Ngaahi Mahaki ʻOku ʻOngoʻi ʻi he Fakatupulaki ʻAonga ʻA e Protein ʻIkai Fakaʻonoponi ʻi he ʻUlo: Ko e Mahaki Pulu Vale mo e Ngaahi ʻAʻahi ki he Moʻui ʻo e Tangata
Ko e hā ʻa e Mahaki Deli Dana?
Ko e ngaahi mahaki ʻoku fakatupu ʻe he fakapikopiko ʻo e ngaahi protein ʻi he ʻatamai ʻe lava ke fakatupu ai ha ngaahi fakatamaki lahi ʻi he senitā sisitemi ʻo e neva. Ko e mahaki deli dana, ʻi he hingoa fakafaitoʻo "bovine spongiform encephalopathy", ʻoku lahi hono mamata ʻi he ngaahi pulu, ka ʻoku malava ke lahi ange hono aafi ʻi he meʻa ʻoku manatuʻi. Ko e ngaahi fekumi fakasaienisi ʻoku fakahaaʻi ai ʻoku fakahokohoko ʻa e mahaki Creutzfeldt-Jakob (CJD) ʻi he kakaí ʻoku fakafekauʻaki fakabailoojikali mo e mahaki deli dana. Ko ia ai, ʻoku lau ia ko ha mahaki ʻoku tokanga lahi ki ai ʻi māmani, pea ʻoku mahuʻinga ki he moʻui ʻo e fanga monumanu mo e moʻui fakalakalaka ʻo e kakaí.
Ngaahi Fakamatalá Fakalalahi fekauʻaki mo e Mahaki Deli Dana
Ko e bovine spongiform encephalopathy, ko ha mahaki neva ʻoku lahi hono fakaʻosi ʻaki ha mate ʻi he ngaahi pulu. Naʻe manatuʻi ʻi he kamataʻanga ko ha meʻa ʻoku fakangatangata pē ki he ngaahi monumanu, ka naʻe mahino ʻi he ngaahi taʻu kuo hili ʻoku malava ke fakafekauʻaki mo e mahaki Creutzfeldt-Jakob ʻi he kakaí (ko e vCJD ʻi he tuʻunga kehekehe). ʻOku fakahaaʻi ʻe he ngaahi fekumi fakasaienisi lahi ʻoku ʻi ai ha faingamālie ke mafola ʻi he kakaí ʻaki hono kai ʻa e ngaahi toʻonga monumanu mo e ngaahi toʻonga neva. ʻI he ngaahi konga mahuʻinga ʻo e mahaki ko e fakapikopiko ʻo e ngaahi protein ʻoku ui ko e prion ʻi he ʻatamai mo e ngaahi toʻonga kehe. ʻOku fakatupu ʻe he fakapikopiko ko ʻeni ha fakatamaki fakalakalaka ʻi he ngaahi selo neva, pea ʻi he kamataʻanga ʻe malava ke hoko ko ha liliu ʻi he ʻulungaanga pe vaivai ʻo e ngaahi hui, pea ʻi he hoko ki mui ange ʻe hoko ko ha mole manatu fakalakalaka mo e mole lahi ʻo e ngaahi malohi fakakaukau.
Ngaahi Meʻa ʻOku Fakatupu ai e Mahaki Deli Dana
ʻOku hoko ʻa e mahaki deli dana ʻi he liliu ʻo e ngaahi protein prion mei he tuʻunga ʻoku ʻikai fakatuʻutāmaki ki ha tuʻunga fakapikopiko mo e fakatuʻutāmaki. Ko e ngaahi protein ko ʻeni ʻoku nau malava ke fakalahi kinautolu mo fakapikopikoʻi e ngaahi protein totonu ʻi he sino. ʻIkai hange ko e ngaahi vailasi, ʻoku ʻikai ke ʻi ai ha koloa genetic ʻa e prion; ʻoku kamata ʻa e founga mahaki ʻaki ha liliu fakafakatuʻutāmaki ʻo e fao tolu ʻo e protein totonu. ʻI he taimi ʻoku aʻu ai e prion fakapikopiko ki he sisitemi moʻui mo e sisitemi neva, ʻoku nau fakapikopiko ʻi he ngaahi selo neva, tautefito ki he ʻatamai, pea fakatupu ai ha mole lahi ʻo e ngaahi ngāue totonu. Ko e ngaahi mahaki prion ʻoku lahi hono fakatamaki ʻi he ngaahi monumanu mo e kakaí, ka ʻoku siʻi hono hoko. ʻI he kakaí, ko e mahaki Creutzfeldt-Jakob ko e lahi taha ia ʻi he kulupu mahaki prion. Ko e meʻa mahuʻinga, ʻoku kehekehe ʻa e ngaahi mahaki prion mei he ngaahi mahaki vailasi pe baketelia, he ʻoku kamata ia mei he liliu hala ʻo e protein totonu ʻa e tangata.
