Isifo Takayasu esithi: Iimpawu and Treatment

Kwiminyaka yakutshanje, uluntu zahlaziywa ngokupheleleyo isimo kwayo iintlobo ezahlukeneyo izifo. Abantu abaninzi namhlanje bakhetha indlela yokuphila esempilweni, zama ukutya ekunene, ukusuka imikhwa emibi. amanyathelo bakhonza nokuthintela izifo ezininzi. Ngelishwa, nkqu indlela eyiyo yokuphila ayikho 100% isiqinisekiso ukuba umntu bungasifumani naloo meko okanye loo sifo.

ulwazi jikelele

isifo Takayasu kaThixo - oku kakhulu umtshiso ngokunzulu endalo ezingapheliyo, nto leyo ibhiyozelwa nasezimpahleni yegazi enkulu. Owona msebenzi wabo kukudlulisela igazi ngokusuka entliziyweni. Kakade ke, ukungasebenzi kakuhle umzimba kuchaphazela umzimba wonke. Kungenjalo, esi sifo kuthiwa sisifo pulseless, Takayasu engqondweni okanye aortoarteritis nonspecific.

Nangokurhala ngcembe lo aorta kunye amasebe awo ngcembe ezonakeleyo umphezulu engaphakathi iimpahla ngokwabo. Ngenxa yoko, kukho ukuqina iqokobhe engundoqo. Kukho ukukhukuliseka phakathi agudileyo umaleko kwezihlunu. Le mithambo ye nangendlela ecwangciswe kakuhle ngokulandelelana aorta kubonakala granuloma, ezibandakanya isikakhulu iiseli ezinkulu. Zonke ezi nkqubo kunokubangela i Buze kunye nolwandiso isitya yegazi ngokwayo, ukwakha kwemithambo. Kwimeko nangakumbi ukuqhubeka kwesifo kufa Imicu ebizwa ngokuba yi-elastic. Ngenxa yoko, ngokuthe ngcembe ukuphazamiseka flow yegazi ibandakanya ischemia amalungu angaphakathi kunye namalaphu. Ngoko ezindongeni ezonakeleyo kwasekwa microthrombuses kunye umonakalo atherosclerotic.

isifo Takayasu kukaThixo ikakhulu olufunyanwa amantombazana kunye namabhinqa abaminyaka ukusuka kwiminyaka eli-15 ukuya kwengama-25. Xa amayeza, okwabizwa iimeko izifo kwizigulane eyindoda.

Iimpawu zokuqala zikholisa ukuvela phakathi kweminyaka eli-8-12. Kubalulekile ukuba uqaphele ukuba esi sifo isasazeka kakhulu eMzantsi Melika kunye mpuma Asia, kodwa amatyala Takayasu syndrome namhlanje kwakhona abhalwe kwimimandla ekwanti ngakumbi.

A incinane

Ngowe-1908, zamehlo ukususela Kwilizwe LaseJapan M. Takayasu wachaza utshintsho zophendlo lwezifo kwiretina ibhaqwe kwi iimviwo elilandelayo ibhinqa eliselula. Kulo nyaka, ezinye iingcali waseJapan iqaphele deformation efanayo ye fundus evela izigulane zabo, nokuba idibene nento ukunqongophala ekuthiwa pulsation kwemithambo iphiramidi. Kuyaphawuleka ukuba ibinzana elithi "isifo Takayasu kaThixo," waqalisa ukuba bathathe kusetyenziswa amayeza kuphela ngo-1952.

Izizathu

I-arhente etiologic kwesi sifo ngoku ayaziwa. Iingcali zithi sifo kunye lonke usulelo ezaziwayo streptococcal, ngokubonakalayo kuxoxwe ngendima Mycobacterium tuberculosis.

Namhlanje, izazinzulu zikholelwa ukuba umba liphezulu ekubunjweni ukuphazamiseka samathambo yi ukungalingani ekuthiwa okukwazi iseli-mediated. Kuyaphawuleka ukuba igazi izigulane, ngokuqhelekileyo ezinophawu ukwanda kwe T-lymphocyte CD4 +, yaye ukuncitshiswa T-lymphocyte CD8 +. Kwakhona, iingcali ukufumanisa ukukhula kwamanani esasaza izakhiwo omzimba, umsebenzi eyonyukileyo elastase kunye cathepsin G. ezizodwa

Njengoko zihlelwe aortoarteriit nonspecific?

isifo Takayasu, ezisekelwe phezu kwendlela ezakhiwe ngayo sisilonda esidumbileyoesingaphakathi, phantsi kwemiqathango ethile zohlulwa ngokwendidi eziliqela.

