Rare Inflammatory Disease Inowanikwa mune Dzimwe Mamwe Mahedheni
Sangano reMediterranean Mediterranean (FMF) rinowanzosangana nehutano hwema genetic hunoonekwa zvikuru mune mamwe marudzi evanhu. Iyo inoratidzirwa nekudzoka kwemazuva ose efivha, appendicitis-sekurwadziwa kwepamu, kuputika kwemapapu, uye kuputika, maziso anorwadza.
Sekugara kusingaperi, kuora mwoyo, FMF inogona kukonzera kukwana kwenguva pfupi uye inonyanya kukanganisa hupenyu hwomunhu.
Nenzira yakanaka, itsva, anti-inflammatory drugs zvese asi yakabvisa zviratidzo zvakawanda zvechirwere ichi.
FMF inoshandiswa pakugadzirisa autosomal disorder , zvinoreva kuti yakagara nhaka kubva kuvabereki vako. Chirwere ichi chinosanganiswa nekuchinja kwemuMediterranean Fever (MEFV) gene rinoshandiswa kupfuura makumi matatu. Kuti munhu aone zviratidzo, iye aizoda kuti ave nhaka yezvinyorwa zvekuchinja kwevabereki vose. Kunyange zvakadaro, kuva nemakopi maviri hazvirevi kurwara nguva dzose.
Kunyange zvazvo zvisingawanzoitiki muhuwandu hwevanhu, FMF inoonekwa kakawanda muSefardic vaJudha, Mizrahi vaJudha, Armenia, Azerbaijan, maArab, maGiriki, maTurkey, nemaItaly.
Kurwara Kwakaita Sei?
Kusiyana nehutachiona hwemotokari iyo iyo maitiro ezvirwere zvekuzvidzivirira anorwisa masero ayo, FMF chirwere chinopisa muviri umo inonzi immate system system (mutambo wekutanga yekuzvidzivirira kwemuviri) haishande sezvinofanira.
Zvirwere zvinoputira muviri zvinoratidzwa nekusavhiringidzwa kwekudzimba, kunyanya sechikonzero chechirwere chakazvarwa.
Ne FMF, mutambo weMEFV unenge uchiita nguva dzose unobata chromosome 16 (imwe yezvikamu makumi maviri nezviviri zvemakromosomes zvinoumba DNA yomunhu ). Chromosome 16 inotarisirwa, pakati pezvimwe zvinhu, kusika puroteni inonzi pyrin inowanikwa mune mamwe masero echeche edzivirira eropa.
Kunyange zvazvo basa re pyrin richiri kusati rakanyatsojeka, vakawanda vanotenda kuti puroteni inokonzera kupisa maitiro ezvirwere zvemuviri nekuchengetedza kuvhiringidza.
Pakati pemakumi matatu nemasere ekusiyana kweMEFV mutation, kune zvine zvakanyatsobatanidzwa nechirwere chechiratidzo.
Zviratidzo
FMF inonyanya kukonzera kupisa kweganda, nhengo dzomukati, uye majoini. Kurwiswa kunoratidzika kune rimwe zuva- kusvika kwemazuva matatu kwemazuva ane musoro uye fever pamwe nemamwe mamiriro ekuvhiringidza, akadai se:
- Pleurisy , kuputika kwembatya yemapapu inoratidzwa nekuputira kunorwadza
- Peritonitis, kuputika kwemuviri mudumbu kunoratidzwa nekurwadziwa, unyoro, fivha, kushungurudza, uye kurutsa
- Pericarditis , kuputika kwembambo yemwoyo inoratidzirwa nehupamhi, kubaya marwadzo epfupa
- Meningitis , kuputika kwemakumbo kunoputira uropi nemutsipa wepelinha
- Arthralgia (marwadzo akabatana) uye arthritis (kubatana kwekubatana)
- Kupararira, kuputika kukuru, kazhinji pasi pemabvi
Zviratidzo zvinotarisana zvakakomba kubva kune unyoro kusvikira zvichishatisa. Kuwedzerwa kwekurwiswa kunogonawo kusiyana kubva mumazuva mashomanana kusvika kumakore mashomanana. Nepo zviratidzo zve FMF zvingagadziriswa kubva paucheche, zvinowanzotanga mumakore makumi maviri.
Matambudziko
Zvichienderana nehuwandu uye huwandu hwekurwisa, FMF inogona kukonzera matambudziko ehutano kwenguva refu. Kunyange kana zviratidzo zvinyoro, FMF inogona kuita kuti puroteni inonzi se serum amyloid A. Inodolle proteins inogona kuwedzera uye inokonzera kukanganisa kune nhengo dzakakura, kunyanya mafungiro.
