Matenda Prion: zimayambitsa, zizindikiro, matenda, mankhwala

matenda Prion - ndi mtundu wapadera wa matenda aakulu neurodegenerative ya anthu ndi zinyama. Iwo yodziwika ndi kuwonongeka mwapang'onopang'ono ubongo, nthawi zambiri kuthetsa imfa pafupi.

Kodi prions?

Izi wapadera mapuloteni dongosolo. Iwo akhoza kukhala kwachibadwa ndiponso gawo la zimakhala anthu athanzi ndi nyama, komanso nthenda, kuchititsa zosiyanasiyana matenda. Mpaka zaka zingapo zapitazo, kuti ankakhulupirira kuti kapangidwe amoyo ayenera kukhala ndi otchedwa zidulo mkati - RNA ndi DNA. Chifukwa cha iwo, n'zotheka kuti zibalane. Mavairasi, bowa, mbalame, nyama "amapanga" zidulo mkati. Poyamba anali ankaganiza kuti ajombera mu zimakhala ndi kosatheka kuti athe kubereka. Prion Mapuloteni zinasinthiratu maganizo.

mamolekyulu awa ankalemba yekha zomanga thupi, koma kusiyana ndi luso la kubereka. Ozama mu chamoyo, iwo chifukwa kusintha ali m'bukuli mu prions yachibadwa pathological, potero kuwonjezera chiwerengero chawo. Zimenezi zimafuna nthawi kuposa kubalana mabakiteriya kapena mavairasi choncho kuchokera pamene adalowa thupi la molekyulu kuti chitukuko cha matenda akhoza kutenga zaka zingapo.

The katundu wa prions

Prions ndi matenda prion yodziwika ndi kukana mkulu. njira ambiri disinfection sizithandiza mu nawo. Prions sizidzawonongedwa ndi otentha, chingathe kupirira kuzizira mpaka madigiri -40 Celsius. Iwo satchula kudziwa cheza UV ndi kusunga katundu wawo pa processing formalin.

Features ya kapangidwe ka zedi kumabweretsa mfundo yakuti thupi sangathe kumenyana nawo. Iye sali angathe kubala akupha prions, osati pomenyana lymphocytes awo sanali kuoneka muzindikire. Izi zikutanthauza kuti malowedwe a mamolekyulu mu thupi kumatanthauza pakutchulidwa matenda.

matenda Prion: mbiri ya anapeza

Mu 1982, Stenli Pruziner woyamba kufotokoza matenda prion, amene anali pambuyo kupereka ya Nobel Prize.

Kale anatulukira, asayansi ntchito zawo anafufuza angapo pathologies anthu ndi nyama, zomwe zimachititsa nthawi yaitali sanathe zotsimikizika. Mu XVIII atumwi ku United Ufumu wakhala anawawerenga, "scrapie" nkhosa. Nyama akudwala kuyabwa kwambiri, matenda kuyenda ndi khunyu, amene ananena kwa CNS. Mu 1957, Daniel Carleton Gajdusek anafotokoza matenda mu fuko asanakhazikike, amene anthu ankakhala kumapiri a Papua New Guinea. The kudwala anali kugwirizana ndi kudya ndi kudutsa kuchokera amapatsirana.

Kuyambira mu 1986, ku England, ndipo kenako m'mayiko ena ambiri, asayansi analemba kubuka ochepa patapita anapatsidwa dzina la "misala ng'ombe matenda". Ndi ambiri amakhudza ng'ombe. "Misala ng'ombe matenda" patapita nthawi yochepa wafika mliri kufanana ndi chifukwa pakuwonekera kwake akhala prions. Mu zaka 90 Akatswiri atsimikizira kufala kwa matenda kwa munthu pamodzi ndi mkaka ndi nyama zoweta.

Panopa kuphunzira zambiri zimayambitsa undiagnosed matenda zathandizira chakuti asayansi anapanga chiwerengero cha maganizo pa chitukuko cha prion chikhalidwe. Pakati pawo ndi kudwala matenda Creutzfeldt-Jakob, matenda a Alzheimer. Zizindikiro ndi zizindikiro za matenda amenewa ndi zambiri zofanana. Ngakhale patsogolo yaikulu mu phunziro mwa matenda amenewa, zambiri amakhalabe kupitirira chomvetsetseka.

