cruitsfeld-jacob rogam

cruitsfeld-jacob rogam (vii. sii. jeey. dii), saadhaaranayaayi "maadu kau disease" allengil "human maadu kau disease" ennu vilikkappedunnu, ithu athinte bse kaundarpaarttil ninnu verthirichariyaan, transmissiable sponchiphom encephalopathy kudumbatthile oru marakamaaya tharam mastishka rogamaanu. prarambha lakshanangalil maanasika prasnangal, perumatta mattangal, vedanaajanakamaaya samvedanangal enniva ulppedunnu. rogathinte pilkkala ghattangalil, rogikalkku mosham ekopanam, demensia, swamedhayaa ulla chalanagal enniva prakadamaayekkaam. exposure rogalakshanangalude vikaasavum thammilulla dairkhyam vyakthamallengilum varshangal muthal pathittaandukal vare aanennu vishwasikkappedunnu. rogalakshanangal aarambikkunnathinu sheshamulla sharashari aayurdairkhyam 13 maasamaan.
thettaaya madakkukalulla proteinukalaaya priyanukal moolamaanu ithu sambhavikkunnath. bovin sponchiphom encephalopathy (bii. ezz. iii) baadhicha beef kazhikkunnathaanu pradhaanamaayum vyaapanathin kaaranamenn vishwasikkappedunnu. anubaadhaykk oru prathyeka janithaka samvedanakshamatha aavasyamaanennum vishwasikkappedunnu. raktha uthppannangaliloodeyo malinamaaya sasthrakriya upakaranangaliloodeyo vyaapanam sambhavikkam. mastishka biopsy vazhiyaan roganirnayam nadathukayenkilum mattu chila maanadandangalude adisthaanathil ithu samshayikkaavunnathaan. iva randum priyans moolamaanengilum ithu saadhaarana cruitsfeld-jacob rogathil ninnu vyathyasthamaan.
vii. sii. jeey. dii. yude chikilsayil supportive cair ulppedunnu. 2020 leeii kanakkanusarichu, 1990-kalile pottippurappettathinnettathu yunaittad kingdatthil 178 kesukalum lokathinte mattu bhaagangalil 50 kesukalum rekhappeduthiyittundu. 2000 muthal eee rogam saadhaaranamallaathayi. saadhaarana aarambikkunna praayam 30 vayassil thaazheyaan. 1996ll skotlandile edinbergilulla deseeya sijedi nireekshana yoonittaan ithu aadyamaayi thiricharinjathu.
adayaalangalum lakshanangalum.
prarambha lakshanangalil maanasika prasnangal, perumatta mattangal, vedanaajanakamaaya samvedanangal enniva ulppedunnu. rogathinte pilkkala ghattangalil, rogikalkku mosham ekopanam, demensia, swamedhayaa ulla chalanagal enniva prakadamaayekkaam. exposure rogalakshanangalude vikaasavum thammilulla dairkhyam vyakthamallengilum varshangalaanennu vishwasikkappedunnu. rogalakshanangal aarambikkunnathinu sheshamulla sharashari aayurdairkhyam 13 maasamaan.
kaaranam.
kalankithamaaya beef.
yukeyil, vii. sii. jeey. diyude praadhamika kaaranam bovin sponchiphom encephalopathy kondu kalankappeduthiya beef kazhikkuka ennathaanu. aarogya samrakshana ejansi 2012 ll nadatthiya oru padanatthil yukeyile 2,000 peril oraal asaadhaaranamaaya priyan adinjukoodunnathinte lakshanagal kaanikkunnuvenn kaanichu.
munbu sasyabhukkukalulla kannukaalikalkk mamsavum asthimarunnum (protein srotassaayi) nalkaanulla manushyante theerumaanam munbu oru mruga rogakariyaayirunnava manushya bhakshya shringalayilekku praveshikkaan kaaranamayathinaal, avide ninnu manushyarkk vii. sii. jeey. dii pidipedaan kaaranamayathinaal, harward medical skoolile global health aandu soshyal medisin vakuppile medisin instructor jonathan kwick, bovin sponchiphom encephalopathy allengil bse manushyanirmmithamaya aadyathe pakarchavyaadhiyaanu allengil "fraankanstain" rogamaanennu prasthaavichu.
raktha uthppannangal.
