka ufunde ukuthola izimpendulo zale mibuzo nokuningi.

Siyini isifo se-prion?

Izifo ze-Prion zibangela ukwehla okuqhubekayo kokusebenza kobuchopho ngenxa yokwakhiwa okungalungile kwamaprotheni ebuchosheni - ikakhulukazi ukugoqwa okungalungile kwamaprotheni abizwa ngama-prion protein (PrP).

Umsebenzi ojwayelekile wala maprotheni okwamanje awaziwa.

Kubantu abanesifo se-prion, i-PrP efingqiwe ingabopha kwi-PrP enempilo, ebangela ukuthi iphrotheni elinempilo liphinde ligoqeke ngokungavamile.

I-PrP enelisiwe iqala ukuqoqa futhi yakhe ama-clumps ngaphakathi kobuchopho, ilimaze futhi ibulale amangqamuzana ezinzwa.

Lo monakalo ubangela izimbobo ezincane ukuba zakhe ezicutshini zobuchopho, zenze kubonakale kufana nesiponji ngaphansi kwemicroscope. Eqinisweni, ungabona nezifo ze-prion ezibizwa nge- "spongiform encephalopathies."

Ungasungula isifo se-prion ngezindlela ezahlukahlukene, ezingafaka:

• Kutholakele. Ukuchayeka ku-PrP engajwayelekile evela kumthombo wangaphandle kungenzeka ngokudla noma imishini yezokwelapha engcolile.
• Ilifa. Ukuguqulwa kwezakhi zofuzo okukhona kuhlobo lofakelo lwe-PrP kuholela ekukhiqizweni kwe-PrP efingqiwe.
• Ngezikhathi ezithile. I-PrP egculisiwe ingahle ikhule ngaphandle kwesizathu esaziwayo.

Izinhlobo zezifo ze-prion

Isifo se-Prion singenzeka kubantu nasezilwaneni. Ngezansi kunezinhlobo ezithile zezifo ze-prion. Imininingwane engaphezulu ngesifo ngasinye ilandela ithebula.

Izifo ze-prion zabantu

• Isifo seCreutzfeldt-Jakob (CJD). Ichazwe okokuqala ngo-1920, i-CJD ingatholwa, izuzwe njengefa, noma ivele kancane. ye-CJD ayivelakancane.
• Isifo esihlukile seCreutzfeldt-Jakob (vCJD). Leli fomu le-CJD lingatholakala ngokudla inyama engcolisiwe yenkomo.
• Ukuqothuka Okudabukisayo Okudabukisayo (FFI). I-FFI ithinta i-thalamus, okuyingxenye yobuchopho bakho elawula imijikelezo yokulala nokuvuka. Esinye sezibonakaliso eziyinhloko zalesi simo ukuqwasha kuyanda. Ukuguqulwa kutholakala njengefa ngendlela ebabazekayo, okusho ukuthi umuntu othintekile unethuba elingamaphesenti angama-50 lokulidlulisela ezinganeni zakhe.
• Isifo seGerstmann-Straussler-Scheinker (GSS). I-GSS nayo izuzwe njengefa. Njenge-FFI, idluliselwa ngendlela evelele. Kuthinta i-cerebellum, okuyingxenye yobuchopho ephatha ibhalansi, ukuxhumanisa, kanye nokulingana.
• Kuru. UKuru wakhonjwa eqenjini labantu baseNew Guinea. Lesi sifo sasidluliselwa ngohlobo oluthile lomkhuba wokudla abantu lapho kudliwa khona izinsalela zezihlobo ezingasekho.