Ngaahi Fakatātā ʻOku Lava ke Mamata ʻi he Mahaki Deli Dana
Ko e ngaahi mahaki ʻoku fakafekauʻaki mo e fakapikopiko ʻo e prion ʻi he ʻatamai ʻoku lahi hono hoko fakafiemālie mo fakaloloa. ʻOku kehekehe ʻa e ngaahi fakatātā ʻi he tuʻunga ʻo e mahaki mo e ngaahi koloa ʻo e prion.
Sporadic CJD: Ko e faingofua taha ke mamata ki ai. ʻOku vave hono lahi ʻa e ngaahi fakatātā pea ʻe lava ke fakatupu ha ngaahi palopalema lahi ʻi he senitā neva ʻi ha vahaʻa taimi nounou. ʻI he kamataʻanga, ʻe malava ke hoko ʻa e mole tauhi, faingataʻa ʻi he lea, mamahi, ongoʻi huhuʻa, liliu ʻi he ʻatamai mo e palopalema ʻi he sio.
Variant pe liliu CJD: ʻOku lahi hono mamata ʻi he ngaahi fakatātā fakafakakaukau. ʻOku malava ke hoko ʻa e loto mamahi, manavahē, fakamāvae mei he kakaí, palopalema mohe mo e fakavāhenga. ʻI he hoko ki mui ange, ʻe vave hono lahi ʻa e mole ngāue neva.
Familiale (Fakatuʻusila) CJD: ʻI he ngaahi tuʻunga genetic, ʻoku kamata ʻa e ngaahi fakatātā ʻi he taʻu siʻi ange pea ʻoku faingofua ange hono lahi.
Fakalakalaka, ʻoku hoko ʻa e mole tauhi sino, ngaahi hui ʻoku hūhū, faingataʻa ʻi he kai, palopalema ʻi he sio mo e lea, pea mo e mole manatu, mole tokanga, liliu ʻi he ʻatamai mo e fakavāhenga.
Fēfē ʻe Fakahoko ai ʻa e Diagnostic ʻo e Mahaki Deli Dana?
Ko e kakai ʻoku nau haʻu mo e ngaahi palopalema neva ʻoku sio ki ai ʻe he toketā neva. Ke ʻoua naʻa fakamalohi mo e ngaahi mahaki neva kehe (hange ko e Parkinson pe Alzheimer), ʻoku fiemaʻu ha sivi mo e vakai fakalahi. Ko e ngaahi founga tokoni ki he diagnostic ko ʻeni:
Magnetic resonance imaging (MR) ʻo e ʻatamai ke mamata ki he liliu ʻi he fao
Electroencephalography (EEG) ke vakai ki he ngāue ʻuhila ʻo e ʻatamai
ʻI he ngaahi tuʻunga fiemaʻu, toʻo ha biopsy mei he toʻonga ʻatamai ke sivi ʻi he fale fakatotolo
He ko e biopsy ʻo e ʻatamai ko ha founga invasive, ʻoku fakaʻaongaʻi pē ʻi he taimi ʻoku ʻikai lava ke maʻu ha ola mahino mei he ngaahi founga diagnostic kehe, pea ke fakapapauʻi ai ʻa e kehekehe ʻo e ngaahi mahaki.
Fakalele mo e Ngaahi Founga Tokangaʻi ʻo e Mahaki Deli Dana
ʻI he taimi ni, ʻoku ʻikai ke ʻi ai ha faitoʻo ʻe lava ke fakamaʻa mo fakafoki ʻaki ʻa e mahaki deli dana (BSE pe vCJD ʻi he kakaí). Ko e ngaahi founga tokangaʻi ʻoku fokotuʻu ki he fakavaivai ʻo e ngaahi fakatātā mo e fakalakalaka ʻo e moʻui lelei ʻo e mahaki. Ki he ngaahi fakatātā hange ko e loto mamahi mo e manavahē, ʻe lava ke tokoni ʻa e tokanga fakafakakaukau mo e faitoʻo ʻi he taimi fiemaʻu. Ki he mamahi ʻo e ngaahi hui mo e ngaahi palopalema sino kehe, ʻe lava ke fakaʻaongaʻi ʻa e ngaahi faitoʻo mamahi. ʻI he hoko ki mui ange ʻo e mahaki, ʻe fiemaʻu ke tokoni ki he tokanga fakataautaha, kai mo e moʻui lelei. ʻI he hoko ʻa e faingataʻa ʻi he kai, ʻe mahuʻinga ʻa e tokanga fakafaitoʻo fakapitoa mo e tokoni palaiativi. Ko e tokanga palaiativi ʻoku fokotuʻu ki he fakavaivai ʻo e ngaahi fakatātā, tokoni ki he feinga ke fehangahangai mo e mamahi, pea fakamuimuiʻi ʻa e moʻui lelei ʻo e mahaki ʻi he ngaahi taimi fakaʻosi.
