I-Amyloidosis ngumndeni wezinkinga lapho amaprotheni angavamile, abizwa ngokuthi ama-amyloid amaprotheni, afakwa ezinhlobonhlobo ezihlukahlukene. Lezi zimali ezingenayo i-amyloid zingaphazamisa kakhulu ukusebenza okuvamile kwezitho zomzimba.
E-amyloidosis yesifo senhliziyo, amaprotheni a-amyloid afakwa emzimbeni wenhliziyo. Ama-amyloid deposits enza izintambo ze-muscular zenhliziyo ezinzima , eziveza ukungasebenzi kahle kwe-diastolic .
E-dysfunction i-diastolic, inhliziyo ayikwazi ukuphumula ngokujwayelekile phakathi kwezinyosi zenhliziyo, ngakho igcwalisa ngegazi kancane kancane. Ngaphezu kwalokho, i-amyloid deposits iyanciphisa ikhono lezinhlanzi zenhliziyo ukuba livumelane ngokujwayelekile.
Ngakho nge-amyloidosis yesifo senhliziyo, umsebenzi wezinhliziyo uyathinteka kokubili ngesikhathi se-diastole (isigaba sokuphumula sokushaya kwenhliziyo) kanye ne-systole (isigaba sokuqhafaza kwenhliziyo) -kuthi inhliziyo ishaya kanjani .
Ngenxa yalezi zinkinga zombili i-diastole ne-systole, ukuhluleka kwenhliziyo kuvamile nge-amyloidosis yenhliziyo. Ngaphezu kwalokho, abantu abanalo mkhuba bathambekele ekuthuthukiseni ukungazinzi kwenhliziyo. I-amyloidosis ye-cardiac yisimo esibi kakhulu esivamise ukunciphisa isikhathi eside sokuphila.
Yini Ebangelwa Ama-Amyloidosis?
Izimo eziningana zingabangela amaprotheni ama-amyloid ukuqoqa kwezicubu. Lokhu kufaka:
- Amyloidosis eyinhloko. Igama elithi "amyloidosis eyinhloko" ekuqaleni libhekisela kuhlobo lwe-amyloidosis lapho kungekho khona imbangela engabonakala khona. Namuhla, i-amyloidosis eyinhloko iyaziwa ngenxa ye-plasma cell disorder. (Ama-Plasma amangqamuzana egazi amhlophe aveza ama-antibodies ). , uhlobo lwama- myeloma amaningi . I-amyloidosis eyinhloko, iphrotheni engavamile eqoqayo ibizwa ngokuthi "i-amyloid light chain" noma i-AL protein. Amaphesenti angaba ngu-50 abantu abane-heart amyloidosis bane-primary amyloidosis, ne-AL-type amyloid deposits. naloluhlobo lwama-amyloidosis enhliziyo ngokuvamile lubuye luhlakulele ama-amyloid deposits ezinso zabo, isibindi namathumbu.
- I-amyloidosis yesibili I-amyloidosis yesibili ivela kubantu abanezinhlobo ezithile zezifo ezingapheli ezivuvukala, ikakhulukazi i- lupus , isifo sofuba , noma isifo samathambo . Ngenxa yokuvuvukala okungapheli, idiphozi "ye-amyloid hlobo A protein" (ebizwa nangokuthi i-AA protein) ingaba ngokweqile. I-amyloidosis yesibili ngokuvamile ihilela izinso, isibindi, i-spleen, nama-lymph nodes. I-amyloidosis yesibili ayithinti inhliziyo, noma kunjalo. Amaphesenti angama-5 kuphela e-amyloidosis enhliziyo abangelwa yi-deposit of AA protein.
- I-Senile amyloidosis I- Senile amyloidosis ithola igama layo lokuthi cishe njalo ibonakala emadodeni amadala, kaningi emadodeni angaphezu kweminyaka engu-70 ubudala. Kulesi simo, i-deposit excessively eyenziwa ngeproteinyini evamile, ebizwa ngokuthi i-TTR protein, eyenziwa esibindi. Ku-senile amyloidosis, i-protein ye-TTR igcina itholakala kuphela enhliziyweni. I-Senile amyloidosis ilandisa cishe ngamaphesenti angu-45 amacala e-amyloidosis enhliziyo.
Izimpawu ze-Cardiac Amyloidosis
Njengoba kukhonjisiwe, i-amyloidosis ye-heart ithinta kokubili ukugcwala kwenhliziyo nokushaya kwenhliziyo, ngakho akufanele kusimangaze umsebenzi jikelele wezinhliziyo uvame ukuwohloka kakhulu.
