Unoda kuziva nezveMarfan Syndrome here? Ngatikurukurei!

Wakamboona here kuti vamwe vanhu vakareba kupfuura vamwe, makumbo avo, minwe, nezvimwewo zvinoita sekunge zvakareba kupfuura zvakajairwa? Kana kuti vane chimiro chisina kujairika chechipfuva, kana kuti vane matambudziko ekuona? Dzimwe nguva, pamwe chete nezviratidzo izvi, panogona kuva nematambudziko emwoyo nemaziso. Ndizvo zvakaita Marfan Syndrome. Ichi chirwere chemajini. Usazvidya mwoyo, ngatitaurei nezvazvo zvakadzama.

Chii chinonzi Marfan Syndrome? Zvichitaurwa zviri nyore...

Zvichitaurwa zviri nyore, Marfan Syndrome chirwere chemajini chinokanganisa nhengo dzemuviri wedu. Zvino ungave uchishamisika kuti chii chinonzi nhengo dzemuviri. Funga nezvazvo, nhengo dzemuviri imhando yenhengo dzinobatanidza nhengo dzakasiyana dzemuviri wedu, dzakadai seganda, mapfupa, mitsipa yeropa, uye mavharuvhu emwoyo, uye zvinobatsira kuti dzive nesimba uye dzigone kuchinjika.

Mumunhu ane chirwere cheMarfan syndrome, nyama iyi inobatana haina simba, uye inonamira zvakanyanya . Yakaita sebhandi rerabha, asi haina simba rakawanda. Ndokusaka chirwere ichi chichigona kukanganisa masisitimu akasiyana mumuviri. Chinogona kukanganisa mwoyo, mitsipa yeropa, maziso, mapfupa nemajoini .

Vanachiremba vanoti izvi chirwere chemajini chine "kushanduka-shanduka kwezviratidzo." Izvi zvinoreva kuti havasi vese vane chirwere ichi vachava nezviratidzo zvakafanana. Vamwe vanhu vachava nezviratidzo zvishoma, nepo vamwe vachava nezvimwe zvishoma. Uye nguva inotora kuti zviratidzo zvioneke inogona kusiyana kubva pamunhu kusvika pamunhu.

Marfan syndrome chirwere chinowanikwa pakuzvarwa . Zvisinei, dzimwe nguva zviratidzo zvinoonekwa uye unogona kunge uri wechidiki kana kupfuura paunoonekwa kuti une chirwere ichi. Ichi chimwe chezvirwere zvinowanzo wanikwa nhaka. Chinokanganisa munhu mumwe chete pavanhu 3,000 kusvika 5,000.

Ndezvipi zviratidzo zveizvi?

Marfan syndrome ine zvinhu zviviri zvikuru. Chimwe i aortic root aneurysm (aortic root aneurysm) . Uku kuzvimba kana kuwedzera kwetsinga huru yeropa inotakura ropa kubva pamoyo wedu kuenda kumuviri (aorta). Chimwe i lenzi yeziso yakabviswa (ectopia lentis) .

Hunhu hukuru huviri uhwu hunogona kukonzera kuti uve nezviratidzo zvakaita seizvi:

• Kurova kwemoyo kunoreva kunzwa kwemoyo wako uchirova nekukurumidza uye chipfuva chako chichirova.
• Kunzwa moyo wako uchirova zvine ruzha uye nekukurumidza.
• Kurwadziwa kweziso.
• Kutadza kufema zvakanaka.
• Kuchinja kwekuona; semuenzaniso, astigmatism uye kuona zviri pedyo zvakanyanya.

Asi nekuda kwekuti Marfan syndrome inogona kukanganisa dzimwe nhengo dzemuviri, zvimwe zviratidzo zvinogona kuitika. Semuenzaniso, zviratidzo zvemuviri zvinogona kusanganisira:

• Chiso chingaratidzika sechakarebesa uye chakatetepa zvishoma.
• Maoko, makumbo, neminwe zvinoonekwa sezvirefu zvikuru kana tichienzanisa nedzimwe nhengo dzemuviri.
• Mazino akawandisa mazino anodhonzwa pamwe chete uye anoita seakaunganidzwa pamusoro pemamwe.
• Kurwara neScoliosis.
• Tsoka dzakati sandara.
• Kushaya simba uye nyore nyore kupatsanurwa kwemajoini.
• Mavanga ekutambanudza anoonekwa paganda kunyangwe huremu hwemuviri husingachinji zvakanyanya.
• Chipfuva chakanyura (pectus excavatum) kana chipfuva chakabudikira (pectus carinatum).
• Muviri murefu, mutete.

