Prion cututtuka: haddasawa, cututtuka, ganewar asali, magani

Prion cututtuka - shi ne na musamman irin tsanani neurodegenerative cututtuka na mutane da dabbobi. Suna halin m kwakwalwa lalacewa, a mafi yawan lokuta kawo karshen tare da sananne mutuwa.

Menene prions?

Wannan na musamman da furotin da tsarin. Su na iya zama duka al'ada da kuma wani ɓangare na tsokoki na lafiya mutane da dabbobi, kazalika da mahaukaci, haddasa daban daban iri cututtuka. Har 'yan shekarun da suka gabata da suka wuce, an yi imani da cewa rayuwa tsarin kamata dauke da ake kira nucleic acid - RNA da DNA. Godiya a gare su, yana yiwuwa a haifa. Ƙwayoyin cuta, fungi, da tsuntsaye, da dabbobi "sun dauki" nucleic acid. A baya can aka zaci cewa, su rashi a kyallen takarda ne ba zai yiwu ba ga cikakken haifa. Prion sunadarai na gaba daya ya juya wadannan ra'ayoyin.

Wadannan kwayoyin suna hada kawai na gina jiki, amma an bambanta da da ikon haifa. Iya shiga a cikin kwayoyin, sun haifar da canji žunsa a cikin al'ada pathological prions, game da shi kara da lambar. Wannan tsari na bukatar karin lokaci fiye da haifuwa na kwayoyin cuta ko ƙwayoyin cuta, don haka daga lokacin da na shiga cikin jikin kwayoyin ga ci gaban da cutar zai iya dauka shekaru da dama.

Da kaddarorin prions

Prions da prion cututtuka ne halin da wani babban juriya. Mafi hanyoyin da disinfection ne m cikin ma'amala da su. Prions ba halaka su da ruwan, za su iya jure sanyi har zuwa -40 digiri Celsius. Ba su nuna ji na ƙwarai to UV radiation da kuma riƙe dũkiyõyinsu a lokacin sarrafa formalin.

Features na tsarin sunadarai na gina jiki take kaiwa zuwa gaskiyar cewa jikin mutum ba zai iya yaqi da su. Ya ba iya samar da antibodies da prions, ba da kai hare hare da lymphocytes ba ze lura. Wannan yana nufin cewa shigar azzakari cikin farji daga kwayoyin a cikin jikin mutum entails da ya faru na wata cuta.

Prion cututtuka: tarihi na samu

A shekarar 1982, Stenli Pruziner farko da aka bayyana prion cututtuka, wanda ya sa aka daga baya bayar da kyautar Nobel.

Long kafin su samu, masana kimiyya a cikin aikin nazari da dama mutum pathologies da dabbobi, wanda sa lokaci mai tsawo ba za a iya ƙaddara. A cikin XVIII karni a United Kingdom an rajista, "scrapie" tumaki. Animals fama da tsananin itching, motsi cuta da kuma seizures, wanda ya nuna har CNS. A shekarar 1957, Daniel Carleton Gajdusek bayyana cutar a cikin fore kabilar, wanda mutane rayu a cikin tsaunuka na Papua New Guinea. A Pathology aka hade da cin naman mutane da suka wuce a daya daga mutum zuwa wani.

Tun shekarar 1986, a Ingila, da kuma daga baya a sauran kasashe, masana kimiyya sun rubuta 'yan annobar cutar da aka daga baya ba da sunan "mahaukaci saniya cuta". Yana yawanci rinjayar da dabbobinku. "Mad saniya cuta" bayan wani kankanen lokaci ya kai annoba rabbai, da kuma dalilin da ya faru sun zama prions. A shekaru 90 masana sun tabbatar da watsa wannan cuta ga mutum tare da madara da nama da dabbobinku.

A halin yanzu, a cikakken nazari da undiagnosed Sanadin cututtuka ya gudummawar da cewa masana kimiyya sun yi wani yawan bada shawarwari ga ci gaban prion yanayi. Daga gare su ne Pathology na Creutzfeldt-Jakob cuta, Alzheimer ta cutar. Alamun da ãyõyin wadannan cututtuka da yawa a cikin na kowa. Duk da m ci gaba a binciken da wadannan cuta, da yawa ya zauna a hayin comprehensible.