Kukwepa kwepoyi , ikoizvo, kushungurudza kwakakomba kweFMF. Isati yasvika pakurapwa kwemishonga inopesana nekuputika, vanhu vane FMF-vane hutano hwepfungwa vaiva nehuwandu hwehupenyu hwemakore makumi mashanu.
Genetics uye Nhaka
Sezvakakonzerwa nekugadzirisa zvinokonzerwa nekugadzirisa zvinodhaka, FMF inowanikwa apo vabereki vaviri vasina chirwere chimwe nechimwe vanogovera gadziriro yakakwana kune vana vavo.
Vabereki vanoonekwa se "vatakuri" nokuti mumwe nomumwe ane imwe yepamusoro (yekare) kopi yejeni uye imwe yekare (mutated) kopi. Izvo chete kana munhu ane maitiro maviri akawandisa ayo FMF inogona kuitika.
Kana vabereki vose vari vatakuri, mwana ane 25 mukana wekodzero yekuwana maitiro maviri akawandisa (uye kuwana FMF), 50 muzana mukana wekuwana imwe yepamusoro uye imwe yezvejini yakawanda (uye kuva mutakuri), uye 25 muzana mukana wekuwana mbiri inowanzoita majini (uye inoramba isina kubatwa).
Nemhaka yokuti kune misiyano inopfuura 30 yeMEFV mutation, kusiyana kwakasiyana-siyana kunogona kupedzisa zvinoreva zvinhu zvakasiyana zvakasiyana. Mune zvimwe zviitiko, kuva nemaEVV maviri mutations kunogona kupa mhirizhonga uye yakawanda ye FMF. Mune vamwe, munhu angave ari kunyanya zviratidzo-asingasviki uye asina ruzivo rwezvakaitika pane dzimwe nguva, kusatsanangurwa musoro kana fivha.
Risk Factors
Sezvisina kufanana neF FMF iri munharaunda yevanhu vakawanda, kune mapoka umo FMF inowedzera yakawanda zvikuru. Iyo ngozi inonyanya kudziviswa kune iyo inonzi "muvambi wevanhu" umo mapoka anogona kutarisa midzi yechirwere kudzokera kune imwechete baba. Nokuda kwekushayikwa kwezvinhu zvakasiyana-siyana mukati memapoka aya (kazhinji nekuda kwekuroora kana kuroorwa kana tsika), zvimwe zviitiko zvisingawanzoitiki zviri nyore kubva kune rimwe chizvarwa kuenda kune rimwe rinotevera.
Kusiyana kwekuchinja kweMEFV kwakatorwa kumashure kusvika panguva dzeBhaibheri apo vafambisi vechikepe vekare vechiJudha vakatanga kutamira kubva kumaodzanyemba kweEurope kusvika kuNorth Africa uye kuMiddle East. Pakati pemapoka akawanda anowanzobatwa neFMF:
- VaJudha veSephardic , vane zvizvarwa zvavo vakadzingwa kubva kuSpain panguva yezana remakore rechi15, vane imwe mukana wechisere yekutakura MEFV gene uye imwe mu 250 mukana wokuwana chirwere ichi.
- Armenian mune imwe mukana yechinomwe yekutakura mutambo weMEFV uye mumwe muzana 500 ekusimbisa chirwere chacho.
- Turkis uye vanhu veArabhu vanewo pakati pe1 000 kusvika kune imwe mukana 2 000 yekuwana FMF.
Kusiyana neizvi, vaJudha veAshkenazi vane imwe mukana yechishanu yekutakura mutambo weMEFV asi imwe chete muhuwandu 73,000 yekuvandudza chirwere chacho.
Kuziva
Kuongororwa kwe FMF kunonyanya kubva pane zvakaitika uye muenzaniso wekurwiswa. Chinokosha pakuzivikanwa kwechirwere ndehurefu hwekurwiswa kusingawanzopfuura mazuva matatu.
Kuyera ropa kunogona kurairwa kuti uongorore rudzi uye chiyero chekuvhiringidzika kuripo. Izvi zvinosanganisira:
- Kuzara kweropa kuwanda (CBC) , kwaiwanzoona kuwedzerwa mumasero machena ekudzivirira
- Erythrocyte sedimentation rate (ESR) , yakashandiswa kuona kuora kwechirwere kana chirwere
- C-reactive protein (CRP) , yakashandiswa kuona kuvhara kwakanyanya
- Serum haptoglobin yaiwanzoona kana masero matsvuku eropa ari kuparadzwa sezvaanoitika nezvirwere zvinopisa
Muyeso unogona kuitwawo kuti uone kana pane albumin yakawandisa mumuguta, zvinoratidza kuora kwepfungwa kusingaperi.