Ndezvipi zvinetso zvechirwere ichi?

Marfan syndrome inogona kukonzera matambudziko akasiyana-siyana anokanganisa mwoyo, maziso, nemapapu.

Matambudziko emwoyo netsinga ndiwo matambudziko anowanzoonekwa neMarfan syndrome. Izvi zvinogona kusanganisira:

• Kutsemuka kwetsinga yeropa: Uku kubvaruka kwemukati memadziro etsinga yeropa. Uku kunokonzerwa nedambudziko.
• Chirwere chevharuvhu yemwoyo: Marfan syndrome inogona kukonzera kuti nyama iri mumavharuvhu emwoyo isashande zvakanaka uye igone kuchinjika.
• Mwoyo wakakura: Nekufamba kwenguva, tsandanyama dzemwoyo wako dzinogona kukura uye kushaya simba.
• Kusashanda zvakanaka kwemoyo (Arrhythmia): Dambudziko iri rinowanzobatanidzwa ne mitral valve prolapse.
• Kubuda kweropa muuropi: Kubuda kwenzvimbo isina simba mutsinga yeropa iri muuropi kana kuti yakakomberedza.

Matambudziko emaziso anogona kusanganisira:

• Matumbu emusoro.
• Dambudziko reGlaucoma.
• Kubviswa kwe retinal.

Kuchinja kwenhengo dzemapapu kunokonzerwa neMarfan syndrome kunogona kuwedzera njodzi ye:

• Asima.
• Kubata kwemapapu.
• Chirwere chemapapu chinogara kwenguva refu (COPD).
• Mapapu akaputsika / pneumothorax.
• Emphysema.
• Chirwere chemabayo.

Chii chinokonzera Marfan syndrome?

Izvi zvinokonzerwa nekuchinja kwemajini . Kunyanya, shanduko mujini - jini reFBN1 - rinoudza masero edu kuti agadzire puroteni inonzi fibrillin . Iyi fibrillin chikamu chikuru che elastic fibers mumatishu edu anobatanidza.

Kazhinji, Marfan syndrome chirwere chinotorwa kubva kune mumwe wevabereki . Iyi inhaka inotonga autosomal.Chirwere chinogara nhaka. Kureva kuti, chirwere ichi chinogona kukonzerwa nekugara nhaka jini kubva kumubereki mumwe chete. Munhu ane Marfan syndrome ane mukana we50% wekutapurira chirwere ichi kumwana mumwe nemumwe.

Zvisinei, muzviitiko zvinenge 25%, chirwere ichi chinogona kuitika nekuda kwekuchinja kwemajini matsva (asina chikonzero chinogona kuzivikanwa), pasina nhoroondo yemhuri.

Vanachiremba vanoongorora sei Marfan syndrome?

Nekuti Marfan syndrome inokanganisa nhengo dzakasiyana dzemuviri, ungangoda rubatsiro rwechikwata chenyanzvi kuti uongorore chirwere chacho uye ugadzire hurongwa hwekurapa.

Kutanga, vanachiremba vanoita zvinhu izvi:

• Bvunza nezvenhoroondo yako yezvekurapa.
• Kuongororwa kwemuviri kuchaitwa kuti kuonekwe kana paine zviratidzo zvechirwere.
• Kubvunza nezvezviratidzo zvako.
• Bvunza kana paine munhu mumhuri akambova nematambudziko ehutano ane chekuita neMarfan syndrome.

Vanachiremba vanowanzo shandisa nzira dzinonzi "Ghent nosology" kuti vaongorore Marfan syndrome. Miedzo inotevera inogona kukurudzirwa kusimbisa chirwere kana kubvisa zvimwe zvirwere zvakafanana:

• Kuongororwa kweCT `(Computer tomography - CT scan)`.
• X-ray yechipfuva.
• Kuongororwa kweE.C.G. `(Electrocardiogram - ECG)`.
• Echocardiogram.
• Kuongororwa kweMRI (Magnetic resonance imaging - MRI)

Kuongororwa kwemajini (uku kuongororwa kweropa) kunogona kutsvaga shanduko mujini reFBN1, iro rinowanzo kukonzera Marfan syndrome. Zvisinei, mhedzisiro yekuongororwa kwemajini uku haisi pachena nguva dzose. Kuongororwa uku kunogona zvakare kutsvaga mamwe mamiriro emajini anokonzera zviratidzo zvakafanana, zvakaita seLoeys-Dietz syndrome.

Marfan syndrome inorapwa sei?