1. Uhlobo lokuqala. Wambetha arch aortic kwaye onke amasebe esukela kuyo.
2. Uhlobo lwesibini. Abachatshazelwa aorta esiswini kunye thoracic.
3. Uhlobo lwesithathu. Wambetha arch aortic edibene thoracic kunye esiswini.
4. Uhlobo lwesine. Isifo iquka pulmonary.

iimpawu

Kuqala kwinto zonke, izigulane ukuqala okhalaza ngakuthile imbonakalo uqaqanjelwa iingalo, buthathaka, ubuhlungu apha esifubeni kunye nentamo. Ngenxa yoko, kukho symptomatology uphawu ukuphazamiseka luvo. Umzekelo, saa ingqalelo, sinciphise komsebenzi, iingxaki memory.

Ukuba isifo linxulunyaniswa luvo optic, izigulane babone kukuhla kakhulu embonweni okanye ukungaboni (ngokuqhelekileyo iliso kuphela enye).

Ngenxa utshintsho kwimikhumbi esimilweni esimbaxa zophendlo lwezifo asakhasayo ekuthiwa insufficiency aortic. Le ngxaki ikhokelela iposi engabalulekanga kunye ukuphazamiseka kwi kwegazi sentliziyo.

Kunye iinguqu ezingephi ngqo kwi esiswini ukuhamba kwegazi aorta kuguga ngokubonakalayo kule imilenze xa ehamba, izigulane zifumana ubuhlungu kunye neentlungu.

Xa emathunjini Umthambo sezintso kwi umchamo ngethuba elilandelayo bafumana erythrocytes. Kwixesha elizayo, kuba amathuba liphezulu kakhulu zokuphuhlisa thrombosis kwemithambo kwezintso.

Kwimeko ukubandakanyeka kwinkqubo yokukrala pulmonary, ogulayo ukuphelelwa umoya, iintlungu esifubeni.

Amayeza namhlanje ezi zigaba ezimbini sifo: oyingozi futhi ezigulo ezondeleyo. Kubalulekile ukuba uqaphele ukuba kuphela ugqirha uyakwazi ukubonisa ngokuchanekileyo uguqulelo yangoku sifo. Le miqondiso kwezonyango ifomu etsolo inokuba Nedlac, oko ukuxilongwa ngexesha elichanekileyo kunokuba nzima kakhulu. Eyona nto kukuba wabonakala ezifanayo kunokwenzeka kuphela nesi sifo njengoko sifo Takayasu kaThixo.

Iimpawu kwixesha ludlule:

• komzimba;
• ndiyabila;
• ukwanda okuncinane kakhulu lobushushu emzimbeni;
• nokudinwa;
• ubuhlungu rheumatism kwamalunga.

Isigaba Chronic ngokuqhelekileyo eyenzeke kwiminyaka 6 ukususela ekuqaleni kwesi sifo. Lubonakala ngemiqondiso ezahlukeneyo clinical.

isifo Takayasu kaThixo esizibonakalisa ngohlobo yintloko rhoqo, ulungelelwaniso kakuhle, ubuhlungu ngethuba amalungu ezinkulu, ezinganyangekiyo buthathaka kwezihlunu.

ekuxilongweni

Qinisekisa phambi isifo abaqeqeshiweyo kuphela xa isigulane ezintathu kwezi mpawu zilandelayo:

• yokuduka ubume esandleni;
• yobudala ngaphezu kweminyaka engama-40;
• phakathi uxinzelelo lwegazi kwi umahluko imilenze eliphezulu ayikho ngaphantsi kwe-10 mm HG. Inqaku.;
• ingxolo ngaphakathi aorta;
• ukwanda ziqhubeke wegazi;
• kwaxhasa landa ESR.

Zonke ezi mpawu zingasentla ngokuqhelekileyo ibonisa isifo Takayasu kaThixo. Iimpawu ingahluka noko kwaye ziyahluka kwimeko nganye.

Kubalulekile ukuqaphela ukuba ugqirha kufuneka nakanjani atyumbe olongezelelweyo uviwo umzimba lomguli. Libonisa kwemichiza / ICBC, nto leyo evumela ukuba ukumisela ecaleni incinane kwisiqhelo, wayenza le sifo. Kwakhona uya kufuna angiography kunye ziphene umahluko. Olu viwo ekhethekileyo X-ray iimpahla, uvumela ukufumanisa endaweni exineneyo kwemithambo. Echocardiography kunika ithuba lokuba ukuvavanya umsebenzi intliziyo. Okubaluleke ngokufanayo, izitya ultrasound. Ngokusebenzisa ultrasound ugqirha ufumana umfanekiso opheleleyo imeko izitya coronary kunye Velocity ithombo legazi. Zonke ezi ndlela ingentla diagnostic zophando kuvumela ukuqinisekisa ubukho iingxaki ezifana aortoarteriit Takayasu.

Yintoni unyango kufuneka?