Izifo ze-prion yezilwane

• I-Bovine spongiform encephalopathy (BSE). Ngokuvamile esibizwa ngokuthi "isifo sezinkomo ezihlanyayo," lolu hlobo lwesifo se-prion sithinta izinkomo. Abantu abadla inyama yezinkomo ezine-BSE bangaba sengozini ye-vCJD.
• Isifo esichitha isikhathi eside (CWD). I-CWD ithinta izilwane ezinjengezinyamazane, i-moose, ne-elk. Ithola igama layo ekulahlekelweni kwesisindo okukhulu okubonwa ezilwaneni ezigulayo.
• I-Scrapie. I-Scrapie yindlela endala kunazo zonke yesifo se-prion, esichazwe emuva kakhulu njengama-1700s. Kuthinta izilwane ezifana nezimvu nezimbuzi.
• I-Feline spongiform encephalopathy (FSE). I-FSE ithinta amakati afuywayo namakati asendle ekuthunjweni. Amacala amaningi e-FSE enzekile e-United Kingdom nase-Europe.
• I-Transceissible mink encephalopathy (TME). Le fomu engavamile kakhulu yesifo se-prion ithinta i-mink. I-mink isilwane esincelisayo esincane esivame ukuphakanyiselwa ukukhiqizwa koboya.
• Hlanganisa i-spongiform encephalopathy. Lesi sifo se-prion futhi asivamile futhi sithinta izilwane ezingaphandle ezihlobene nezinkomo.

Yiziphi izimbangela eziyinhloko zesifo se-prion?

Izici eziningana zingakubeka engcupheni yokuhlaselwa yisifo se-prion. Lokhu kufaka phakathi:

• Izakhi zofuzo. Uma othile emndenini wakho enesifo se-prion esizuzwe njengefa, usengozini enkulu yokuba nokuguquka.
• Ubudala. Izifo ze-proradic prion zivame ukukhula kubantu abadala.
• Imikhiqizo yezilwane. Ukudla imikhiqizo yezilwane engcoliswe yi-prion kungadlulisa isifo se-prion kuwe.
• Izinqubo zokwelashwa. Izifo ze-Prion zingadluliselwa ngemishini yezokwelapha engcolile kanye nezicubu zemizwa. Amacala lapho lokhu kwenzeke khona afaka ukudluliswa ngokufakelwa kwe-cornea okungcolisiwe noma ama-graf mater graf.

Ziyini izimpawu zesifo se-prion?

Izifo ze-Prion zinezikhathi ezinde kakhulu zokufukamela, imvamisa ngokulandelana kweminyaka eminingi. Lapho izimpawu zikhula, ziya ngokuya ziba zimbi, kwesinye isikhathi zisheshe.

Izimpawu ezivamile zesifo se-prion zifaka:

• ubunzima ngokucabanga, inkumbulo, nokwahlulela
• izinguquko zobuntu ezinjengokunganaki, ukudinwa, nokudangala
• ukudideka noma ukudideka
• ukuphazamiseka kwemisipha okungazibandakanyi (myoclonus)
• ukulahleka kokuxhumana (ataxia)
• ukukhathazeka ukulala (ukulala)
• inkulumo enzima noma engacacile
• ukungaboni kahle noma ukungaboni

Sitholakala kanjani isifo se-prion?

Njengoba izifo ze-prion zingaveza izimpawu ezifanayo kwezinye izifo ze-neurodegenerative, kungaba nzima ukuzihlonza.

Ukuphela kwendlela yokuqinisekisa ukutholakala kwesifo se-prion kungukuhlolwa kwengqondo okwenziwa ngemuva kokufa.

Kodwa-ke, umhlinzeki wezokunakekelwa kwempilo angasebenzisa izimpawu zakho, umlando wezokwelapha, kanye nokuhlolwa okuningana ukusiza ukuthola isifo se-prion.

Izivivinyo abangazisebenzisa zifaka:

• Ukucabanga kwe-Magnetic resonance (MRI). I-MRI ingakha isithombe esiningiliziwe sobuchopho bakho. Lokhu kungasiza abahlinzeki bezempilo ukuthi babone izinguquko ezakhiweni zobuchopho ezihlotshaniswa nesifo se-prion.
• Ukuhlolwa kweCerebrospinal fluid (CSF). I-CSF ingaqoqwa futhi ihlolwe omaka abahlotshaniswa ne-neurodegeneration. Ngo-2015, kwasungulwa isivivinyo ukuze kutholakale ngqo izimpawu zesifo se-prion yabantu.
• I-Electroencephalography (I-EEG). Lokhu kuhlolwa kuqopha umsebenzi kagesi ebuchosheni bakho.