Hapana mushonga weMarfan syndrome. Zvisinei, kune nzira dzakasiyana-siyana dzekurapa dzinogona kukubatsira kudzora zviratidzo zvako uye kudzivirira matambudziko. Izvi zvinosanganisira:

• Mishonga.
• Kuongororwa kwechiremba nguva dzose.
• Kutungamirirwa kwemabasa emuviri.
• Kuvhiyiwa.

Unoda chirongwa chekurapa chakanangana nematambudziko ako ehutano.

Mishonga

Mimwe mishonga inogona kubatsira kudzivirira kana kudzora matambudziko:

• Beta-blockers: Izvi zvinodzivirira kana kunonotsa kukura kwetsinga huru dzeropa. Vanachiremba vanokurudzira kutanga kurapwa uku nekukurumidza sezvinobvira kune vanhu vane Marfan syndrome. Kana usingakwanise kutora izvi nekuda kwechirwere chakadai seasthma kana nekuda kwemigumisiro, chiremba wako anogona kukupa mushonga we calcium channel blocker .
• MaAngiotensin II receptor blockers (ARBs):Mishonga iyi inogonawo kubatsira kuderedza kukura kweura hukuru.

Kana wakavhiyiwa vharuvhu yemwoyo nekuda kweMarfan syndrome, uchafanira kutora mishonga inorwisa ropa rinopwanya ropa kweupenyu hwako hwese.

Kutarisirwa kwenguva dzose kwechiremba

Unofanira kugara uchiongororwa nachiremba kuti uwane zvinhu izvi:

• Mwoyo netsinga dzeropa - kunyanya saizi yediaphragm yako hombe.
• Maziso.
• Sisitimu yemafupa.

Nenzira iyi, timu yako yezvekurapa inogona kutarisa shanduko uye kuona chero matambudziko anogona kuitika nekukurumidza. Vachakuudza kuti unofanira kuuya kangani kunoongororwa izvi.

Kuongororwa uku kunowanzo sanganisira bvunzo dzemifananidzo dzakadai se:

• Kuongororwa kweCT.
• Kuongororwa kwemoyo (Echocardiograms).
• Kuongororwa kweMRI.

Kutungamirirwa kwemabasa emuviri

Kurovedza muviri kwakaoma uye kunorema kunogona kumanikidza diaphragm yako nedzimwe nhengo dzemuviri dzinobatwa neMarfan syndrome. Saka, unofanira kushanda nachiremba wezvekurovedza muviri kuti uwane maekisesaizi akachengeteka nemitambo yakakunakira.

Vanachiremba vanowanzokurudzira maekisesaizi ane simba rakaderera kusvika pakati nepakati. Zvisinei, kana wakambovhiyiwa pa mitral valve yako kana midzi yevharuvhu, ungangoda kurovedza muviri zvishoma nezvishoma.

Kazhinji, unofanira kudzivisa zvinhu zvakaita se:

• Mabasa anosanganisira kubata mweya wako uye kushanda nesimba (Valsalva maneuver).
• Bata mitambo.
• Kurovedza muviri kusvika kuneta.
• Maekisesaizi anosanganisira kubata nzvimbo imwe chete kwenguva yakareba, akadai semapuranga uye madziro, anonzi maekisesaizi e isometric.

Kuvhiyiwa kwemoyo

Zvinangwa zvikuru zvekuvhiyiwa kwemoyo kweMarfan syndrome ndezvekudzivirira vharuvhu yako yeaortic kuti isakure kana kuputika uye kurapa matambudziko evharuvhu. Iwe nechikwata chako chekurapa muchasarudza pamwe chete kana muchida kuvhiyiwa, mushure mekufunga nezvezvinhu zvakati wandei.

Aya ndiwo mavhiyiro anowanzoitwa eMarfan syndrome:

• Kugadziriswa kana kutsiviwa kwevharuvhu yeAortic.
• Kugadziriswa kweaneurysm yeaorta inokwira.
• Kugadziriswa kana kutsiva vharuvhu yeMitral.
• Kugadziriswa kweaorta yechipfuva.

Kana uchida kuvhiyiwa, edza kusarudza chipatara chikuru chine ruzivo nerudzi rwekuvhiyiwa kwauri kuita. Chikwata chako chekuvhiya chinofanirawo kunzwisisa Marfan syndrome zvakanaka.

Chii chaungatarisira kana uchirarama neMarfan syndrome?

Kana uine Marfan syndrome, uchafanirwa kugara uchisangana nachiremba uye kuziva nezvemuviri wako. Marfan syndrome haibate munhu wese nenzira imwe chete, saka rwendo rwako nechirwere ichi ruchava rwako wega. Chikwata chako chekurapa chichakubatsira kuti ugadzirire kuchinja kwechirwere chako.