Kubva pamigumisiro iyi, chiremba anogona kuraira chiedza chemajini kuti asimbise mutemo weMENFV. Mukuwedzera, chiremba angakurudzira kuedza kutsvaga umo mushonga unonzi metaraminol unogona kukurudzira chimiro chakaipa cheFMF, kazhinji mukati maawa makumi maviri ejoi. Chigumisiro chakanaka chinogona kupa chiremba unhu hwakanaka hwekuvimba mukuita FMF kuongorora.
Kurapa
Hakuchina mushonga weF FMF. Mishonga inonyanya kutungamirirwa nekutungamirirwa kwezviratidzo zvakakomba, kazhinji kacho nemishonga isingashandisi-kupisa zvinodhaka seVertaren (diclofenac) .
Kuti kuderedze kuoma kana kuwandisa kwekurwiswa, anti-gout drug Colcrys (colchicine) inowanzotaurwa sechimiro chechirwere chisingaperi. Vakuru vanowanzogadzirirwa 0.6-milligram tablet iyo inotorwa kaviri zuva rega rega.
Izvo zvinobudirira maColcrys mukurapa FMF iyo 75 muzana yevanotambura vanorondedzera kusadzokororazve kwezvirwere, asi 90 muzana vanorondedzera kuwedzerwa kwechiratidzo. Uyezve, kushandiswa kweColcrys kunoonekwa kuderedza zvikuru njodzi yeFFF zvinetso, kusanganisira kushaya mafungiro.
Colcrys inogona kushandiswawo kubata chirwere chakakomba, chinowanzopiwa sendima imwechete, 0.6-milligiramu inotorwa maawa ose emadhora mana, inotevera 0.6 milligrams inotorwa maawa maviri nemaawa maviri, uye inoguma ne 0.6 milligrams inotorwa maawa gumi nemana pamadhora mana .
Zvimwe zvikanganiso zveColcrys zvinosanganisira kuvhiringidzika kwemimba, chirwere chekushaya ropa , uye kuputika kweuropathy (a numbness kana pini-uye-nenyuchi kunzwa kwemaoko nemakumbo). Zviratidzo izvi zvinonyanya kudzivirirwa nokutora madhoma maduku. Kunyanyisa kwechirwere kunogona kukonzera kutyisa kwakakomba nezviratidzo zvekunyunyutira, kurutsa, chirwere, uye kurwadziwa mumimba.
Colcrys haigoni kushandiswa kune vanhu vane hosha dzisina kukodzera.
Shoko Rinobva
Kana uchitarisana nehutano hwakanaka hweHediterranean Mediterranean fever, zvakakosha kutaura nemumwe chiremba wezvirwere kuti unzwisise zvakakwana zvinoratidzirwa zvinorevei uye zvinosarudzwa nemishonga yako.
Kana zvakatemwa Colcrys, zvakakosha kutora mushonga wezuva nezuva sezvinorayirwa, kana kutamba kana kuwedzera kuwanda kwezvaunoita. Vanhu vanoramba vakagadzirira kurapwa vanogona kutarisira kuva neupenyu hwemazuva ose uye mararamiro ehupenyu.
Kunyangwe kana kurapwa kunotanga mushure mekunge chirwere chetsvo chave chichigadzirwa, kaviri-kushandiswa kwezuva rimwe kweColcrys kunogona kuwedzera kutarisira hupenyu zvakapfuura makore makumi mashanu akaonekwa muvanhu vane chirwere chisina kutenderwa.
> Sources:
> Fujikura, H. "Global epidemiology yeFamilial Mediterranean fever mutations kuburikidza nehuwandu hwevanhu." Mol Genet Genomic Med. 2015; 3 (4): 272-82.
> Padeh, S, uye Yakov, B. "Familial Mediterranean fever." Curr Opin Rheumatol . 2016; 28 (5): 523-29. DOI: 10.1097 / BOR.0000000000000315.
> Sonmez, H .; Batu, E ;; uye Ozen, S. "Chirongwa cheMediterranean Mediterranean: maonero azvino." J Inflamm Res. 2016; 9: 13-20.