Prion maʻi: mea, lalau mai, symptoms,ʻike, lapaʻau

Prion maʻi - mea he kūikawāʻano o ka loa neurodegenerative maʻi o nā kānaka a me nā holoholona. Ka mea, i wehewehe aku e holumua ai lolo poino, ma ka hapanui o na hihia hope me ka make imminent.

He aha nā prions?

Kēia kūikawā kumuʻiʻo 'ole. Ka mea hiki e nā maʻamau a me ka hapa o na nā'aʻaʻa o ola kānaka a me nā holoholona, e like me'āno'ē, ma o na la acee? Iuo oeiia o ka maʻi. A he mau makahiki aku nei, ka mea i manaoio aku i ka mea ola 'ole e komo i ka ai-kapaia nukelea nāʻakika - RNA a me ka DNA. Mahalo iā ia, ia mea hiki ke paʻi. Nā mea hoʻomaʻi, fungi, na manu, na holoholona "hoʻohui pū" nukelea nāʻakika. Mua ia i ea ae i ko lakou wa e kaawale aku i loko o nā'aʻaʻa mea hiki ole i ka piha paʻi. Prion nā polokina e loa huli i kēia mau manaʻo.

Mau nā lātoma i haku wale o ke kumuʻiʻo, akā, ka mea, ua kapa hoʻohanohano ma ka hiki, e paʻi. Penetrating i loko o ka meaola, ka mea, i ka loli he malaila i loko o ka maʻamau pathological prions, hiki mahuahua i ko lakou helu. Kēia kaʻina Pono oi manawa ma mua o ka'ōlelo o koʻohune a me nā mea hoʻomaʻi, no laila, mai ka manawa mai o ke komo ana i loko o ke kino o ka lātoma a hiki i ke kūpono 'ana o ka maʻi hiki ke lawe mau makahiki.

Nā waiwai o ka prions

Prions a prion maʻi i wehewehe aku e ka ia'ku kiʻekiʻe. Ka hapanui o ki ina hana like o ka disinfection i ineffective i ka Hana ana me ia. ka mea, i luku Prions e hoolapalapa, ke hoole aku i ke anu i ka -40 kekelē Kelekia. Ka mea, e ole hōʻike pā lonoaʻili i ka UV pāhawewe a me ka hoopaa i ko lakou mau waiwai i ka manaʻoe hana formalin.

Hiʻona o ka 'ole o ke kumuʻiʻo nā lātoma hiki aku ai i ka mea i na kanaka kino hiki ole kaua aku iā lākou. He mea e hiki ke ku e nā mea'aʻaʻa prions,ʻaʻole e hoomaau lākou mau lymphocytes i ole he i ka kaʻina hana. Kēia 'o ia hoʻi i ka anal o nā lātoma i loko o ke kanaka kino entails ka hanana o ka maʻi.

Prion maʻi: mōʻaukala o ka loaʻa

I ka 1982, Stenli Pruziner ho'ākāka 'ia ma mua prion maʻi, no ka i ka mea, i hope i hookoia ka Nobel uku.

Lōʻihi ma mua o ko lākou loaʻa, nä känaka 'epekema ma kā lākou hana hookolokolo aku la ia i ka helu o ke kanaka pathologies a me na holoholona, a i ka lōʻihi manawa hiki ole ke nānā'. Ma ka XVIII kenekulia i loko o ka United Kingdom ka mea, ua kakau, "scrapie" hipa. Animals loaa mai loa ka ohane ame, ka hele kāna hana a me ke kāʻili 'ana, i kuhikuhi ai i ka CNS. I ka 1957, Daniel Carleton Gajdusek hōʻike i ka maʻi i loko o ka muaʻohana, nona na kanaka i noho ai i loko o ka Hawaii o Papua New Guinea. Ke pathology Ua pili me ka cannibalism, a hala ia mai kekahi kanaka i kekahi.