Kodi inu kachilomboko?

Mu mankhwala amakono, pali njira zitatu matenda.

1. Transmissible. Prions ali inasamutsidwa kuchoka ku mtundu wa zolengedwa zoyamwitsa ndi mzake. Poyamba otchulidwa kuli otchedwa mitundu chotchinga. Izi zikutanthauza kuti kusintha kwa ng'ombe si zotheka kuti munthu. Asayansi kutsutsa mfundo imeneyi. zedi akhoza kuchoka nyama yowola kapena anthu. Zimayambitsa matenda prion chifukwa cha ingestion nyama / kachilombo nyama mkaka, ntchito minofu kwachilengedwenso (corneal kupatsidwa zina, mankhwala magazi, ndi zina zotero. D.). biomaterials zosiyanasiyana madigiri osiyanasiyana pathogenicity. Wamkulu infectivity amasonyeza ubongo minofu, sitepe yotsatira wotanganidwa magazi ndi mankhwala.
2. Kumtundu. Matendawa akufotokozera pamaso pa masinthidwe chibadwa, akutulukira mu khromozomu 20. Ndi malowa ali ndi udindo pamaso pa yachibadwa prion mapuloteni. kugwira ntchito akadali bwino kudziwika. Pankhani ya masinthidwe jini m'malo wathanzi prion pathological apanga, kumene kumakhala kumabweretsa chitukuko cha matenda.
3. Ayi (mowiriza yatsoka mapuloteni nthenda).

Choncho, matenda prion zimakhala khalidwe cholowa ndiponso matenda. Kaya njira malowedwe a nthenda mapuloteni mu thupi, zingayambitse matenda ena.

Chimene chimayambitsa prions mu thupi?

mapulotini nthenda yodziwika ndi luso lawo chifukwa spongiform encephalopathies, mwachitsanzo CNS. Kuyambira mfundo morphological maganizo, izi zikutanthauza mapangidwe cavities maselo a ubongo, imfa okhudza mitsempha, overgrowth mu malo awo ndi kutha connective minofu ya manja a ubongo. Against maziko a mapangidwe masango mipukutuyi prion amyloid anati. Zonsezi zimachititsa saonetsa zizindikiro zoonekeratu kutupa.

matenda Kodi prion?

Pakali pano, asayansi molondola mayina matenda ochepa, amene chifukwa cha nyumba nthenda mapuloteni:

• Creutzfeldt-Jakob matenda;
• kuru matenda;
• Alpers (chofewa ngati siponji mwapang'onopang'ono encephalopathy) matenda;
• koopsya banja tulo;
• matenda Gerstmann-Shtreusslera-Scheinker.

Chotsatira, kuyang'ana pa chilichonse mwatsatanetsatane kudwala lapansi.

matenda Creutzfeldt-Jakob

matenda Creutzfeldt-Jakob amakhala ndi kuchuluka kwake, kotero akatswiri razdeleli mu njira zingapo:

• ayi;
• banja;
• iatrogenic;
• latsopano atypical mawonekedwe.

Ayi matenda chimake musanayambe zochitikazi anthu ambiri kwambiri. zizindikiro adzawonekera pambuyo zaka 55. Komabe, pa zaka zochepa ziwerengero zasintha. Pambuyo kuoneka zokhudza mliri wa "nthenda ya misala ng'ombe" ambiri anayamba kulemba milandu mitundu atypical chifukwa matenda ng'ombe. Kwa mtundu uwu wa khalidwe ndi maonekedwe kale. Nthawi zambiri achinyamata akuvutika. Zizindikiro anawagawa m'magulu awiri makonzedwe: ubongo ndi maganizo. Poyamba, matenda, n'kuchiumba mutu, tulo chisokonezo, utachepa kudya. Pang'onopang'ono, zizindikiro zimenezi anawonjezera kukumbukira kuwonongeka, imfa ya masomphenya. mavuto a m'maganizo ndi anawonetseredwa mu mawonekedwe a kuyerekezera zinthu m'maganizo ndi zinyengo. Matendawa ali ndi makhalidwe oipa kukula mofulumira mu gawo lotsiriza amakhala ndi kulephera kuyenda wathunthu wa thupi. Munthu kuso- koneza ntchito ziwalo m'chiuno. Ndi matenda amenewa anthu moyo osaposa zaka ziwiri.