2018 leeii kanakkanusarichu, vii. sii. jeey. dii ulla vyakthikalude rakthathil priyans undaakaamenn thelivukal soochippikkunnu, pakshe edaykkideyulla sii. jeey. dii ulla vyakthikalil ithu anganeyalla.
rakthappakarchayiloode vii. sii. jeey. dii pakaraan kazhiyumennu 2004-leeii oru report kaanichu. rogathinte oru valia pakarchavyaadhi sameepabhaviyil undaayekkaamennathinaal eee kandethal aarogya udyogasthare aashankappeduthi. vii. sii. jeey. dii anubaadhaykkulla rakthaparisodhana saadhyamaanengilum raktadana parishodhanaykk ithuvare labhyamayittilla. raktha vitharanam samrakshikkunnathinu kaaryamaaya niyanthranangal nilavilundu. 1980 januvari muthal rakthappakarcha labhicha aareyum rakthadaanathil ninnu yuke sarkkar vilakki. 1999 muthal albumin polulla bhagika uthppannangal nirmmikkaan yuke raktham upayogikkunnathinu yukeyil nirodhanam nilavilundu.
eee niyanthranangal dvitheeya anubaadhakalude svayam nilanirthunna pakarchavyaadhi thadayunnathinu eathenkilum vidhathil sahaayichekkaamenkilum, rogabadhithamaaya rakthadaanam athrayaanennu anjjaathamaanu, athu ganyamayirikkaam. rakthappakarchayiloode maranangal 176 aayi anchiratti uyarumennu 2013 juunil sarkkaarin munnariyipp nalkiyirunnu.
2002 meyy 28 nuu, yunaittad stetts phudu aandu drugg administration 1980 muthal 1996 vare chila europian rajyangalil (allengil yunaittad kingdatthil moonnu maasam) kuranjathu aarr maasamenkilum chelavazhicha aareyum rakthadaanathil ninnu ozhivaakkunna oru nayam earppeduthi.ezz. eee nayam kaaranam europil thamasikkunna sainika udyogastharum avare aasrayikkunnavarum 7 sathamaanathiladhikam daathaakkale maattivaykkumennu pratheekshichirunnu. pinneedu eee nayathile mattangal aadyam europian rajyangalil motham anchu varshamo athil kooduthalo ulla sivilian yaathrakalkkulla niyanthranam laghookarichu (sainikamaanengil aarr maasamo athil kooduthalo) pinneedu 2022 ll athu poornnamaayum neekkam cheythu. ennaal yukeyilekkulla yaathraykkulla moonnu maasathe niyanthranatthil mattamilla.
nyoosilandil, 2000-ll nyoosiland blud sarvees (enn. ezz. bii. ezz) 1980 januvarikkum 1996 desemberinum idayil yunaittad kingdatthil (ail oof maan, chaanal ailands ulppede) sthiramaayi thamasikkunna daathaakkale aarr maasamo athil kooduthalo thadayunnathinulla nadapadikal avatharippichu. eee nadapadiyude falamaayi nyoosilandile sajeeva rakthadaathaakkalil patthu sathamaanam perum akkaalat rakthadaanathinu ayogyaraayi. 1980 januvari muthal yunaittad kingdatthil rakthappakarcha sweekarikkunna daathaakkale shaashvathamaayi thadayunnathinaayi 2003 ll enn. ezz. bii. ezz niyanthranangal kooduthal vipuleekarichu, 2006 eprilil republic oof irelandineyum francineyum ulppeduthunnathinaayi niyanthranangal kooduthal neetti.
1980 januvarikkum 1996 desemberinum idayil germmaniyil aarumaasathil kooduthalo fraansil oru varshamo chelavazhicha aareyum rakthadaanathil ninnu shaashvathamaayi vilakkiyirikkunna samanamaaya chattangal nilavilundu.
kaanadayil, 1980 januvari 1 muthal 1996 desember 31 vare ukeyilo francillo motham moonnu maasamo athil kooduthalo chelavazhichittundenkil vyakthikalkk rakthamo plasmio daanam cheyyaan arhathayilla. 1980 januvari 1 muthal 2001 desember 31 vare republic oof irelandil motham anchu varshamo athil kooduthalo chelavazhichittundenkilo 1980 januvari 1 muthal 1996 desember 31 vare soudi arabiayil motham aarr maasamo athil kooduthalo chelavazhichittundenkilo 1980 muthal yuke, france allengil padinjaaran europil rakthappakarcha nadathiyittundengilo avarkk arhathayilla.
polandil, 1980 januvari 1 num 1996 desember 31 num idayil yuke, ireland, france ennividangalil motham aarumaasamo athil kooduthalo chelavazhicha aareyum sambhavana cheiyunnathil ninnu shaashvathamaayi vilakkiyittundu.
fraansil, 1980 januvari 1 num 1996 desember 31 num idayil yunaittad kingdatthil thaamasikkukayo thaamasikkukayo cheytha aareyum sambhavana cheiyunnathil ninnu shaashvathamaayi vilakkiyittundu.