Mai ka makahiki 1986, ma England, a me ka hope o na mea he nui nā'āina, ua kākau epekema he mau outbreaks i hope haawiia mai i ka inoa o "hehena bipi maʻi". Ua puleʻAmelika, e loli ai i nā holoholona. "Pupule bipi wahine maʻi" ma hope o ka pōkole wā o ka manawa ua i laha huliāmahi, a ua lilo i ke kumu no kona hanana prions. I ka 90 makahiki poe akamai i ua ai EYI o keia maʻi, e ke kanaka a me ka waiū a me ka mōhai holoholona.

Currently, he piha study me undiagnosed kumu o ka maʻi i ka pilina i ka mea i kńnaka i i ka helu o nā noi no ka hooulu ana o ka prion ano. Iwaena o lakou mea o ka pathology o Creutzfeldt-Jakob maʻi, Alzheimer ka maʻi. Symptoms a me na hoailona o kēia mau illnesses i ka hailonaʻana i loko, he pono ole. I loko nō o ka papakaukau E hana 'ia i loko o ka hoʻopaʻa' ana i kēia mau kāna hana, nui i koe ma kela aoao o comprehensible.

Pehea oe i loaaʻi ka iho mailoko o?

Ma ke kālā no lapaau, eia ekolu aoao o ka maʻiʻaʻai.

1. Transmissible. i hoolilo Prions mai kekahi mammalian? ueo i kekahi. Hōʻike muaʻia aʻe i ke ola ana o ka ai-kapa? Ueo'ākeʻakeʻa mai. Kēia 'o ia hoʻi i ka hoolilo mai i ka bipi mea,ʻaʻole e hiki i ka ke kanaka. ʻepekema hōʻoleʻia kāna i kēia nānaina. Kumuʻiʻo nā lātoma hiki ke pū i hiki mai i iho mailoko o ka holoholona a me kanaka. Kumu o ka prion mai i ke kumu ma ka 'ai, inu o kaʻai / iho mailoko holoholona waiū, i ka hana o ka liluwelo'aʻaʻa (corneal transplantation, koko huahana, a no laila, ma luna o. D.). Ke Kula Kaiapuni 'biomaterials i kekahi mau degere o ka pathogenicity. Ka nui infectivity hōʻike lolo'aʻaʻa, ka mea e hiki mai ana ka anu u ku koko a me ke koko me nā huahana.
2. Hereditary. I ka maʻi e hoʻomōhala ana i loko o ke alo o aaiaoe mutations, ka pukaʻana i loko o ka 20th chromosome. He kēia wahi mea kuleana no ke alo o ka mea maʻamau prion kumuʻiʻo. Kona hana ua nō poorly ike. Ma ka hihia o ka'ōewe mutations kahi o olakino prion pathological synthesized, a inevitably hele aku ai i ka ulu ana o ka maʻi.
3. Sporadic (noho'na makaainana i ulu hanana o'āno'ē kumuʻiʻo).

Penei, prion maʻi hiki e nā hereditary a me ka infectious ano. Nānā 'ole o ke ano o ke anal o'āno'ē kumuʻiʻo i loko o ke kino, ka mea hiki i ka maʻiʻaʻai ana i na mea e ae.

He aha ke kumu o ka prions i loko o ke kino?

'āno'ē nā polokina wehewehe aku ma kā lākou mau hana, e i spongiform encephalopathies, oa CNS. Mai ka morphological wahi o ke hoomaopopo, i kēia 'o ia hoʻi ke kahua o cavities i loko o ka lolo keena, neuronal make, overgrowth ma ko lakou wahi, a pau connective'aʻaʻa atrophy o ka lolo. E kū'ē i ke kāʻei kua o ke kahua o nā huhui waina a lākou o ka prion amyloid plaques malama. A pau kēia mau keʻano o ka hanaʻana i hoʻomaka me ahuwale na hoailona o ka li nui.

He aha maʻi i prion?

I ka lā, nä känaka 'epekema ke pololei inoa i kekahi mau maʻi, a nō ka mea, o ka'āno'ē kumuʻiʻo hale:

• Creutzfeldt-Jakob maʻi;
• kuru maʻi;
• Alpers (a naele ia e holumua ai encephalopathy) maʻi;
• onaʻohana Kanikani'āʻula;
• maʻi Gerstmann-Shtreusslera-Scheinker.

Next, nānā i kēlā me kēia i loko o oi au mamuli o ka pathology.

