Tusitala: Louise Ward
Aso O Foafoaga: 5 Fepuari 2021
Faafouga Aso: 11 Aukuso 2025
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Anotusi

O faʻamaʻi Prion o se vaega o faʻaletonu o le neurodegenerative e mafai ona aʻafia ai tagata ma manu.

O nei mafuaʻaga e ala i le teuina o le masani ai gaugau proteins i le faiʻai, lea e mafai ona mafua ai suiga i:

  • manatua
  • amio
  • gaioi

E seasea maua faʻamaʻi Prion. E tusa ma le 350 mataupu fou o faʻamaʻi prion e lipotia i tausaga taʻitasi i le Iunaite Setete.

O loʻo galulue pea tagata suʻesuʻe e malamalama atili e uiga i nei faʻamaʻi ma ia maua se togafitiga lelei. I le taimi nei, o faʻamaʻi prion e iʻu lava ina oti.

O a ituaiga eseese o faʻamaʻi prion? Faʻafefea ona e atinaʻeina? Ma e i ai se auala e puipuia ai i latou?

Faʻaauau faitau e iloa ai tali i nei fesili ma sili atu.

O le a le maʻi prion?

Prion faamaʻi mafua ai le alualu i luma alualu i luma o faiʻai gaioiga ona o le sese faʻatulagaina o polotini i le faiʻai - aemaise lava le faʻasologa o polotini taʻua prion proteins (PrP).

O le masani ai galuega o nei polotini e leʻo iloa nei.


I tagata e maua i faʻamaʻi prion, o le sese faʻapipiʻi PrP mafai ona fusifusia i le maloloina PrP, lea mafua ai le maloloina polotini e gaugau foi le masani ai.

Misfolded PrP amata ona faʻaputuputu ma fausia ni faʻaputuga i totonu o le faiʻai, faʻaleagaina ma fasioti neula sela.

O lenei faʻaleagaina mafua ai pu laiti e fausiaina i faiʻai sela, faia foliga mai omomi pei o lalo o se microscope. O le mea moni, oe ono vaʻaia foi faʻamaʻi prion taʻua o "spongiform encephalopathies."

E mafai ona e atiaʻe se faʻamaʻi prion i ni auala eseʻese, e ono aofia ai:

  • Mauaina. Faʻaalia i le le talafeagai PrP mai se puna i fafo atu mafai ona tupu e ala i meaai faʻaleagaina poʻo meafaigaluega faafomaʻi.
  • Tofiina Fetuunaiga o loʻo i ai i le kenera o tulafono laiti mo le PrP e tau atu ai i le gaosiga o le sese o le PrP.
  • Sporadic. E le mafai ona faʻapipiʻi PrP e aunoa ma se mafuaʻaga lauiloa.

Ituaiga o faʻamaʻi prion

E mafai ona tupu faʻamaʻi Prion i tagata uma ma manu. Lalo o nai eseʻese ituaiga o faʻamaʻi prion. Nisi faʻamatalaga e uiga i faʻamaʻi taʻitasi mulimuli i le laulau.


Tagata faʻamaʻi prionFaamaʻi prion manu
Creutzfeldt-Jakob faamaʻi (CJD)Bovine spongiform encephalopathy (BSE)
Variant Creutzfeldt-Jakob faamaʻi (vCJD)Faamaʻi maʻimau taimi (CWD)
Le mautonu o le aiga (FFI)Scrapie
Gerstmann-Straussler-Scheinker syndrome (GSS)Feline spongiform encephalopathy (FSE)
KuruTransmissible mink encephalopathy (TME)
Ungulate spongiform encephalopathy

Tagata faʻamaʻi prion

  • Creutzfeldt-Jakob faamaʻi (CJD). Muamua faʻamatalaina i le 1920, CJD mafai ona mauaina, tofiina, pe sporadic. ole CJD e taʻatele.
  • Variant Creutzfeldt-Jakob faamaʻi (vCJD). Lenei ituaiga o CJD mafai ona maua e ala i le 'aʻafia o aano pisapisao o se povi.
  • Le mautonu o le aiga (FFI). FFI afaina le thalamus, o le vaega lea o lou faiai e puleaina moe ma ala faʻamoeina. O se tasi o faʻailoga iloga o lenei tulaga o le faʻateteleina o le le mautonu. O le suiga e maua i se tulaga malosi, o lona uiga o le tagata aʻafia e iai le 50 pasene avanoa e tuʻuina atu ai ia latou fanau.
  • Gerstmann-Straussler-Scheinker syndrome (GSS). GSS foi ua maua. Pei ole FFI, e tuʻufaʻatasia i se faiga sili. E afaina ai le cerebellum, o le vaega lea o le faiʻai e puleaina paleni, faʻamaopoopoina, ma paleni paleni.
  • Kuru. O Kuru na iloa i se vaega o tagata mai New Guinea. O lea faamaʻi na feaveaia e ala i tu ma aga masani o le ituaiga o tagata, lea na susunuina ai toega o aiga ua maliliu.