Umphumela ovelele kakhulu we-amyloidosis enhliziyo ukuhluleka kwenhliziyo. Eqinisweni, izimpawu zokuhluleka kwenhliziyo-ikakhulukazi i- dyspnea ne-edema ephawulekayo (ukuvuvukala) -yinto evame ukuholela ekuxilongweni kwe-amyloidosis.
E-amyloidosis ye-cardiac ebangelwa i-pro protein (primary amyloidosis), izitho zangasese zivame ukuthinteka ngaphezu kwenhliziyo. Ngakho laba bantu bavame ukuba nezimpawu zamathunjini, njengokulahlekelwa yidla, ukudlala okuncane, nokulahlekelwa isisindo. Ngaphezu kwalokho, i-pro protein i-AL ibuye iqoqwe emithanjeni encane yegazi, engabangela ukulimala, i- angina , noma i- claudication (ubuhlungu be-muscle nokuzikhandla).
Abantu abane-amyloidosis ye-cardiac bajwayele ukuvumelanisa syncope (iziqephu zokulahlekelwa kolwazi). Nge-amyloidosis, i-syncope ingaba uphawu oluvuthayo, ngoba luvame ukubonisa ukuthi ukugcinwa kwemisipha yempilo kuye kwasulwa cishe ngaphesheya kwemingcele yayo. Ngokuqondile, abantu abane-amyloidosis ethinta inhliziyo yabo nemithambo yegazi angeke bakwazi ukubuyisela kunoma imuphi umcimbi obhekene nenselele kakhulu ohlelweni lwe-cardiovascular, ngisho nakancane. Umcimbi onjalo ungabandakanya isiqephu se-vasovagal esingase sibangele izikhathi ezimbalwa zokuzondla komunye umuntu.
Ngakho-ke, lapho ukufa okungazelelwe kwenzeka kubantu abane-heart amyloidosis, ukuwa kwe-cardiovascular suddenly is a cause.
Lokhu kuhluke kakhulu kubantu ababhekana nokufa okuzumayo kwezinye izinhlobo zesifo senhliziyo, okungahle kube khona isimiso se- cardiac arrhythmia (ikakhulukazi i- ventricular tachycardia noma i- ventricular fibrillation ) cishe njalo imbangela. Ngenxa yalokho, ukufakela i-defibrillator engasetshenziselwa abantu abane-cardioactive amyloidosis ngokuvamile akulondolozi ukusinda. Lapho abantu abane-heart syndrome amyloidosis isipiliyoni syncope, ingozi ukufa ngokuzumayo ezinyangeni eziningana eziningana ziphakeme.
Ngama-amyloidosis enhliziyo, ama-amyloid deposits ngokuvamile avela ngaphakathi kwenqubo yokuqhutshwa kagesi kagesi. ( Funda mayelana nesistimu yokuqhuba .) Ku-senile amyloidosis, uhlobo lwe-TTR lwama-protein alondoloza ngokuvamile luholela ebhokraridi ephawulekayo (isigqi senhliziyo esincane), futhi sidinga ukumiswa kwe- pacemaker engunaphakade. Kodwa-ke, nge-AL-type amyloidosis bradycardia akuvamile, futhi ngokuvamile akuholeli ku-pacemaker.
Abantu abane-amyloidosis ye-cardiac ngokuvamile bavame ukwenza ama-blood clots kalula, kokubili emithanjeni yegazi nasenhliziyweni, okuholela engozini enkulu kakhulu yokushaywa isisu kanye ne- thromboembolism .
I-neuropathy ye-peripheral nayo yinkinga ejwayelekile kubantu abane-AL amyloidosis.
I-Cardiac Amyloidosis Ithola Kanjani?
Odokotela kufanele bacabange ukuthi kungenzeka ukuthi inhliziyo i-amyloidosis yenhliziyo noma nini lapho umuntu enesifo senhliziyo ngenxa yezizathu ezingalindelekile, ikakhulukazi uma i-dyspnea ne-edema yizimpawu ezivelele kakhulu.
Kumuntu onokuhluleka kwenhliziyo entsha, ukutholakala komfutho wegazi ophansi, isibindi esikhulu, i-peripheral neuropathy, noma amaprotheni emcimbini, kufanele futhi kukhumbuze ukuthi kungenzeka ukuthi i-amyloidosis ye-cardiac.
I- electrocardiogram in amyloidosis yenhliziyo ingabonisa ukushisa okuphansi (okungukuthi, isibonakaliso kagesi sincane ngaphezu kwesivamile), kodwa ngokuvamile i-echocardiogram ehlinzeka ngamagama angcono kakhulu ekutholeni ukuxilongwa okulungile.