Creutzfeldt-Jakob maʻi

Creutzfeldt-Jakob maʻi ua wehewehe aku ma kona 'ano, no laila poe akamai i razdeleli ia i loko o kekahi mau' ano apau loa.

• sporadic;
• ohana;
• iatrogenic;
• hou atypical palapala.

Sporadic maʻiʻano mua noʻonoʻo i ka loa prevalent. Kona mua symptoms hou ma hope o ka makahiki o 55 makahiki. Eia naʻe, ma ka hope mau makahiki 'ikepili helu i loli. Ma hope iho o ka helehelena o ka 'ike ma luna o ke ahulau o "hehena bipi maʻi" e increasingly hoomaka ae la e palapala hihia o atypical ano aie i na holoholona maʻiʻaʻai. No kēiaʻano o ke ano o ka mua kona helehelena. Ma ka hapanui o hoopii, ui kanaka e ae. Symptoms i maheleia i mau conditional hui: neurological a me ka psychiatric. Initially, mailoko o na hiohiona headache, hiamoe haunaele, ua hoʻemi ikaika loa ka makemake. Ua, mau symptoms hou iaiyoe hoʻopilikia, poho o ka hihiʻo. Ka noʻonoʻo 'kāna hana i hoikeia mai ma ka palapala o ka hallucinations a me delusions. I ka maʻi ua wehewehe aku ma ka kokololio kūpono 'ana i loko o ka hope ke kahua ua wehewehe aku ma ka piha immobility o ke kino. A kanaka haalele i ka mana hoʻomalu ma luna o ka hana o ka pelvic nā loko. Me ia i kaʻike, kanaka noho i oi aku i na makahiki elua.

I ka helehelena o kaʻohana palapala ua kena ae la ia e mutations ma ka'ōewe pae i loko o ka ciia o ka 20th chromosome. I ka maʻi ua wehewehe aku ma ka autosomal Hoʻohaku külana. Ka mua hoailona ana i loko o kahi o 5 makahiki mua aku ma mua i loko o sporadic palapala.

Iatrogenic palapala e hoʻomōhala ana i like me ka hopena o ke kanaka maʻiʻaʻai iʻoki kino. Ana helu 'ike no keia koho mea,ʻaʻole he maʻi, no ka mea, he pa e hooiaio i ka pathogenesis o prion maʻi. Ke incubation au papa koa mai ka 7 mahina a hiki i 12 makahiki. Ua Ua nānā 'ia e kekahi hui malu pu ana o kekahi mau aʻe: i ke kaʻina hana o ka anal o'āno'ē nā polokina i loko o ke kino, i ko lakou helu, ka mua genotype kanaka. Ke koho wikiwiki loa e ulu ana maʻi e kauoha anal o prions ma ka lolo'aʻaʻa like me ka hopena o keʻoki kino. More manawa ua koi 'ia i maʻiʻaʻai ma luna o ke kāʻei kua o corneal transplantation paha o dura mater. E hoomanawanui i ke kiʻekiʻe o ka ulu 'cerebellar ataxia, hoʻopōʻino lehelehe a me ka Muscle leo, dementia.

"Pupule bipi wahine maʻi" hoomaka ae la e kuai pilina ma hope o ke ahulau i loko o nā holoholona i loko o nā 90s. Prion maʻi, na symptoms o ka iʻikeʻia ma waena o nā makahiki o ka 30 a hiki i kahi o 40 makahiki, mea make i kānaka. E like me ka iatrogenic palapala, neurological symptoms predominate no mentally.

Onaʻohana Kanikani'āʻula.

Kēia maʻi o ka autosomal Hoʻohaku ano, a ua ho'īli wale ma succession. Ona Kanikani'āʻula mea pohihihi keia. Ua ua ikeia ma ka 'epekema mai ka makahiki 1986. Kona mua symptoms ma waena o nā makahiki o nā makahiki 25 a hiki i kahi o 71 makahiki.