Selashwa kanjani isifo se-prion?

Okwamanje alikho ikhambi lesifo se-prion. Kodwa-ke, ukwelashwa kugxile ekunikezeni ukunakekelwa okusekelayo.

Izibonelo zalolu hlobo lokunakekelwa zifaka:

• Imithi. Eminye imithi ingabhalwa ukusiza ukwelapha izimpawu. Izibonelo zifaka:
  - ukunciphisa izimpawu ezingokwengqondo ngama-anti-depressants noma ama-sedatives
  - ukuhlinzeka ukukhululeka kobuhlungu kusetshenziswa imishanguzo ye-opiate
  - ukunciphisa imisipha yemithi ngezidakamizwa ezifana ne-sodium valproate ne-clonazepam
• Usizo. Njengoba lesi sifo siqhubeka, abantu abaningi badinga usizo lokuzinakekela nokwenza imisebenzi yansuku zonke.
• Ukunikezela nge-hydration nezakhamzimba. Ezigabeni ezithuthukile zalesi sifo, kungadingeka uketshezi lwe-IV noma ishubhu yokondla.

Ososayensi bayaqhubeka nokusebenza ukuthola ukwelashwa okuphumelelayo kwezifo ze-prion.

Ezinye izindlela zokwelapha ezingaphenywa zifaka phakathi ukusetshenziswa kwama-anti-prion antibodies kanye no "" okuvimbela ukuphindaphindeka kwe-PrP engajwayelekile.

Ngabe isifo se-prion singavinjelwa?

Kuthathwe izinyathelo eziningana ukuvimbela ukudluliswa kwezifo ze-prion ezitholakele. Ngenxa yalezi zinyathelo ezisebenzayo, ukuthola isifo se-prion ekudleni noma endaweni yezokwelapha manje sekuyivelakancane kakhulu.

Ezinye zezinyathelo zokuvikela ezithathiwe zifaka:

• ukubeka imithetho eqinile yokungenisa izinkomo emazweni lapho kwenzeka khona i-BSE
• ukuvimbela izingxenye zenkomo njengobuchopho nomgogodla ukuthi zisetshenziswe ekudleni kwabantu noma izilwane
• ukuvimbela labo abanomlando noma abasesengozini yokuchayeka kwisifo se-prion ekunikeleni ngegazi noma ezinye izicubu
• kusetshenziswa izindlela eziqinile zokuvala inzalo entweni yezokwelapha eye yahlangana nezicubu zomzimba zomuntu onesifo se-prion esisolwayo
• ukubhubhisa amathuluzi wezokwelapha alahlwayo

Okwamanje ayikho indlela yokuvimbela amafomu we-prion azuzwe njengefa noma okungajwayelekile.

Uma othile emndenini wakho enesifo esivela ku-prion, ungacabanga ukubonisana nomeluleki wezofuzo ukuze uxoxe ngengozi yakho yokuthola lesi sifo.

Ukuthatha ukhiye

Izifo ze-Prion ziyiqembu elingajwayelekile lezinkinga ze-neurodegenerative ezibangelwa amaprotheni agoqwe ngokungajwayelekile ebuchosheni bakho.

Amaprotheni asongelwe kahle enza ama-clumps alimaza amangqamuzana ezinzwa, aholele ekwehleni okuqhubekayo kokusebenza kobuchopho.

Ezinye izifo ze-prion zidluliselwa ngofuzo, kanti ezinye zingatholakala ngokudla okungcolile noma ngemishini yezokwelapha. Ezinye izifo ze-prion zikhula ngaphandle kwesizathu esaziwayo.

Okwamanje alikho ikhambi lezifo ze-prion. Esikhundleni salokho, ukwelashwa kugxile ekunikezeni ukunakekelwa okusekelayo kanye nezimpawu zokunciphisa.

Abaphenyi bayaqhubeka nokusebenza ukuthola okuningi ngalezi zifo nokuthuthukisa izindlela zokwelashwa ezingaba khona.