Maonekedwe a mtundu banja chifukwa cha masinthidwe mlingo jini mu zone ya khromozomu 20. Matendawa ali ndi makhalidwe makhalidwe autosomal lalikulu. Zizindikiro zoyambirira zimachitika za 5 Zaka kale kuposa mu mawonekedwe ayi.

mawonekedwe Iatrogenic akufotokozera chifukwa cha matenda omwe pa opaleshoni. mudziwe Statistical kwa njirayi si matenda, kuyambira n'zovuta kutsimikizira pathogenesis matenda prion. Nthawi makulitsidwe ranges kuchokera miyezi 7 ndi zaka 12. Amaonekera ndi osakaniza zifukwa zingapo: ndondomeko malowedwe a mapuloteni nthenda mu thupi, chiwerengero chawo, choyambirira genotype munthu. The yachangu kukula matenda ndi malowedwe mwachindunji prions mu ubongo minofu chifukwa cha opaleshoni. More nthawi chofunika pa matenda pa maziko a kupatsidwa zina corneal kapena Mater Dura. Odwala pang'onopang'ono osauka cerebellar ataxia, kulankhula mkhutu ndi minofu kamvekedwe, maganizo.

"Misala ng'ombe matenda" adayamba kupeza kugwirizana pambuyo mliri ng'ombe mu 90s lapansi. Prion matenda, zizindikiro a wosawoneka zaka zapakati pa 30 ndi zaka pafupifupi 40, ndi kumapha anthu. Monga mtundu iatrogenic, zizindikiro ubongo amachuluka pa maganizo.

Dzitetezeni banja tulo

matenda ndi autosomal lalikulu mtima, ndipo zimenezi imafalikira yekha ndi motsatizana. Dzitetezeni tulo ali osowa. Iye amadziwika sayansi kuyambira mu 1986. zizindikiro zake woyamba zaka zapakati pa zaka 25 za 71 chaka.

Epidemiology matenda prion wa mtundu uwu ndi insufficiently kufufuza. The chizindikiro chachikulu cha amapha banja kugona ndi sleeplessness. thupi pang'onopang'ono wotaya umatha kusintha kwathunthu gawo la maso ndi tulo. Komanso, odwala amaoneka matenda kuyenda ndi kufooka minofu. Koma nthawi zina matenda autonomic zimene zimasonyeza kuthamanga magazi kwambiri thukuta. Matenda a m'maganizo Tingaone amantha, kuyerekezera zinthu m'maganizo zithunzi ndi nkhani likukulira chisokonezo. Chifukwa tulo zonse kutopa zikachitika, wodwala afa.

Kuru

mawonekedwe prions opatsirana anali kuphunzira mwatsatanetsatane chifukwa cha matenda, Komanso mwapadera, fuko la odya anzawo. Mpaka 1956 pakati pa anthu a Papua - New Guinea anagawanika otchedwa mwambo wa kudya mwambo - kudya ubongo wa munthu wakufa. Akukhulupirira kuti mmodzi wa mamembala a fuko limeneli adanyamuka matenda amene kenaka linafalikira anthu ena zokometsera. Popeza Kulipira mwambo uwu milandu matenda analemba kangapo kamodzikamodzi lero matenda pafupifupi zimachokeradi.

Nthawi makulitsidwe ranges kuchokera zaka 5 mpaka 30. N'chifukwa chake matenda Kuru amatchedwa "wosakwiya HIV matenda" gulu. Matenda matenda cerebellar akuwonetseredwa ndi osamvera kuseka, Pomeza kukanika ndi minofu kufooka. Mu masiteji osachiritsika kukhala maganizo. Anthu ndi matenda izi moyo zosaposa miyezi 30.

matenda Alpers

matenda makamaka kumachitika ana (pa zaka 18). Matenda imafalikira mu autosomal unsembe recessive, mu nkhani ya mwangozi awiri tizilombo majini makolo. Mwa zizindikiro zazikulu tingaone kusawona ndi khunyu. Mu mabuku mankhwala Buku pali kufotokozera matenda pachimake zikuchitika mwa mtundu sitiroko. matenda Alpers amadziwikanso yodziwika ndi zotupa a chiwindi, amene mwamsanga akufotokozera mu chiwindi aakulu, kutsiriza ndi matenda enaake. Odwala akufa chifukwa cha kuledzera kwa miyezi 12 kuchokera pamene matenda zizindikiro woyamba.