Faamaʻi prion manu

  • Bovine spongiform encephalopathy (BSE). E masani ona taʻua ole "mad cow disease," o lenei ituaiga maʻi prion e afaina ai povi. Tagata e 'aʻai aano o manu mai povi ma BSE mafai ona i ai i se tulaga lamatia mo vCJD.
  • Faamaʻi maʻimau taimi (CWD). CWD aafia ai manu pei o aila, moose, ma elk. E maua lona igoa mai le mamafa mamafa pa'ū matauina i maʻi manu.
  • Scrapie Scrapie o le sili ona leva ituaiga o faʻamaʻi prion, na faʻamatalaina i tua atu o le 1700s. E afaina ai manu pei o mamoe ma 'oti.
  • Feline spongiform encephalopathy (FSE). FSE aafia ai pusi fale ma pusi vao i le tafeaga. Ole tele o mataupu ole FSE na tutupu ile United Kingdom ma Europa.
  • Transmissible mink encephalopathy (TME). Lenei seasea lava ituaiga o faʻamaʻi prion aafia mink. O le mink o se tamaʻi meaola e masani na faʻalauteleina mo le gaosiga o fulufulu.
  • Ungulate spongiform encephalopathy. O lenei faʻamaʻi prion e seasea lava foʻi ma aʻafia ai manu feʻai e fesoʻotaʻi ma povi.

O a mea taua muamua e ono afaina ai prion faamaʻi?

E tele mea e ono mafai ai ona e ono aʻafia ile faʻamaʻi prion. E aofia ai:


  • Kenese Afai o se tasi i lou aiga ua maua i se maʻi tupuaga prion, oe i se sili atu aʻafiaga o le i ai foi o le mutation.
  • Tausaga O faʻamaʻi Sporadic prion e masani ona atiaʻe i tagata matutua.
  • Manu oloa. O le faʻaumatiaina o meaola e faʻaleagaina i le prion e mafai ona faʻasalalauina atu ai se faʻamaʻi prion ia oe.
  • Faiga faʻafomaʻi. O faʻamaʻi Prion e mafai ona sosolo e ala i vailaʻau ma vailaʻau ua pisia. O mataupu na tupu ai lenei mea e aofia ai le tuʻufaʻatasia e ala i le faʻafefeteia o togatoto i totonu o le kornea.

O a faʻailoga o faʻamaʻi prion?

O faʻamaʻi Prion e uumi taimi na faʻamama ai, e masani ona tele tausaga. A tupu auga, latou alualu i luma leaga, o nisi taimi vave.

Faʻailoga masani o faʻamaʻi prion aofia ai:

  • faigata i mafaufauga, manatuaina, ma le faʻamasinoga
  • uiga suia e pei o le le fiafia, le mautonu, ma le faanoanoa
  • le mautonu poʻo le le mautonu
  • musele maso lemu (myoclonus)
  • leiloa o faʻamaopoopo (ataxia)
  • faʻalavelave moe (le moe ai)
  • tautalaga faigata poʻo le tautala lemu
  • faʻaletonu le vaʻai poʻo le tauaso

Faʻafefea ona iloa faʻamaʻi prion?

Talu ai o faʻamaʻi prion e mafai ona maua mai ai faʻailoga tutusa i isi faʻaletonu o le neurodegenerative, e ono faigata ai ona suʻesuʻeina.

Pau lava le auala e faʻamaonia ai le faʻamaonia o le faʻamaʻi prion e ala lea ile faia ole biopsy ole faiʻai pe a maliu.

Peitaʻi, e mafai e se tausi soifua maloloina ona faʻaaoga ou faʻailoga, talaʻaga faʻafomaʻi, ma le tele o suʻega e fesoasoani ai e faʻamaonia le faʻamaʻi prion.