Vakai Fakalakalaka ʻo e Māmani
Ko e mahaki deli dana mo e ngaahi mahaki prion ʻi he kakaí ʻoku siʻi hono hoko ʻi māmani, ka ʻoku tokanga lahi ki ai ʻi he moʻui fakalakalaka. ʻOku tokanga lahi ʻa e ngaahi fonua lahi ki he puleʻi ʻo e ngaahi koloa monumanu fakatuʻutāmaki mo e fakahoko ʻa e ngaahi founga maluʻi ki he moʻui fakalakalaka. ʻI he ngaahi tuʻunga ʻoku masiasi ai ki he mahaki prion, ʻoku mahuʻinga ke maʻu ʻa e tokoni mei he ngaahi kautaha moʻui fakapitoa ki he famili mo e kakaí.
Ngaahi Fehuʻi ʻOku Fai Fiemālie
1. Ko e hā ʻa e mahaki deli dana?
Ko e mahaki deli dana ko ha mahaki neva fakalakalaka mo e fakatuʻutāmaki ʻoku hoko ʻaki hono fakapikopiko ʻo e ngaahi protein prion ʻi he ʻatamai, pea ʻoku lahi hono mamata ʻi he ngaahi pulu. Ko e taha ʻo e ngaahi tuʻunga ʻi he kakaí ko e mahaki Creutzfeldt-Jakob.
2. Fēfē ʻe mahaki deli dana ʻe mafola ki he kakaí?
ʻOku manatuʻi ʻe lava ke mafola ʻaki hono kai ʻa e ngaahi koloa mei he monumanu mahaki ʻoku ʻi ai ʻa e ʻatamai pe toʻonga neva. Ka ʻoku siʻi ʻa e faingamālie ʻo e mafola ʻi he ngaahi fonua ʻoku tokanga lahi ki he puleʻi ʻo e meʻakai mo e ngaahi monumanu.
3. Ko e hā ʻa e ngaahi fakatātā ʻo e mahaki ko ʻeni?
ʻI he kamataʻanga, ʻe malava ke hoko ʻa e loto mamahi, liliu ʻi he ʻulungaanga, vaivai ʻo e ngaahi hui mo e palopalema tauhi. ʻI he hoko ki mui ange, ʻe hoko ʻa e mole tauhi, palopalema ʻi he sio mo e lea, mole manatu mo e mole lahi ʻo e ngaahi malohi fakakaukau.
4. Ko e mahaki Creutzfeldt-Jakob ʻoku tatau mo e mahaki deli dana?
Ko e variant (vCJD) ʻo e mahaki Creutzfeldt-Jakob ʻi he kakaí ʻoku manatuʻi ʻoku fakafekauʻaki mo e prion mei he mahaki deli dana, ka ʻoku kehekehe ʻa e CJD masani mo e BSE mei he ngaahi konga kehe.
5. ʻE lava ke faitoʻo ʻa e mahaki deli dana?
ʻI he taimi ni, ʻoku ʻikai ke ʻi ai ha faitoʻo ʻe lava ke fakafoki pe fakamaʻa ʻa e mahaki. ʻOku fokotuʻu ʻa e tokanga ki he fakavaivai ʻo e ngaahi fakatātā mo e fakalakalaka ʻo e moʻui lelei ʻo e mahaki.
6. ʻOku fakatuʻutāmaki ʻa e mahaki deli dana ki he kakai kotoa?
ʻOku siʻi ʻaupito ʻa e mahaki ko ʻeni pea ʻoku fakafekauʻaki lahi mo e kai ʻo e ngaahi koloa monumanu fakatuʻutāmaki. ʻOku siʻi ʻa e faingamālie ʻo e mafola ʻi he ngaahi fonua ʻoku tokanga lahi ki he maluʻi.