Gerstmann syndrome-Shtreusslera-Scheinker

Izi njira ndi n'chinthu chofunikira mtundu matenda otengera kwa makolo. chosowa kwambiri (Mwachitsanzo pafupifupi mamiliyoni 10 anthu). Zizindikiro yoyamba amaugwiritsa odwala zaka 40. Kukula kwa matendawa amayamba ndi matenda cerebellar. Poyamba chizungulire akuwonekera. Monga matenda ili m'kati kuyan'anila koordinatornye pang'onopang'ono kayendedwe palokha kumakhala kovuta. Pamodzi ndi zizindikiro ali kuoneka matenda a minofu kamvekedwe, kuchepa masomphenya ndi kumva, mavuto ndi kumeza ndi kuberekana. Pomaliza gawo atathana madokotala maonekedwe a maganizo. The moyo amayembekezeka odwala ichi matenda kwa zaka 10.

matenda a Alzheimer ndi matenda Parkinson

matenda a Alzheimer ndi matenda Parkinson, zizindikiro ndi zizindikiro zimene ndi chikhalidwe wamba, kukhala mu njira zofanana prion matenda. Mamolekyulu wa beta amyloid, matchalitchi anayamba mapuloteni ndi nyumba zina kupanga miyala ya tizilombo chikhalidwe mu ubongo minofu. Komabe, kuti akamkole matenda awa zosatheka. Izi zikutanthauza kuti fibrils amyloid anapanga ndi tinthu zowonongeka mapuloteni, koma "wathanzi" kanthu "kudwala" sizikhudza.

Posachedwapa, asayansi anachita mndandanda wa maphunziro mbewa, yemwe anakana kopanda ichi. Pambuyo oyamba mapulotini tizilombo mu ubongo kwathunthu wathanzi nyama ali ndi khalidwe amyloid mipukutuyi. Izi zikutanthauza kuti tizilomboto mapuloteni angathe kupatsira dongosolo wathanzi. Kupezeka ali akatswiri pa yunivesite ya Texas. Posachedwapa adzafika ntchito ina asayansi ku London, lomwe limasonyeza kuti Alzheimer matenda, zizindikiro ndi zizindikiro za matenda ingathandize kuti wadutsa kuchokera ku amapatsirana.

Kumbukirani kuti matenda Parkinson amakhala ndi imfa kupita patsogolo manyuroni yotulutsa dopamine, ndi mtundu. Chifukwa cha ichi, munthuyo kutanganidwa lamulo kayendedwe ndi akulu tonus, amene anaonetsa kugwedeza, akukhudzidwira stiffness. Parkinsonism amadwala munthu aliyense zana amene anatembenuka sikisite kunja. Matendawa amayamba chitukuko ndi kutengeka pang'onopang'ono, umene uli makamaka noticeable munthu wavala kapena amatenga chakudya. Kenako, wosweka kulankhula, Pomeza bongo. Mwatsoka, mankhwala mwina amalangiza mankhwala oletsa anthu ndi matenda a "matenda Parkinson". Zizindikiro za matenda akhoza mitigated ndi mankhwala symptomatic. Komabe, ambiri mankhwala amachititsa angapo mavuto.

matenda a Alzheimer - matenda yodziwika ndi lifotabe wa manyuroni, kuchititsa odwala kukhala maganizo. Zizindikiro zoyambirira za matenda angayambe mwamsanga zaka 40. Iwo matenda nthawi zambiri bwino anthu ophunzira. Munthu ndi mkulu mlingo wa nzeru mwayi bwino kuthana ndi zizindikiro za Alzheimer chifukwa cha kugwirizana ambiri pakati manyuroni.