Epidemiology o prion maʻi o kēia 'ano e insufficiently kaupaona i nā. I ka papa kuhikuhiE symptom o ka onaʻohana Kanikani'āʻula mea sleeplessness. Ke kino i nā haalele i kona hiki, e hoopau ai i ke hoʻomaʻa i ka pae ana o wakefulness a me ka hiamoe. No hoi, e hoomanawanui i ikeia ka hele kāna hana a me ka Muscle nawaliwali. A ua noho hihia o autonomic kāna hana i hoike mai ai ia māhuahua ke koko, excessive sweating. O ka noʻonoʻo 'kāna hana hiki e kaulana panic ho'āhewa wale, pilikia makapō hallucinations, a me intermittent Episode o ka hilahila. No ka mea, Kanikani'āʻula ikaika exhaustion i, i ka hoʻomanawanui make.

Kuru

Infectious prions palapala i aʻo i loko o au mamuli no o ka mea maʻi, nui ua hōʻike hewa, heʻohana o cannibals. A hiki i ka makahiki 1956 i waena o nā kupa o Papua - New Guinea i haawi ai-kapa mooolelo oʻano hana cannibalism - ai ana i ka lolo o kekahi mea i make kanaka. Ua Ua manaoio aku i kekahi o na lala o keia ohana ku ae maʻiʻaʻai, a ma hope, e halii mai i nā poʻe ma hope o kaʻano hana. Mai ka ke kāpaeʻana o keia moolelo o ka maʻi hihia i ua hoʻopaʻa 'ia ma kekahi mau manawa i emi pinepine i keia la keia maʻi kokoke' aʻole i kānāwai.

Ke incubation au papa koa mai ka 5 a hiki i 30 makahiki. Ia mea ke kumu o ka Kuru maʻi haawiia keia i like waeʻano "lohi mea hoʻomaʻi maʻiʻaʻai". Maʻi cerebellar kāna hana hoikea mai oia, me ka uncontrollable akaaka, moni 'ia dysfunction a me ka Muscle nawaliwali. Ma nā kahua o ka hoʻonaʻauao maʻi o ka ulu dementia. People me kēiaʻike, e ola i hou aku 30 mahina.

Alpers maʻi

Ka maʻi ka hapanui ia lŘlŘ i kahi mau keiki liilii (ma lalo o makahiki 18). Maʻi ua pū i hiki i ka autosomal recessive ano, ma ka hihia o ka coincidence o elua pathogenic nā'ōewe poe kupuna, a me ka makuwahine. Mawaena o ka papa kuhikuhiE symptoms hiki ke hōʻike 'blurred hihiʻo a me ke kāʻili' ana. I ka 'inikua pili puke ma laila nō nā wehewehena o ka huʻi maʻi' ia ma ka hahau type. Alpers maʻi ua i wehewehe aku e lesions o ke akepaa, a koke e hoʻomōhala ana i loko o maʻi hepatitis a pau me ka cirrhosis. Nā mea maʻi i make, no ka intoxication no 12 mahina ma hope oʻike, o na symptoms mua.

Gerstmann maʻi pale-Shtreusslera-Scheinker

Kēia AOAIAaOO Ua hoalii mai ia me hereditary maʻi type. Loa pohihihi (kekahi hihia e pili ana i 10 miliona kanaka). Ka mua hoailona i ana ike ma nā mea maʻi no 40 makahiki. Ka hooulu ana o ka maʻi pale hoʻomaka me cerebellar kāna hana. Initially pōniuniu ikeia. E like me ka maʻi progresses koordinatornye aʻe 'ana i nā kuokoa neʻeʻana i ole i ke Akua. A me ka helu 'ia ma symptoms ikea kāna hana o ka Muscle leo, he emi ma ka hihio, a me ka lohe, pilikia me ka moni' ia a me ka paʻi. Ma hope-ke kahua paa kauka hoike ana mai o ka dementia. Ke ola expectancy o nā mea maʻi i kēiaʻike, i ke 10 makahiki.