7. Ko e hā e ngaahi sivi ki he diagnostic?
ʻOku fai ʻa e vakai neva, MR, EEG mo e ngaahi sivi fakafotunga ʻo e ʻatamai. ʻI he ngaahi tuʻunga kehe, ʻe fiemaʻu ha biopsy ʻo e toʻonga ʻatamai ke fakapapauʻi ai.
8. ʻOku mafola ʻa e mahaki?
ʻOku ʻikai ke mafola faingofua ʻa e ngaahi mahaki prion mei he tangata ki he tangata. Ka ʻoku ʻi ai ha faingamālie ʻo e mafola ʻaki hono fetuʻutaki mo e ngaahi toʻonga fakapikopiko mo e ngaahi meʻafaitoʻo ʻoku fetuʻutaki mo e sisitemi neva.
9. Kapau ʻoku ʻi ai ha mahaki prion ʻi he famili, ʻoku lahi ʻa e faingamālie?
ʻI he ngaahi tuʻunga fakatuʻusila, ʻe malava ke lahi ʻa e faingamālie. ʻOku kamata vave ange pea lahi ange hono hoko ʻa e ngaahi mahaki prion genetic.
10. Fēfē ʻe tokangaʻi ai ʻa e mahaki?
ʻI he fakalakalaka ʻo e mahaki, ʻoku fiemaʻu ha tokanga fakataautaha, tokoni ki he kai mo e moʻui lelei. ʻOku fokotuʻu ke maʻu ha tokoni palaiativi mo e kau ngaue fakapitoa.
11. Ko e hā e lahi ʻo e BSE mo e CJD ʻi māmani?
Ko e ngaahi mahaki ko ʻeni ʻoku siofi lahi, ka ʻoku malava ke nau fakatupu ngaahi ola mamafa. ʻOku tokolahi e ngaahi fonua ʻoku nau vakai tuʻuloa ki he ngaahi koloa mei he fanga monumanu mo e maluʻi ʻakau.
12. ʻOku malava ke taʻe hoko ʻa e mahaki ʻakau vale?
Koeʻuhi ko e tokanga lahi ki he ngaahi koloa mei he fanga monumanu mo e meʻakai ʻa e fanga monumanu, pea mo e toʻo ʻa e ngaahi konga fakatuʻutamaki mei he meʻakai ʻa e tangata, kuo fakasiʻisiʻi lahi ai e faingamālie ke mafola.
13. ʻOku totonu ke fehuʻi ki he mahaki prion kapau ʻoku ʻi ai ha ʻuluʻi pe vaivai ʻa e ngaahi hui?
ʻIkai. ʻOku malava ke hoko ʻa e ngaahi fakaʻilonga ko ʻeni ʻi he ngaahi mahaki kehekehe. Kapau ʻoku vave hono hoko ʻa e ngaahi palopalema fakafakakaukau mo e mole fakakaukau, ʻoku mahuʻinga ke ʻalu ki he faitoʻo.
14. Ko e hā e meʻa ʻoku fiemaʻu ke fakapapauʻi ai e mahaki?
ʻI he tokolahi ʻo e taimi, ʻoku totonu ʻa e ngaahi fakaʻilonga fakafaitoʻo, ngaahi sivi fakatātā mo e ngaahi sivi falehoko; ka ʻi he ngaahi tuʻunga fehuʻi, ʻe lava ke fakakaukauʻi ha biopsi. ʻOku mahuʻinga ke vakaiʻi ʻe he faitoʻo poto.
15. Ko e hā e founga ke malu mei he mahaki?
ʻOku fautuʻu ke muimui ki he ngaahi fakahinohino ʻa e kau taki moʻui, fili e meʻakai malu, pea ʻaloʻi e ngaahi koloa mei he fanga monumanu ʻoku fehuʻi ai.
Ngaahi Konga Fakamālieʻi
Kautaha Moʻui ʻo Māmani (WHO): Ngaahi Mahaki Prion
Centres for Disease Control and Prevention (CDC): Bovine Spongiform Encephalopathy (BSE) & Variant Creutzfeldt-Jakob Disease (vCJD)
European Food Safety Authority (EFSA): BSE mo e ngaahi mahaki prion ʻi he fanga monumanu mo e kakai
Prusiner SB. Prions. Proceedings of the National Academy of Sciences. 1998
United States Food and Drug Administration (FDA): BSE & vCJD Information