check republicil, 1980 num 1996 num idayil ukeyilo francillo aarumaasathil kooduthal chelavazhichavarkko 1980 nuu sesham yukeyil rakthappakarcha labhichavarkko rakthadaanathinu anuvaadamilla.
finlandil, 1980 januvari 1 num 1996 desember 31 num idayil motham aarr maasathiladhikam britiish dveepukalil thaamasichavaro thaamasichavaro aaya aareyum sambhavana cheiyunnathil ninnu shaashvathamaayi vilakkiyittundu.
beejadaanam.
yuu. eiii. yill.ezz.oru kaalath scandinavian beejathinte janapriyamaaya irakkumathi thadayunna cruitsfeld-jacob rogathinte apakadasaadhyathayil ninnu prachodanam ulkkond eathenkilum daathaakkalude beejathinte irakkumathi fda nirodhichu. ennirunnaalum, cruitsfeld-jacob laingikamaayi pakarunnathinu thelivukalonnumillaathil apakadasaadhyatha valare kuravaanennathaanu shaasthreeya samavayam.
thozhil malineekaranam.
fraansil, fraansil 2019lum maricha cruitsfeld-jacob rogam baadhicha avasaanathe rund irakal naashanal research instittyoott phoor agrikalchar, phudu aandu envayonmentile (intre) gaveshana saankethika vidagtharaayirunnu. 2019ll 33-aamm vayassil maricha emily joumain 2010ll oru thozhil apakadatthinu irayaayi, eee samayat rogabadhithamaaya masthishkam malinamakkiya oru upakaranam upayogichu svayam kuthukayaayirunnu. malineekaranatthinte eee paathayude falaprapti praimettukalil vyakthamaayi prakadamaanu. pieret pieret maricha addeham samanamaaya thozhil apakadatthinu irayaayi. roganirnayathinu sesham, pakarchavyaadhi priyanukalekkurichulla gaveshana pravarthanangalekkurichu ellah franch laborattarikalilum moratorium aarambhichu. 2022 maarchil, eee rund maranangaludeyum thozhilparamaaya kaaranam intre thiricharinju. ithu eee laborattarikalile jeevanakkaarude surakshayekkurich gurutharamaaya chodyangal uyarthunnu. theerchayaayum, eee marakamaaya apakadasaadhyathaye abhimukeekarikkunna ejantumaarude samrakshanathil gurutharamaaya parajayangal parisodhanakal rekhappeduthiyittundu, eee rogathinte deerghamaaya incubation kaalayalav bhaviyil puthiya kesukal undaakumenna bhayathilekku nayikkunnu, athinaal valia aashankayundu.
mattu kaaranagal.
annaakkukalude thalachoru kazhichathiloode oru vyakthikk vii. sii. jeey. dii baadhichathinaal annaakkukalil ninnullath polulla mattu tharatthilulla thalachorukal kazhikkunnath shupaarsha cheiyunnilla.
samvidaanam.
yukeyil malinamaaya beef upabhogam kooduthalaanengilum, vii. sii. jeey. dii cheria ennam aalukale baadhichittundu. rogamulla aalukalude janithakashaasthrathil ithinu oru vishadeekaranam kandethanakum. priyan rogathil attimarikkappedunna manushyante pii. aarr. enn. pii protein, amino assid 129-ll methionin allengil valin upayogichu prathyakshamaaya shaareerika vyathyaasangalonnumillathe sambhavikkam. motham velutha janasamkhyayude 40 sathamaanathinu rund methionin adangiya alleelukalum 10 sathamaanathinu rund valin adangiya alleelukalum backie 50 sathamaanam eee sthaanathu heterosaigas aanu. vii. sii. jeey. dii. ulla oraalkk maathrame hetarosaigas ullathayi kandethiyulloo; baadhichavaril bhooribhaagathinum methionin adangiya roopathinte rund pakarppukal undaayirunnu. baadhikkaathavar yadhaarthathil rogaprathirodhasheshiyullavarana atho rogalakshanangal prathyakshappedunnathuvar kooduthal incubation kaalayalav maathrame ulloo ennu ithuvare arivayittilla.
roganirnayam.
nischayaathmakamaayi.
sijedi vakabhedam kandethunnathinu mastishka koshangalude parisodhana aavasyamaanu. thaazhepparayunna sthireekarana savisheshathakal undaayirikkanam
vargeekaranam.