Ta yaya za ka samun kamuwa?

A zamani magani, akwai hanyoyi uku na kamuwa da cuta.

1. Transmissible. Prions aka canjawa wuri daga daya dabbobi masu shayarwa nau'in zuwa wani. Baya aka ambata kasancewar haka ake kira jinsin shãmaki. Wannan yana nufin cewa transfer daga wata sãniya ba zai yiwu ga mutum. Masana kimiyya ɓãta wannan ra'ayi. Sunadarai na gina jiki da za a iya daukar kwayar cutar daga kamuwa dabba ko mutum. Sanadin prion cututtuka suna lalacewa ta hanyar da ingestion nama / kamu dabba madara, da yin amfani da nazarin halittu nama (corneal dasawa, jini kayayyakin, da sauransu. D.). Daban-daban biomaterials da daban-daban digiri na pathogenicity. A mafi girma infectivity show kwakwalwa nama, mataki na gaba shagaltar da jini kayayyakin.
2. Hereditary. Haka kuma cutar tasowa a gaban kwayoyin maye gurbi, kunno kai a cikin 20th chromosome. Yana da wannan yanki yake da alhakin gaban al'ada prion gina jiki. Its aiki ne har yanzu talauci da aka sani. A cikin hali na gene maye gurbi maimakon lafiya prion pathological hada, wanda babu makawa zai kai ga ci gaban da cututtuka.
3. Harben (maras wata-wata faru na mahaukaci gina jiki).

Saboda haka, prion cututtuka na iya zama duka hereditary da kuma dauke da kwayar cutar hali. Ko da kuwa da hanyar na shigar azzakari cikin farji na mahaukaci gina jiki a cikin jiki, shi zai iya sa kamuwa da cuta ga wasu.

Abin da ya haddasa cikin prions a cikin jiki?

Mahaukaci sunadaran halin da ikon sa spongiform encephalopathies, watau CNS. Daga cikin morphological ra'ayi, wannan yana nufin samuwar cavities a cikin kwakwalwa Kwayoyin, neuronal mutuwa, overgrowth a wurin da kuma kawo karshen connective nama atrophy na kwakwalwa. Kan bango na samuwar gungu na prion amyloid plaques kiyaye. Duk wadannan matakai faruwa ba tare da shakka akwai ayoyi na kumburi.

Abin da cututtuka ne prion?

Don kwanan wata, masana kimiyya iya daidai suna 'yan cututtuka, wanda su ne hanyar da mahaukaci gina jiki Tsarin:

• Creutzfeldt-Jakob cuta.
• Kuru cuta.
• Alpers (spongy m encephalopathy) cuta.
• m iyalinmu rashin barci.
• cuta Gerstmann-Shtreusslera-Scheinker.

Next, dubi kowane a more daki-daki cikin Pathology.

Creutzfeldt-Jakob cuta

Creutzfeldt-Jakob cuta ne halin da bambancin, don haka masana razdeleli shi a dama siffofin:

• harben.
• iyali.
• iatrogenic.
• sabon atypical form.

Harben cuta embodiment baya dauke da mafi prevalent. Da farko bayyanar cututtuka bayyana bayan da shekaru 55 da shekaru. Duk da haka, a cikin shekaru 'yan shekaru da lissafi ya canza. Bayan bayyanar bayanai a kan cutar da "mahaukaci saniya cuta" suna ƙara ya fara rikodin lokuta da atypical siffofin saboda dabbõbi kamuwa da cuta. Domin irin wannan halayyar farkon bayyanar. A mafi yawan lokuta, matasa sha wahala. Alamun kasu kashi biyu matukar kungiyoyin: neurological da tabin hankali. Da farko, kamu alama ciwon kai, barci tashin hankali, rage ci. A hankali, da waɗannan cututtuka kara memory hanawa, asarar hangen nesa. Shafi tunanin mutum da cuta suna bayyana a cikin nau'i na hallucinations da rũɗi. A cutar halin da m ci gaba a karshe mataki ne halin da cikakken immobility na jiki. A mutum hasarar iko a kan aiki na pelvic gabobin. Tare da irin wannan ganewar asali mutane rayuwa ba fiye da shekaru biyu.