Alzheimer ka maʻi , a Parkinson ka maʻi

Alzheimer ka maʻi, a Parkinson ka maʻi, symptoms a me na hoailona a i ka pono maoli, ka ulu 'ana ma ka like ala e prion maʻi puʻuwai. Nā lātoma o Hoʻokolohua amyloid, Tau kumuʻiʻo a me nā hale i lilo hoʻokomo kālā o pathogenic ano ma ka lolo'aʻaʻa. Eia naʻe, e hopu i kēia mau illnesses ole i ke Akua. Kēia 'o ia hoʻi i ka amyloid fibrils i hanaʻia ma ka pōʻino, ua make kumuʻiʻo nā lātoma, akā, "ola" hana "maʻi" Aole i pili.

More nei, ua mālama 'epekema i ka moʻo o ka haʻawina i loko oʻiole, a hoole i kēia mea mahuʻi. Ma hope iho o ka hoike ana o pathogenic nā polokina ma o ka lolo ka loa ola holoholona ka mea, he ano amyloid plaques. Kēia 'o ia hoʻi i ka pathogen kumuʻiʻo hiki nō iho mailoko o ke ola i ke' ole. Ke Kaka no i hoʻomalu mai i ke Kulanui o Texas. I ka wā e hiki mai kokoke, e hele mai i kekahi hana o nā kānakaʻepekema, mai London, i hoike ia Alzheimer ka maʻi, symptoms a me na hoailona o ka maʻi hiki loa ke hele mai kekahi kanaka i kekahi.

Keia iloko o ua Parkinson ka maʻi ua wehewehe aku e holumua ai lilo o neurons e paka dopamine, he neurotransmitter. No ka mea, o keia, ke kanaka i meaʻehaʻeha hooponopono ana o ka neʻe 'ana a me ka muscular tonus, a ua hoikeia mai ma ka tremor, nohona ma oolea. Parkinsonism poino mai kela kanaka haneri ka poe i huli kanaono wale aku no. Ka maʻi hoʻomaka kona ulu me na mea noi, i mea oi loa aku hoi kekahi 'wā i kekahi kanaka e ukuhi i ka ma' ole ai i ka meaʻai. A laila, wawahi ai i ka olelo, moni 'ia reflexes. Eia naʻe, no ka lapaʻau i ole waiho aku i ka hoʻokō lapaʻau no ka poʻe me kaʻike, o "Parkinson ka maʻi". Na hoailona a me symptoms o keia maʻi hiki ke mitigated e symptomatic Inc. Naʻe, ka hapanui o kēia mau ai 'kumu i ka helu o kaʻaoʻao loa.

Alzheimer ka maʻi - he maʻi wehewehe aku ma ka a maloo aku o neurons, ma o na la e hoomanawanui i hoʻomōhala dementia. Ka mua symptoms o keia maʻi hiki ke hana i kakahiaka nui me na makahiki 40. Ua Ua diagnosed i loko o ka hapanui o na hihia poorly hoʻonaʻauao kanaka. A kanaka me ka pae kiʻekiʻe o ke akamai i ka manawa kūpono i loaʻa, e Makuakane me na symptoms o Alzheimer ka no o ka mea nui pili 'o Randy ma waena o neurons.

Ka maʻi hoʻomaka kona ulu me ka iaiyoe kāna hana. Ke iniiaiie ke kahua ana hele unnoticed i ka 'ē aʻe. Ka loiloi mua symptoms i pinepine ana, e uhi mai ai hewa ia ma ka stress a me ka excessive workload i hana. E like me ka Holomua o ka lapaʻau kiʻi o ka maʻi mutates. Ke hoomanawanui ua hou orient lakou ia lakou iho ma ka makahiki, mai kona hoʻomanaʻo kulu mua loaʻa nā mākau o ke kākau 'ana, ka heluhelu. Mua, hoʻopoina i ka ia hanana ma ka manawa. I ka wā o ka pathology hoʻomaka i ka holomua, ka mea, he pono ke hana i ka wa pono a pau, e malama i hiki i kekahi kanaka ka i ka pakiko-hana, e ho'āʻo i ka pale aku i ka hanana o ke kaumaha. Ke kākoʻo pāʻoihana o keia pilikia ke kōkua hou ikaika kōkua hoʻomalu hālāwai hoʻolohe 'ole i ka pono i hoʻokū pono makaaniani. I Kekahi Mau lapaʻau o Alzheimer ka maʻi pale hakuʻole. I ka wā o ka iniiaiie symptoms ia mea nui, e kiʻi i piha ka ninaninau ma ka neurologist. no ka iapaau ana o ka poe akamai i pele waiho aku ai 'no ka mea maʻi, e kōkua a me ke koi aku kona ulu ana.