Yeuka kuti hausi wega. Chikwata chekurapa chinogara chiri newe.

Nekuda kweruzivo rwakawanda nezveMarfan syndrome uye nzira dzakavandudzwa dzekurapa, vanhu vane Marfan syndrome vava kurarama kwenguva yakareba kupfuura zvavairarama makore ekuma1970 asati asvika. Nguva yekurarama yemunhu ane Marfan syndrome yave yakafanana neyemunhu asina chirwere ichi. Zvisinei, nguva yekurarama yevarume ipfupi zvikuru pane yevakadzi.

Chirwere chemwoyo chinoramba chiri chikonzero chikuru cherufu muMarfan syndrome. Izvi zvinonyanya kuitika kune vanhu vanofa kamwe kamwe kana chirwere ichi chikasazivikanwa. Njodzi yacho yakakurawo kune avo vanoongororwa chirwere ichi nguva isati yasvika.

Marfan syndrome nehutano hwepfungwa

Pane zvinhu zvakawanda zvinogona kukanganisa hutano hwepfungwa dzako uye hupenyu hwako kana uchirarama neMarfan syndrome. Semuenzaniso:

• Marfan syndrome chirwere chisingaperi uye chinoda kurapwa kwehupenyu hwese.
• Kukanganiswa kwakaitwa chirwere ichi nechitarisiko chako.
• Kurwadziwa kwenguva refu uye kuneta.
• Zvirambidzo pakuita mabasa emuviri (izvi zvinowanzo kanganisawo hukama hwevanhu).
• Kumanikidzwa kwekuronga mhuri.

Nekuda kweizvi, unogona kunge uri panjodzi huru yezvinhu zvakaita se:

• Kuzvidya mwoyo.
• Kuora mwoyo.
• Kushungurudzwa nevamwe.
• Kusasangana kwevanhu panzvimbo imwe.

Vachengeti vevana pamwe chete nevemhuri dzine vanhu vane Marfan syndrome vari panjodzi yekusangana nematambudziko aya epfungwa.

Hutano hwepfungwa dzako hwakakosha sehutano hwako hwemuviri.

Kana uri kunzwa kushushikana nekuda kweMarfan syndrome, usakanganwa kutsvaga rubatsiro kubva kune nyanzvi yehutano hwepfungwa, senge chiremba wepfungwa . Ungawanawo zvichibatsira kubatana neboka rekutsigirana.

Hupenyu neMarfan syndrome hunogona kunzwa semusuwo unotenderera wekuonana, kurapwa, uye shanduko dzemararamiro. Asi bvunzo yega yega uye kuonana kwega kwega kunokubatsira kudzivirira matambudziko anokonzerwa neMarfan syndrome uye kurarama hupenyu hwakanaka sezvinobvira. Chikwata chako chekurapa chinewe kuburikidza nezvose izvi. Wana rutsigiro rwavo nenhungamiro. Kana uchinzwa sekunge waremerwa nezvose izvi, tsvaga rubatsiro kubva kune nyanzvi yehutano hwepfungwa.

Zvinhu zvakakosha zvekurangarira (Mharidzo Yekuenda Kumba)

Zvakanaka, ngatitarisei zvimwe zvezvinhu zvakakosha zvaunofanira kurangarira kubva pane zvatataura nezvazvo:

• Marfan syndrome chirwere chinokonzerwa nemajini., inokanganisa nhengo dzemuviri wedu.
• Izvi zvinogona kukanganisa nzvimbo dzakasiyana-siyana dzakadai semwoyo, maziso, nemapfupa. Zvakakosha zvikuru kugara uchiongororwa nachiremba .
• Kunyange zvazvo pasina mushonga wakakwana weizvi, kune mishonga yakanaka yekudzora zviratidzo uye kudzivirira matambudziko.
• Tevedzera mirairo yachiremba wako nemazvo. Inwa mishonga yako nenguva uye ongororwa zvaunenge waudzwa.
• Unofanira kungwarira zvikuru pakuita mabasa emuviri. Bvunza chiremba wako kana murapi wemuviri kuti maekisesaizi api akakunakira.
• Chengetedzawo hutano hwepfungwa dzako. Usazeza kutsvaga rubatsiro kana uchida rubatsiro.
• Hausi wega, une timu yevanachiremba, mhuri, neshamwari kuti vakubatsire.

Ndinovimba ruzivo urwu ruchakubatsira. Kana muine mimwe mibvunzo, musazeza kubvunza chiremba wenyu.

Marfan Syndrome, chirwere chemajini, nhengo dzinobatana, chirwere chemwoyo, mapfupa, maziso, fibrillin