Bayyanar wani iyalinmu nau'i ne ya sa ta maye gurbi a cikin gene matakin a cikin Shiyyar na 20th chromosome. A cutar halin wani autosomal rinjaye hali. A farko alamun faruwa a game da 5 shekaru a baya fiye da a harben form.

Iatrogenic form tasowa a sakamakon adam kamuwa da cuta a lokacin tiyata. Ilimin kididdiga bayanai don wannan zabin ne ba wani rashin lafiya, tun da shi ne da wuya a tabbatar da pathogenesis na prion cututtuka. A shiryawa zamani jeri daga 7 months shekaru 12. Aka ƙaddara ta a hade da dama dalilai: aiwatar da shigar azzakari cikin farji na mahaukaci sunadarai a cikin jiki, ga adadinsu, da asali genotype mutum. The fi sauri girma cuta ta kai tsaye shigar azzakari cikin farji na prions a kwakwalwa nama a sakamakon tiyata. More lokaci ake bukata a lokacin kamuwa da cuta a kan bango na corneal dasawa ko na Dura Mathes. Marasa lafiya hankali tasowa cerebellar ataxia, illa jawabin da tsoka sautin, gigin-tsufa.

"Mad saniya cuta" ya fara saya munasaba bayan da annoba a cikin dabbõbin ni'ima a cikin 90s. Prion cuta, da bayyanar cututtuka da wanda ya bayyana tsakanin zamanai na 30 zuwa game da shekaru 40, shi ne m da mutane. Kamar yadda da iatrogenic form, neurological cututtuka fi yawa a kan tunani.

Fatal iyalinmu rashin barci

Wannan cuta ne autosomal rinjaye hali, wanda ake daukar kwayar cutar ta hanyar kawai jere. Fatal rashin barci ne rare. Ta aka sani a kimiyya tun shekarar 1986. Its farko bayyanar cututtuka dake da shekaru 25 da shekaru su game da 71 da shekara.

Epidemiology na prion cututtuka na da irin wannan suna arancinsu bincika. Babban alama na m iyalinmu rashin barci ne sleeplessness. The jiki a hankali hasarar da ikon cikakken daidaita cikin lokaci na wakefulness da kuma barci. Har ila yau, marasa lafiya bayyana motsi cuta da tsoka rauni. Akwai sun kasance lokuta na autonomic cuta cewa bayyananne ya karu jini da ya wuce kima sweating. Shafi tunanin mutum cuta za a iya lura tsoro harin, na gani hallucinations, kuma intermittent aukuwa na rikice. Saboda rashin barci akai ci auku, da haƙuri mutu.

Kuru

Kamuwa prions fom da aka yi karatu a daki-daki, saboda cuta, mafi musamman, a kabilar cannibals. Har shekara ta 1956 tsakanin mazauna Papua - New Guinea aka rarraba abin da ake kira hadisin al'ada cin naman mutane - cin kwakwalwa na marigayin mutum. An yi imani da cewa daya daga cikin mambobi na wannan kabilar tashi kamuwa da cuta, wanda daga baya ya yadu zuwa sauran mutane bayan da na al'ada. Tun da soke wannan al'ada da cutar lokuta da aka rubuta a sau da yawa m akai-akai yau wannan cuta kusan ba ya faruwa.

A shiryawa zamani jeri daga 5 zuwa shekaru 30. Wannan shi ne dalilin da ya sa Kuru cuta ake magana a kai a matsayin "m cutar kamuwa da cuta" category. Rashin lafiya cerebellar cuta bayyana tare da wanda ba a iya lura da shi ba dariya, hadiya tabarbarewa da tsoka rauni. A m matakai na bunkasa gigin-tsufa. Mutane tare da wannan ganewar asali rayuwa ba fiye da 30 months.

Alpers cuta

Cutar mafi yawa yakan faru a cikin matasa da yara (a karkashin 18 years). Rashin lafiya yana daukar kwayar cutar a cikin wani autosomal recessive hanya, a cikin hali na daidaituwa na biyu pathogenic kwayoyin halittu ubanni da uwaye. Daga cikin babban bayyanar cututtuka za a iya gano Heart hangen nesa da kuma seizures. A likita reference books akwai kwatancin m rashin lafiya da ke faruwa ta hanyar bugun jini irin. Alpers cutar da ake kuma halin da raunuka na hanta, abin da sauri tasowa a cikin kullum hepatitis da kuma kawo karshen tare da cirrhosis. Marasa lafiya suna mutuwa saboda maye for 12 watanni bayan ganewar asali na farko bayyanar cututtuka.