O suʻega latou te ono faʻaaofia ai:

  • Maneta resonance imaging (MRI). E mafai e le MRI ona fausia se ata auiliili o lou faiʻai. Ole mea lea e mafai ona fesoasoani ile au faʻafomai e vaʻaia suiga ile faiʻai faiʻai e fesoʻotaʻi ma faʻamaʻi prion.
  • Cerebrospinal suavai (CSF) suʻega. CSF mafai ona aoina ma tofotofoina mo faʻailoga fesoʻotaʻi ma neurodegeneration. I le 2015, na faia ai se suʻega e faʻapitoa lava le faʻailogaina o faʻamaʻi o le prion tagata.
  • Electroencephalography (EEG). O lenei suʻega e faʻamaumau ai le eletise i lou faiai.

Faʻafefea ona togafitia faʻamaʻi prion?

E leai se togafiti mo faʻamaʻi prion. Ae ui i lea, togafitiga e taulaʻi atu i le sauniaina lagolago lagolagosua.

Faʻataʻitaʻiga o lenei ituaiga o tausiga e aofia ai:

  • Vailaau. O nisi vailaʻau mafai ona faatonuina e fesoasoani togafitia auga. O faʻataʻitaʻiga e aofia ai:
    - faʻaititia faʻalemafaufau faʻailoga ma antidepressants poʻo vailaʻau faʻamalieina
    - faʻaavanoa tiga tiga i le faʻaaogaina o vailaʻau oona
    - faʻamamaina maso maso ma vailaʻau pei o sodium valproate ma clonazepam
  • Fesoasoani A o agaʻi atu i luma le faʻamaʻi, e tele tagata e manaʻomia le fesoasoani i le tausiga o latou lava ma faʻatinoina gaioiga i aso uma.
  • Tuʻuina atu hydration ma niuterene. I le alualu i luma laʻasaga o le faʻamaʻi, IV vai poʻo se fafagaina paipa ono manaʻomia.

Sa faaauau pea ona galulue saienitisi e saili se togafitiga lelei mo maʻi prion.

Nisi o togafitiga ono o loʻo suʻesuʻeina e aofia ai le faʻaaogaina o anti-prion antibodies ma le "" e taofia ai le toe faia o faʻaletonu PrP.

E mafai ona puipuia le faʻamaʻi prion?

E tele gaioiga ua faia e puipuia ai le feaveaia o maua prion faamaʻi. Ona o nei gaioiga vave, o le mauaina o se prion faʻamaʻi mai meaai pe mai se fomaʻi nofoaga ua matua seasea lava.

O nisi o laʻasaga puipuia e aofia ai:

  • faʻatulagaina tulafono faʻatapulaʻa ile faʻatauina mai o povi mai atunuʻu e iai le BSE
  • faasaina ia vaega o le povi pei o le faiʻai ma tuaulaula mai le faʻaaogaina i meaai mo tagata poʻo manu
  • puipuia i latou e iai se talaʻaga poʻo ni lamatiaga mo le aafia i faʻamaʻi prion mai le foaʻiina atu o le toto poʻo isi mea
  • faʻaaogaina o ni metotia faʻamalosi tino e faʻamalosia ai mea faʻafomaʻi na fesoʻotaʻi ma le neura o se tasi e masalomia o loʻo maua i se maʻi prion
  • faʻaleagaina mea faʻafomaʻi e mafai ona tiaʻi

E leai se auala i le taimi nei e puipuia ai measina tuʻufaʻatasi poʻo sporadic o prion faʻamaʻi.

Afai o se tasi o lou aiga na maua i se faʻamaʻi tupuaga prion, oe ono mafaufau e faʻafesoʻotaʻi ma se fesoasoani i le kenera e talanoaina ai lou ono aʻafia i le faʻamaʻi.

Faʻaaogaina taua

O faʻamaʻi Prion o se vaega e seasea tupu i le neurodegenerative faʻafitauli e mafua mai i le le masani ai gaugau porotini i lou faiʻai.

O le misfolded protein fausia kulupu e faʻaleagaina neula sela, ma mafua ai i le alualu i luma alualu i luma i le faia o faiʻai.

O nisi faʻamaʻi prion e faʻasalalauina faʻamaʻi, aʻo isi e mafai ona maua e ala i meaʻai faʻaleagaina poʻo mea faigaluega faʻafomaʻi. Isi faʻamaʻi prion atiaʻe aunoa ma se mafuaʻaga iloa.

E leai se togafiti mo faʻamaʻi prion. Nai lo lena, togafitiga taulai atu i le tuuina atu lagolago lagolagosua ma faamamaina auga.

O loʻo faʻaauau pea ona galulue tagata suʻesuʻe e saili atili e uiga i nei faʻamaʻi ma atiaʻe ai ni togafitiga ono.

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