classic cruitsfeld-jacob rogathil ninnu vyathyasthamaaya oru avasthayaanu vii. sii. jeey. dii (randum prp priyans moolamaanu undaakunnath). classic, veriyant sijedi enniva cruitsfeld-jacob rogathinte upavibhagangalaanu. sijedi rogathinu moonnu pradhana vibhaagangalund sporadic sijedi, heridari sijedi, aquared sijedi, veriyant sijedi itrogenic sijedicoppam aquared groupilaanu. classic roopathil edaykkideyullathum paarambaryavumaaya roopangal ulppedunnu. sporadic cjdyaanu ettavum saadhaaranamaaya tharam.
icd-10 nuu vii. sii. jeey. dick prathyeka coode illa, atharam kesukal cruitsfeld-jacob disease coode (a81.0) prakaram report cheyyappedunnu.
epidemiology.
"2006-ll "lancet" nirddeshichathu, kuruvinekkurichulla avarude padanangalil ninnu, pappuva nyoo giniyayile samanamaaya oru rogam vikasippikkaan vii. sii. jeey. dick 50 varshathiladhikam eduthekkaam ennaanu. pappuva nyoo giniyayile narabhojathiloodeyaakaam kudumbamgangal maranapetta bandhuvinte mrithadeham dukhatthinte adayaalamaayi kazhikkumbol kuru pakarnnathennaan avakaashavaadathinu pinnile kaaranam. soochippikkunnu pappuva nyoo giniyayil narabhojithvam nirodhichu.
ennirunnaalum, irupathaam noottaandinte avasaanathil, chila pappuva nyoo ginian kammyoonittikalil kuru pakarchavyaadhi anupaathathiletthi, athinaal vii. sii. jeey. dick 20 muthal 50 varsham vare samanamaaya incubation kaalayalav undaakaamenn soochippikkunnu. soochippikkunnu pappuva nyoo giniyayil morchari narabhojithvam nirodhichirunnuvenkilum, aaa sambradaayam avasaanichuvennu ithinarthamilla ennathaanu eee sidhaanthathinte vimarsanam. pathinanju varshathinu sesham eee sambradaayam thudarunnathaayi pappuvanmaar jared diamond ariyichu.
neenda incubation kaalayalavukal prolsaahippikkunnathayi ariyappedunna kuru ulla chila aalukalil janithaka vyathiyaanam eee gaveshakar shradhichu. 1990 kalude thudakkathil sijedi baadhicha vyakthikal bovin sponchiphom encephalopathy (bse) yude asaadhaaranamaaya hraswa incubation kaalayalavukalulla oru vyathyastha janithaka upajanasankhyaye prathinidheekarikkunnuvennu avar nirddeshichittundu. ithinartham, kooduthal varshangalkk sesham prathyakshappedunna, kooduthal incubation kaalayalavukalulla, vii. sii. jeey. dii ulla niravadhi aalukal undakam ennaanu.
vii. sii. jeey. diyil neurological lakshanagal aarambhikkunnathin rund varsham munbu vare lymphoidilum appendix tissuevilum priyan protein kandethanakum. yukeyile valia thothilulla padanangal dashalakshathil 493 enna thothil praabalyam kaanikkunnu, ithu report cheyyappetta kesukalude yadhaartha ennathekkaal kooduthalaanu. eee kandethal dhaaraalam rogalakshanangalillaatha kesukaleyum nireekshikkendathinte aavasyakathayeyum soochippikkunnu.
samoohavum samskaaravum.
1997ll kentakkiyile niravadhi aalukal sijedi vikasippichu. ellaavarum annaakkukalude thalachoru kazhichathaayi kandethi. rogavum eee bhakshanakramavum thammilulla yaadrishchikamaaya bandhatthil ulppettirikkaam.
2008ll, vii. sii. jeey. diyude chila kesukalude vishalamaaya exposure neenda incubation kaaranam manushyarude randaamathe tharamgatthinulla saadhyathayekkurich yuke shaasthranjar aashanka prakadippichu. 2015ll newyorkkil ninnulla oraalkk annaante thalachoru kazhichathinu sesham vii. sii. jeey. dii vikasichu. 2017 november muthal 2018 epril vare, rochasttaril rogam samshayikkunna naalu kesukal uyarnnu.
yunaittad kingdom.