Gerstmann ciwo-Shtreusslera-Scheinker

Wannan wani zaɓi an ranked a matsayin hereditary cuta irin. Very m (daya hali game da 10 da mutane miliyan). A farko alamun yawanci gani a marasa lafiya a kan shekaru 40 da. A ci gaba da ciwo fara da cerebellar cuta. Da farko dizziness bayyana. Kamar yadda cutar progresses koordinatornye keta hankali m motsi zama ba zai yiwu ba. Tare da aka jera bayyanar cututtuka bayyana cuta da tsoka sautin, da rage a hangen nesa da kuma ji, da matsaloli tare da hadiya da haifuwa. A karshen-mataki gyarawa likitoci manifestations na gigin-tsufa. A tsawon rayuwar marasa lafiya tare da wannan ganewar asali har zuwa shekaru 10.

Alzheimer ta cutar da Parkinson ta cuta

Alzheimer ta cutar da Parkinson ta cuta, cututtuka da kuma mu'ujizan da na kowa hali, masu tasowa, a irin wannan hanya to prion ailments. Kwayoyin na beta amyloid, tau da furotin da kuma da sauran fannin jiki kuma samar adibas na pathogenic yanayi a kwakwalwa nama. Duk da haka, don kama wadannan cututtuka zai yiwu ba. Wannan yana nufin cewa amyloid fibrils an kafa ta lalace sunadarai na gina jiki, amma "lafiya" mataki "lafiya" ba tambaya.

More kwanan nan, masana kimiyya sun gudanar da wani jerin karatu a beraye, wanda hana wannan zato. Bayan gabatarwar pathogenic sunadarai a cikin kwakwalwa ne gaba daya lafiya dabba yana da halayyar amyloid plaques. Wannan yana nufin cewa pathogen gina jiki zai iya har yanzu harba wani m tsarin. A samu nasa ne kwararru daga Jami'ar Texas. A nan gaba za su zo da wani aiki na masana kimiyya daga London, wanda ya nuna cewa Alzheimer ta cuta, cututtuka da kuma alamun rashin lafiya zai iya cikakken a wuce daga mutum daya zuwa wani.

Ka tuna cewa Parkinson ta cutar halin m asarar neurons cewa samar da dopamine, a neurotransmitter. Saboda wannan, mutumin da yake damuwa tsari na ƙungiyoyi da kuma murdede tonus, wanda aka bayyana da tremor, overall stiffness. Parkinsonism shan wahala daga kowane ta ɗari mutum wanda ya jũya sittin kasashen waje. Haka kuma cutar ta fara faduwa ci gaba da jinkirin motsi, wanda shi ne musamman m idan wani mutum yana sanya a kan ko daukan abinci. Daga bisani, karya magana, hadiya reflexes. Abin baƙin ciki, da magani ba bayar da shawarar da wani tasiri magani ga mutane da ganewar asali "Parkinson ta cuta". Da ãyõyinMu, kuma bayyanar cututtuka da wannan cutar za a iya saukake ta symptomatic far. Duk da haka, mafi yawan wadannan kwayoyi sa wani yawan illa.

Alzheimer ta cutar - wata cuta halin da withering bãya daga neurons, haddasa marasa lafiya ci gaba gigin-tsufa. A farko bayyanar cututtuka da wannan cuta na iya faruwa a farkon shekaru 40. An kamu a mafi yawan lokuta talauci ilimi mutane. A mutum da wani babban matakin da hankali mafi damar jimre da bayyanar cututtuka na Alzheimer ta saboda da yawa sadarwa tsakanin neurons.