ʻike, o ka prion mai

I Kekahi Mau PalekanaEND_LINK ana i ole a ianoiyuaa a poe. No ka laʻana, like me EEG i loaʻaa me ia ma Creutzfeldt-Jakob maʻi, eia ma nā lolo pathologies. Ua wehewehe aku ma ka haahaa MRI PalekanaEND_LINK manaʻo nui, mai 80% o ka hoʻomanawanui iki mai hoike ole-kekahi mau lepa a. Naʻe, i kēia study e leie aku mākou e hōʻike lolo atrophy. Kona oolea ua ano hoomainoino like me ka holomua o kanaka prion maʻi.

Ka palena pauʻike, ua lawe mai ia me nā mea a pau i ka pathologies, kekahi hoike ana o ka i mea dementia (Alzheimer ka maʻi, vasculitis, neurosyphilis, herpes encephalitis a me na mea e ae).

lapaʻau hoʻokokoke mai

Eia naʻe, i kēia manawa, a pau prion mai i maʻi. Hoʻomanawanui iki mai ia symptomatic Inc me anticonvulsants, a ho'ēmi i kā hoomanawanui wale. Ke kēla loaa ole ia. A pau ike prion mai i ona i kanaka ano.

Currently,ʻepekema, mai a puni ke ao nei e 'Imi huli i ke ao lāʻau. Mālama 'ia ka hoʻohana' ana noiʻi holoholona. Ua Ua ea ae ka hana i loko o ke kaua e kū'ē i illnesses ia anei i aeee e e hoʻohana hope, e like me ka loa, he pono ole ka hū. Hoʻokolohua ai 'Ke mai i ke kiʻekiʻe ka hoʻokōʻana, no laila, i ko lakou manao ua noʻonoʻo' ole.

kāohi ana

Mai ka hooulu ana o sporadic, a ili Lolina o prion mai, e hoomalu ia lakou ihoʻaneʻane hiki ole i. maʻi kekahi hiki ke eliminated e hele i ka hana aaiaoe ninaninau. Eia naʻe, ia mea ma ko makou aina, he mea paʻakikī, no ka mea, ke keʻena hoʻokolohua lawelawe i kēiaʻano o kaʻike,, e pili wale aku no.

Ma ka hihia o hereditary maʻi mua hāpai keiki i ka manao e kukakuka me kekahi kauka-geneticist. Kēia e kōkua pale wā e hiki mai pilikia a me ke ola o ke keiki.

I mea e hoʻopale ai ia lakou iho, mai Creutzfeldt-Jakob maʻi, ka mea, ua? Aeiiaiaoaony e haawi mai ai ana i ka ai, mai ka aina kahi hihia o nā holoholona maʻi kākau. Mua o nā mea a pau a mākou e kamaʻilio e pili ana iʻEulopa'āina. Oe E hoi mai e ia i loko o ka lapaau ana o na hoomakaukau i mai i ke koko o nā holoholona a me kānaka. Ua mea maikaʻi, e puku ia me ka kemikala analogues.

Prion maʻi - ua i lawa? Anoee ano o infectious a me ka hereditary lesions e ana i loko o ke kanaka kino ma ke kāʻei kua o ka anal o'āno'ē nā polokina. Ma ka hapanui o na hihia, ka mea, he aha ka mea i ka Central ko mākou hopohopo nenoaiu. Ka lapaʻau kiʻi Ua wehewehe aku e like symptoms. Initially, he kanaka no ke kahe ikaika loa ka makemake a me ka hihiʻo, nee ōhumu i loko o ka lewa. Ma ke kahua hope loa o ka ulu dementia ka wā o ka hoʻomanawanui mea e hiki ke kū kaʻawale lawe malama ana noʻu iho. I ka hopena o kekahi kanaka i loohia mai mea mau i ka ia - ka make. Currently, kauka i ole aha lunakānāwai e ku e na pathologies o keia ano.