Ukuba uthathe ingqalelo thina into yokuba esi sifo sanda kakhulu ngexesha esakhula, unyango kusenokuba nzima ngakumbi. Ngokuqinisekileyo oko kufuna indlela ofanelekayo epheleleyo kunye nokuthobela imithetho ethile yothintelo. In ingakumbi iimeko ezinzima angafuna ungenelelo olukhawulezileyo yotyando.

unyango Takayasu kuthetha isicelo of imithi. Kuba nokuzinziswa xi esimbaxa, ngokuqhelekileyo owabelwe B-blocker yaye kuthiwa blocker calcium channel. Ukunciphisa amathuba egazi gazi izigulane Sicebisa ukuthatha anticoagulants (lithetha "lweeHedfowuni" kunye nabanye). Uthomalaliso kwesi sifo kwakhona kubandakanya ukusetyenziswa corticosteroids neziyobisi vasodilator (iziyobisi "prednisolone" kunye m. P.). Kubalulekile ukuba uqaphele ukuba wokugqibela kakhulu ekunciphiseni impendulo samathambo ngumzimba.

njani enye woyise sisifo Takayasu kaThixo? Unyango lwe sifo ngoku kusebenzisa ekuthiwa ulungiso igazi extracorporeal. Le nkqubo inzima kakhulu. Luquka ukhetho ezizodwa eziyila igazi zophendlo lwezifo ngqo kwaye ukumqumbisa sifo. Uqwalaselo ezi zinto lwenzeka ngaphandle emzimbeni wakhe.

unyango ngotyando

Ngokutsho iingcali, unyango elondoloza akusoloko kusenzeka ukuba boyise arteritis Takayasu kaThixo. Iimpawu ekwazile ukuyeka nje ixeshana. Kakade ke, imilinganiselo efana ngokwaneleyo, kungekudala zize likakade. Kungenxa yoko le nto, kwezinye iimeko, oogqirha Siyaleza ngamandla surgery, ibuyisela ngokupheleleyo patency yesingqengqelo nemithambo.

Yayibubungqina eziphambili kutyando:

• hypertension phezu imvelaphi renovascular syndrome;
• ekuphazamiseni le aorta;
• ingozi ischemia intliziyo.

Ukuba sithethe iinkqubo zotyando eziqhubekayo, kodwa amaxesha amaninzi zimelwe resection yabantu abangakwaziyo zophendlo lwezifo le aorta, esa nemithambo kunye endarterectomy. Xa indlela ofanelekayo, isigulane phantse lonke belibala yintoni arteritis Takayasu kaThixo.

ukuvimbela

Nawuphi ngezifo, njengoko siyazi, zinokuthintelwa, kwaye sifo, wayexakekile. amanyathelo azizithintelo ukuthini xa unyango ngokukhawuleza i kwezifo ezosulelayo kunye nohlobo wentsholongwane egazini (pharyngitis, tonsils, pyelonephritis kunye t. D.). Ngokutsho kweengcali, ezi zifo ezondeleyo iya isiba isiseko kakuhle ukwenzela uphuhliso Takayasu engqondweni, ngoko izigulane basengozini.

Kunyanzelekile ukuba ukomeleza amajoni omzimba kulo nyaka. Ukulungiselela le njongo, iingcali Sicebisa kuthatha kwizakhiwo afikelelekayo, nidle okulungileyo, umthambo.

Outlook

unyango ezaneleyo ngexesha kunciphisa kakhulu ingozi yokufa. Ngaphezu koko, ama-90% ezigulane unyango abanobuchule ixesha ubomi malunga ne-15 iminyaka.

Ke ukuba iingxaki, isikakhulu nobangela wokufa iingozi cerebrovascular (50%) kunye iposi engabalulekanga (25%).

isiphelo

Izigulane ziye ukuba unesifo arteritis Takayasu kaThixo, kufuneka siqonde ukuba le ngxaki ifuna unyango lwexesha elide kunye zinjalo zonke iingcebiso kagqirha. Kungenjalo, izihlandlo eziliqela umngcipheko ukunyuka nesifo sentliziyo.

Ukuze ube unyango kakhulu, zonke izigulane ngaphandle kokukhetha, kufuneka bangenele rhoqo uvavanyo lonyango kwaye uthabathe cazululo ezintsonkothileyo.

Ngelishwa, ukuba balahle esi sifo akwenzeki. Nangona kunjalo, ukusetyenziswa kweziyobisi kuvumela ukudlulisela isigulo ibe kuxolelwe, nto leyo evumela izigulane ukuba baphile ubomi obuqhelekileyo, ngaphandle abafumana ubuhlungu kwaye hayi ukuba bacinge malunga ngxaki ezifana nesifo Takayasu kaThixo. izigulane Photo kubonisa ngokucacileyo le ngxelo.

kakhulu therapy impumelelo ixhomekeke umsebenzi inkqubo zophendlo lwezifo kunye ubukho iingxaki. Ukongeza, i-ngaphambili aya kwafunyaniswa ngokuchanekileyo, i-ALS kuba nethemba. Ukuba uyakholwa iingcaphephe, ukulwa esi sifo lunokwenzeka kwaye oko kuyimfuneko.