Haka kuma cutar ta fara faduwa ci gaba da memory cuta. A farko mataki yawanci ke kada a gane shi ga wasu. A farko bayyanar cututtuka sukan kokarin rufe up ko zargi a kan danniya da kuma wuce kima workload a wurin aiki. Kamar yadda ci gaban na asibiti hoto da cutar mutates. A haƙuri ne ba Orient kansu a sarari, daga memory drop baya samu basira da rubuce-rubuce, karanta. Da farko, manta na gaba aukuwa a kan lokaci. Lokacin da Pathology fara ci gaba, wajibi ne a yi amfani da kowane damar kula da mutum ikon kai sabis, don kokarin hana abin da ya faru na ciki. A bayani na wannan matsalar iya taimaka more iko taimakon ji ko yadda ya kamata Fitted tabarau. Specific magani daga Alzheimer ta ciwo ba ya wanzu. Lokacin da farko bayyanar cututtuka, yana da muhimmanci don samun cikakken jarrabawa da wani neurologist. domin lura da masana yawanci bayar da shawarar kwayoyi da cutar sauƙaƙe da kallafa ta ci gaba.

Ganewar asali prion cututtuka

Specific bincike matakan da aka ba a halin yanzu wakilta. Alal misali, irin wannan EEG binciken kamar yadda a Creutzfeldt-Jakob cuta, suna a sauran kwakwalwa pathologies. Yana da halin low MRI bincike muhimmanci, tun 80% na marasa lafiya bayyanãwa ba takamaiman sakonni. Duk da haka, wannan binciken ya yale mu mu gane kwakwalwa atrophy. Its mai tsanani ne aggravated a matsayin ci gaban mutum prion cututtuka.

A bambanci ganewar asali ne da za'ayi tare da duk pathologies, daya bayyana abin da yake gigin-tsufa (Alzheimer ta cuta, vasculitis, neurosyphilis, herpes encephalitis da sauransu).

Jiyya ta kusance

Abin baƙin ciki, a halin yanzu, duk prion cututtuka ne m. Marasa lafiya sanya symptomatic far da anticonvulsants, wanda kawai ya rage wahala. A hasashen m. Duk aka sani prion cututtuka ne m ga dabi'ar mutum.

A halin yanzu, masana kimiyya daga ko'ina cikin duniya suna rayayye neman a duniya magani. Gudanar da bincike da yin amfani da dabbobi. An zaci cewa a yaki da cututtuka irin kara Kwayoyin za a yi amfani da daga baya, kazalika da mafi yawan na kowa yisti. Gwajin kwayoyi a halin yanzu ba su da wata high dace, don haka su nufi yana dauke da bai dace ba.

m matakan

Daga cikin ci gaban harben kuma gaji bambance-bambancen karatu na prion cututtuka su kare kansu kusan ba zai yiwu. Wasu cututtuka da za a iya shafe ta wucewa na musamman kwayoyin jarrabawa. Duk da haka, shi ne a kasar yana da wuya sosai, saboda dakin gwaje-gwaje yin wannan irin ganewar asali, ne sun fi mayar da kasashen waje.

A cikin hali na hereditary cututtuka kafin daukar ciki an rika tuntubar likita-jini. Wannan zai taimaka kauce wa nan gaba matsaloli tare da kiwon lafiya na yaro.

Domin kare kansu daga Creutzfeldt-Jakob cuta, shi ne shawarar zuwa daina cin nama daga cikin yankuna inda lokuta na shanu cuta rubuta. Da farko muna magana ne game da kasashen Turai. Ya kamata ka kuma ba za a yi amfani da magani na shirye-shirye sanya daga jinin dabba ko mutane. Yana da kyau a maye gurbin su da roba analogues.

Prion cututtuka - ba isasshe karatu siffofin cututtuka da kuma hereditary raunuka da faruwa a cikin jikin mutum a kan bango na shigar azzakari cikin farji na mahaukaci sunadaran. A mafi yawan lokuta, suka shafi cikin tsakiya m tsarin. The asibiti hoto ne halin da irin wannan bayyanar cututtuka. Da farko, mutumin rasa ci da kuma hangen nesa, gaji da damuwa da daidaituwa a sarari. A karshe mataki na bunkasa gigin-tsufa a lokacin da haƙuri shi ne kasa da kansa kula da kaina. A sakamakon wani ciwo ne ko da yaushe guda - mutuwa. A halin yanzu, likitoci ba su da tasiri magani a kan pathologies wannan yanayi.