Ngokuvamile i- echocardiogram ibonisa ukuqina kwensimu yezinhliziyo zombili zomzimba. Ngaphezu kwalokho, i-amyloid ibeka ngokuzenzakalelayo imidwebo ye-echo "ukubukeka" okuhlukile okuvela ngaphakathi kwesisu senhliziyo. Ama-clots egazini enhliziyweni abonakala njalo njalo.
Ngesikhathi i-echocardiogram ivame ukuholela endleleni eya ekuxilongweni, ukuphazamisa ukuxilongwa kwe-amyloidosis kudinga i-biopsy yezicubu ezibonisa ama-amyloid deposits. Kubantu abane-AL amyloidosis, i-biopsy ingatholakala emanzini omzimba noma emathunjini e-bone. Noma kunjalo, i-biopsy yenhliziyo iyadingeka nge-TTR amyloidosis (futhi ngezinye izikhathi ngisho ne-AL amyloidosis). Inhliziyo ye-biopsy ivame ukwenza ngokungajwayelekile, esebenzisa inqubo ye-catheter.
I-Cardiac Amyloidosis Injani?
Ngokuvamile, i-amyloidosis yesifo sinempilo embi. Kodwa-ke, eminyakeni yamuva nje izindlela ezintsha zokwelashwa zenzelwe i-amyloidosis yenhliziyo, futhi abantu abanezimo banezizathu zokuzizwa benethemba elithile kunalokho okungenzeka babe eminyakeni embalwa eyedlule.
Ukwelashwa kwe-heart amyloidosis kungacatshangelwa ezingxenyeni ezimbili: ukwelashwa kwenhliziyo ukuhluleka, nokwelashwa kwesimo esibucayi esikhiqiza i-amyloid deposits.
Ukwelashwa Kwehluleka Inhliziyo
Ukwelapha ukuhluleka kwenhliziyo okubangelwa i-amyloidosis yenhliziyo kuhluke kakhulu ekuphatheni ukuhluleka kwenhliziyo okubangelwa ezinye izimo. Nakuba abakwa- beta-blockers kanye ne- ACE inhibitors kuyizinto eziyinhloko zokwelapha izinhlobo eziningi zokwehluleka kwenhliziyo, lezi zidakamizwa (kanye nabavimbeli be-calcium-channel ) zingenza ukuhluleka kwenhliziyo kwe-amyloid kubi nakakhulu. Lezi zimingcele zenza ukwelashwa kokuhluleka kwenhliziyo kuyinselele enkulu ku-amyloidosis.
Ukusetshenziswa kwama- diuretics e-loop , afana ne-furosemide (Lasix), yiyona ndlela esemqoka yokwelapha kwezokwelapha e-amyloidosis yenhliziyo. Lezi zidakamizwa ngokuvamile ziphumelela kakhulu ekunciphiseni i-edema enamandla kakhulu evame ukuhambisana nalesi simo, futhi ingakwazi ukukhulula i-dyspnea (enye isifo esivamile) kakhulu. I-Loop diuretics ivame ukusetshenziselwa ukulinganisa okuphezulu, futhi inganikezwa nge-intravenously uma kudingeka.
Ama-blockers a-Beta akufanele asetshenziswe ku-amyloidosis yenhliziyo. Ikhono lenhliziyo lokupompa lilinganiselwe kakhulu kule nkinga, futhi ngesikhathi esifanayo inhliziyo ayikwazi ukugcwalisa ngegazi kahle kakhulu. Ngenxa yalokho, izinga lokushaya kwenhliziyo liyadingeka ukuze kugcinwe ukwanele okwenziwe inhliziyo enhliziyweni yamyloidosis. Lokhu kusho ukuthi ukuvimbela i-beta, ngokunciphisa izinga lokushaya kwenhliziyo, kungabangela ukuchithwa okungazelelwe kwalaba bantu. Abavimbela i-calcium nabo banganciphisa izinga lokushaya kwenhliziyo, futhi kufanele futhi kugwenywe.
Kubantu abane-AL-type amyloidosis, i-ACE inhibitors ingaveza ukunciphisa okujulile (futhi okungenzeka kubulale) ekucindezelekeni kwegazi-mhlawumbe ngoba idiphozi ye-amyloid emithanjeni yomzimba igcina isimiso sezinsiza ekukhokheleni ukwehla kwezingcindezi lapho i-ACE inhibitors ivame ukudala khona. Lokhu kwehla okukhulu komfutho wegazi ngokuvamile akubonwanga kubantu abane-TTR amyloidosis, futhi kulaba bantu abathile be-ACE inhibitors bangalingwa ngokucophelela.