Matendawa amayamba chitukuko ndi matenda kukumbukira. Mbali yaikulu zambiri amaona anthu ena. Zizindikiro koyamba nthawi zambiri kuyesera kubisa kapena akuwachititsa nkhawa ndi wakewo kwambiri pa ntchito. Monga kudwala wa chithunzi matenda a mutates matenda. Wodwala salinso oyendetsa okha danga, pamtima dontho wake luso kale anapeza kulemba, kuwerenga. Choyamba, tisaiwale chochitika lotsatira pa nthawi. Pamene kudwala akuyamba patsogolo, m'pofunika ntchito mpata uliwonse kukhala ndi luso la munthu kudziona utumiki, kuyesa kuteteza zosayembekezereka maganizo. Njira yothetsera vuto zimenezi kungathandize wamphamvu kwambiri kumva chithandizo kapena magalasi bwino zoyenera. mankhwala enieni matenda a Alzheimer kulibe. Pamene zizindikiro chachikulu n'kofunika kuti tinafotokoza wathunthu ndi matenda okhudza ubongo a. zochizira akatswiri zambiri amalangiza mankhwala matendawa kuti magawowa ndipo akukangamizani chitukuko.

Matenda a matenda prion

miyeso yeniyeni matenda si panopa zinkaimira. Mwachitsanzo, ofanana akugwirira anapezazo monga matenda Creutzfeldt-Jakob, ndi pa pathologies zina ubongo. Iwo yodziwika ndi otsika Kujambula matenda tanthauzo, popeza 80% odwala awulule chizindikiro si yeniyeni. Komabe, phunziroli amatilola kudziwa ubongo manja. ukulu wake zikuchitika monga momwe ikuyendera matenda prion anthu.

Matenda masiyanidwe ikuchitika ndi pathologies onse, mawonetseredwe mmodzi amene ndi maganizo (Alzheimer matenda, vasculitis, neurosyphilis, nsungu encephalitis ndi ena).

likuyandikira chithandizo

Mwatsoka, panopa, matenda onse prion ndi wosachiritsika. Odwala anatumizidwa mankhwala symptomatic ndi anticonvulsants, amene yekha kuchepetsa mavuto. The Mapa zokhumudwitsa. Onse matenda odziwika prion amapha ndi umunthu.

Panopa, asayansi padziko lonse zikukangalika kufufuza mankhwala chilengedwe. Imachitika kafukufuku pogwiritsa ntchito nyama. Amaganiza kuti polimbana ndi matenda ngati maselo tsinde zidzagwiritsidwanso ntchito ina, komanso yisiti ambiri. mankhwala Experimental panopa mulibe Mwachangu aatali, kotero cholinga chawo ankaona zosayenera.

zodzitetezera

Kuyambira chitukuko cha mitundu ayi ndipo anatengera matenda prion kudziteteza zosatheka. Matenda ena angathe kuthetsedwa ndi kudutsa wapadera majini bwino. Komabe, mu dziko lathu ziri zovuta, chifukwa zasayansi akuchita mtundu uwu matenda, kwakukulukulu kunja.

Pankhani ya matenda otengera pamaso mimba akulangizidwa kukaonana ndi dokotala-zachibadwa. Izi zingathandize kupewa mavuto m'tsogolo thanzi la mwanayo.

Pofuna kudziteteza ku matenda Creutzfeldt-Jakob, Ndi bwino kusiya kudya nyama m'madera kumene matenda a matenda ng'ombe olembedwa. Choyamba tikulankhula za ku Ulaya. Muyeneranso kugwiritsidwa ntchito pa matenda a zokonzekera anapangidwa kuchokera magazi a nyama kapena anthu. Ndi bwino m'malo mwa analogues kupanga.

Prion matenda - si mokwanira kuphunzira mitundu ya zotupa opatsirana ndi cholowa zimene zimachitika mu thupi pa maziko a malowedwe mapulotini nthenda. Nthawi zambiri, iwo bwanji chapakati mantha dongosolo. Chithunzi matenda amakhala ndi zizindikiro zofanana. Poyamba, munthu anataya kudya ndi masomphenya, kusokoneza mgwirizano mu mlengalenga. Pa chomaliza osauka maganizo pamene wodwala akulephera kuchita paokha kusamalira ndekha. Chifukwa cha nthenda iliyonse yofanana - imfa. Panopa, madokotala alibe mankhwala polimbana ndi pathologies a chikhalidwe ichi.