vii. sii. jeey. dii. yill ninnulla aadyathe manushya maranam yunaittad kingdatthilaanu sambhavichathu; wiltshare kaumarakkaaranaaya steefan charchil 1995 meyy 23 nuu 19 aam vayassil marichu. yukeyile 2,000 peril oraal eee rogathinte vaahakaraanennum malinamaaya beef kazhikkunnathumayi bandhappettirikkunnavum gaveshakar vishwasikkunnu. sarway innuvareyulla ettavum sakthamaaya vyaapana alavu nalkunnu-koodaathe munbum ellah genodypeukalilum kandethiyathinekkaal vishalamaaya praayathilulla asaadhaaranamaaya priyan protein thirichariyunnu, ithu "anubadha" thaarathamyena saadhaaranamaayirikkaamennu soochippikkunnu. eee puthiya patanam 32,000-ladhikam anjjaatha appendix saambilukal parishodhichu. ivayil 16 saambilukal asaadhaaranamaaya priyan protein positteev aayirunnu, ithu motham janasamkhyayil 493 enna thothil soochippikkunnu, allengil 2,000 peril oraal vaahakaraakaan saadhyathayund. rund lingangalilum vyathyastha janana koottangalil (1941-1960,1961-1985) vyathyasamonnum kaanapettilla, koodaathe moonnu vishalamaaya bhoomishaasthraparamaaya pradeshangalil asaadhaaranamaaya priyan vyaapanathil vyakthamaaya vyathyasamonnum undaayirunnilla. 16 positteev saambilukalude janithaka parisodhanayil britiish janasamkhyayumaayi thaarathamyappeduthumbol priyon protein (pii. aarr. enn. pii) encode cheiyunna jeeninte kodoon 129-ll valin homocygus (vii. vii) jeenodaippinte uyarnna anupatham kandethi. ithu vii. sii. jeey. dii ulla 176 peril ninnu vyathyasthamaan, avarellam innuvare methionin homocygus (emm) genotype aanu. eee vivi genotype ulla vyakthikalkk kooduthal samayathekku incubation kaalayalavil eee avastha vikasippikkaan saadhyathayund ennathaanu aashanka.
human bii. ezz. iii foundeshan.
2000-ll vii. sii. jeey. dii baadhichu marichavarude kudumbangal oru sannadha pinthunaa samgham roopeekarichu. samanamaaya anubhavathiloode kadannupokunna mattu kudumbangale pinthunaykkuka ennathaayirunnu lakshyam. oru deseeya help line, oru cair guide, oru websait, kudumba sauhruda shringhala ennivayiloodeyaanu eee pinthuna nalkiyathu. 21-aamm noottaandinte thudakkathil eee pinthunaa grouppukalkku internettu saannidhyam undaayirunnu. foundeshante pinnile prerakashakthi lesttar firkins aayirunnu, addehathinte ilaya makan rogam baadhichu marichu.
2000 octoberil loard philipsinte adhyakshathayil bii. ezz. iii. yekkurichulla sarkkar anweshana report prasidheekarichu. muun kansarvetteevu party kaarshika mantrimaaraaya joonn gummer, joonn mcgregor, dougless hogg ennivare bii. ezz. iii report vimarshichu. bii. ezz. iii. oru prathisandhiyilekku valarnnathu theevramaaya krishiyude falamaanennu report nigamanam cheythu, prathyekichu pashukkalkk pashuvinteyum aadukaludeyum avashishtangal nalkunnathinte falamaanu. koodaathe, prathisandhi kaikaaryam cheytha reethiye report vimarshichu. bii. ezz. iii. kku speeshees barrier kadakkaan kazhiyumenna saadhyatha pariganikkaan vimukhathayundaayirunnu. britiish beef kazhikkaan surakshithamaanennu sarkkar pothujanagalkk urappu nalki, krishi mantri joonn gummer thante makalkku oru burger nalki. britiish gavanment prathikaranatthil sajeevamaayathinekkaal kooduthal prathikarikkukayaayirunnu; 1996 maarchil ellah britiish beef kayattumathikalkkum lokamembaadum earppeduthiya nirodhanam gurutharamaaya saambathika thirichadiyaayirunnu.
vii. sii. jeey. dii ullavare paripalikkaan bandhukkale sahaayikkunnathin oru paricharana paakkej ulppeduthunnathinaayi foundeshan nashtapariharam aavasyappettirunnu. aarogya, saamoohika sevanangalude aparyapthamaaya pinthunayekkurichu vyapakamaaya paraathikal undaayittund. 2001 oktobarile phillips reportinethudarnnu, vii. sii. jeey. dii baadhicha britiish janathaykkaayi sarkkar oru nashtaparihara padhathi prakhyaapichu. multy-milyan poundu saambathika paakkejin melnottam vahichathu vii. sii. jeey. dii trasttaanu.
ekadesham 2000-ll central landanil vii. sii. jeey. dii moolam marichavarkkulla oru smaaraka phalakam sthaapichu. lambethile sent thomas hospitalinte athirthi mathililaanu ithu sthithicheyyunnathu.