Ukufakelwa kwenhliziyo akuyona inketho kubantu abane-AL-type amyloidosis, ngoba ngokuvamile banesifo esibalulekile kwezinye izitho eziningana. Ngenkathi abantu abane-TTR-uhlobo lwe-amyloidosis ngokuvamile benesifo esinqunyiwe enhliziyweni, ngokuvamile badala kakhulu ukuthi bangabhekwa njengabantu abafanelekile ukufakelwa kwe-cardiac. Ukufakelwa kwezitshalo kungase kube inketho kumuntu omncane ongavamile ophethe i-TTR-uhlobo lwe-amyloidosis yomzimba.
Ukwelashwa Kwe-Disorder Kubangelwa i-Amyloidosis
Primary, AL-uhlobo u-Amyloidosis. Lolu hlobo lwe-amyloidosis luvame ukubangelwa ukukhanya okungavamile kwamaseli e-plasma okwenza ubuningi be-AL-type amyloid. Ngenxa yalokho, eminyakeni yamuva iminyango ye-chemotherapeutic iye yasungulwa ukuze izame ukubulala i-clone engavamile yamaseli. I-high-dose melphalan elandelwa ukufakelwa komnkantsha wamathambo yilona ukwelashwa okuvame ukuphakanyiswa. Ngeshwa, abantu abane-AL amyloidosis abanesifo senhliziyo abavame ukuphilile ngokwanele ukubekezelela lolu hlobo lwezokwelapha. Amanye amakhemikhali e-chemotherapy angasetshenziswa kulaba bantu, kodwa, okungenani impendulo eyingxenye ibonakala kuningi labo. Uma i-AL amyloidosis ingatholakala futhi iphathwe ngaphambi kokuba ibe yinkimbinkimbi, umphumela othuthukisiwe kungenzeka kakhulu.
I-Amyloidosis yesibili. Kuyingcosana yabantu abancane abane-heart amyloidosis abanesi simo. Kodwa-ke, ukwelashwa okunamandla kwesifo sokuvuvukala esingaphansi kunganciphisa ukuqhubeka kwe-amyloidosis.
Senile Amyloidosis. Kubantu abane-heart amyloidosis obangelwa yi-TTR amyloid, amaprotheni amaningi ngokweqile akhiwa esibindi. Kuvela ukuthi i-TTR-uhlobo lwama-amyloidosis lunezinhlobo ezimbili. Ngenye yalezi zinhlobo, ezihlukahlukene ezingavamile ezibonakala kubantu abasha, ukufakelwa kwesibindi kususa umthombo we-TRR-type amyloid iphrotheni futhi kumisa ukuqhubeka kwe-amyloidosis. Ngeshwa, kubantu asebekhulile abanesifo esivamile, i-TTR-type amyloidosis, isibalo sokufakelwa kwesibindi asithinti ukuqhubeka kwesifo.
Izidakamizwa zingaphansi kophenyo olwenzelwe "ukuqiniswa" kweprotheni ye-TTR ukuze ingabe isakwazi ukuqongelela njengama-deposy amyloid. Kodwa-ke, kusencane kakhulu ukuthi ukwazi ukuthi lezi zidakamizwa zizokwenza ngcono yini umphumela we-TTR-uhlobo lwe-amyloidosis yomzimba.
Ngaphansi
I-amyloidosis ye-cardiac yisimo esibi kakhulu esibangela izimpawu ezibalulekile, futhi kunciphise kakhulu impilo ende. Izimo eziningana ezingaphansi zingenza i-amyloidosis, futhi ukwelashwa okungcono-futhi ngezinga elithile ukubikezela-kuhlukahluka nohlobo lwamaprotheni anamloyi afakwa emahlakheni.
Naphezu kwalezi zici ezibuhlungu, kwenziwa inqubekela phambili enkulu ekuqondeni izinhlobo ezahlukene ze-amyloidosis yomzimba, nasekukhatheni amasu okuphatha ngendlela efanele ngayinye yazo.
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> Dubrey SW, Hawkins PN, iFalk RH. Ama-Amyloid Izifo Zenhliziyo: Ukuhlola, Ukuxilongwa, nokudluliselwa. Inhliziyo 2011; 97:75.
> Kristen AV, uDengler TJ, u-Hegenbart U, et al. Ukufakelwa kwe-Prophylactic ye-Cardioverter-defibrillator ezigulini ezine-Cardiac Amyloidosis enomzimba kanye neNgcono Ephezulu Yokufa Kwegazi Okungazelelwe. I-Heart Rhythm 2008; 5: 235.
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