Amyloidosis imhuri yekushungurudzika umo mapuroteni asina kunaka, anonzi amyloid mapuroteni, akaiswa mumatunhu akasiyana-siyana. Izvi zvinyorwa zvinyorwa zvinogona kukanganisa zvakanyanya basa rinowanzoshandiswa nhengo dzomuviri.
Mumoyo wemamyloidosis, amyloid mapuroteni anoiswa mumoyo wemisungo. Izvo zvinyorwa zveamyloid zvinogadzirisa masvingo emisungo yemwoyo yakaoma , izvo zvinounza diastolic kusagadzikana .
Mukusagadzikana kwe diastolic, mwoyo haugone kupumha kazhinji mukati memwoyo wakarova, naizvozvo unoberepa neropa zvishoma zvakanyatsonaka. Mukuwedzera, ma-amyloid anoisa kuderedza kukwanisa kwemuviri wemwoyo kuti zvibvumirane kazhinji.
Nokudaro nemoyo wemamyloidosis, chirwere chemoyo chinokanganisa zvose panguva ye diastole (chikamu chekuzorora chekushaya moyo) uye systole (chikamu chekudzivirira chekushaya moyo) -pakati iyo mwoyo inorova sei .
Sekuguma kwezvinetso izvi zvose diastole uye systole, kukanganisa kwemwoyo kwakajairika nemoyo wemamyloidosis. Mukuwedzera, vanhu vane chirwere ichi vanowanzokura zvakanyanya kuora mwoyo kusagadzikana. Cardiac amyloidosis chirwere chakakomba zvikuru chinowanzoita kuti upenyu huvepo.
Chii Chinokonzera Amyloidosis?
Mamiriro ezvinhu mazhinji anogona kukonzera mapuroteni emamyloid kuti aunganidze mumatumbu. Izvi zvinosanganisira:
- Primary amyloidosis. Zita rokuti "primary amyloidosis" pakutanga raireva rudzi rweamyloidosis umo pasina chikonzero chinogona kuonekwa.Nhasi, primary amyloidosis inozivikanwa kuti inokonzerwa ne plasma cell disorder. (Masero eplasma ndiwo masero eropa anokonzera zvirwere ). , iyo inomirira myeloma yakawanda.Nokuti primary amyloidosis, chirongwa chisina kuchena chinowedzera chinonzi "amyloid light chain" kana kuti AL protini. Inenge 50 muzana yevanhu vane mwoyo yemyloidosis vane primary amyloidosis, neAl-type amyloid deposits. pamwe nemhando iyi yemamyloidosis yemwoyo kazhinji inowedzerawo ma-amyloid anoisa muitsvo dzavo, chiropa uye mumatumbo.
- Secondary amyloidosis Secondary amyloidosis inowanikwa kune vanhu vane chirwere chechirwere chechirwere chisingarapiki, kunyanya lupus , chirwere chinoputika chirwere , kana rheumatoid arthritis . Nemhaka yekusagadzikana kusingaperi, mari ye "amyloid type A protein" (inonziwo AA protein) inogona kuva yakawandisa. Secondary amyloidosis kazhinji inosanganisira itsvo, chiropa, spleen, uye lymph nodes. Secondary amyloidosis hazviwanzokanganisa mwoyo, zvisinei. Kunongova ne5 muzana chete yemoyo yemamyloidosis inokonzerwa nehomwe yeAA protein.
- Senile amyloidosis Senile amyloidosis inowana zita rayo kubva pane kuti nguva dzose inowanikwa mumavarume vakuru, kazhinji mune varume vanopfuura makore makumi manomwe. Muchiitiko ichi, kudarika zvakanyanya kunowanikwa kune protein inenge isiri yemazuva ose, inonzi TTR protein, inobudiswa muchiropa. Mu senile amyloidosis, chirongwa cheTTR chiripo chinowanzowanikwa mumoyo chete. Senile amyloidosis inokwana makumi mashanu nezana makumi mashanu ematambudziko emwoyo yemamyloidosis.
Zviratidzo zveCardiac Amyloidosis
Sezvakataurwa, moyo wamyloidosis unobata zvose kuzadzwa kwemwoyo uye kuputika kwemwoyo, saka haifaniri kushamisa kushanda kwemoyo wekare kunowedzera kuipa zvakanyanya.
Mhedzisiro yakakurumbira yemoyo yemamyloidosis inoshaya mwoyo. Izvozvi, zviratidzo zvekushaya kwemoyo-zvakanyanya kunyorwa dyspnea uye inokosha edema (kuzvimba) -icho chinowanzotungamirira kuongororwa kweamyloidosis.
Mumoyo wemamyloidosis inokonzerwa neApro protein (primary amyloidosis), nhengo dzomuviri dzinowanzobatwa mukuwedzera pamwoyo. Saka ava vanhu vanowanzova nezviratidzo zvepamuviri, zvakadai sokurasikirwa kwechido, kusangarika kwepakutanga, uye kuora kurasikirwa. Mukuwedzera, chirongwa cheA AL chinowedzera kuwedzera mumidziyo midzi yeropa, iyo inogona kukonzera kubvunda nyore, angina , kana kuti claudication (musuru inoputika nekuedza).
Vanhu vane moyo wamyloidosis vanonyanya kutarisana ne syncope (zvikamu zvekurasikirwa kwekuziva). Ne amyloidosis, syncope inogona kuva chiratidzo chinonyangadza, nokuti inowanzoratidza kuti mwoyo yevasimba yakave yakatambanudzwa zvishoma nezvishoma. Kunyanya, vanhu vane amyloidosis vanobata mwoyo yavo nemidziyo yeropa vangasakwanise kudzoka kubva kune chero chiitiko chinonyanya kuoma mwoyo yematambudziko, kunyange kwenguva pfupi. Chiitiko chakadaro chinogona kusanganisira chiitiko chevasovagal chinogona kungoita kuti nguva shomanana yezinzwa mune mumwe munhu.
Saka, apo rufu rwe kamwe kamwe runoitika kune vanhu vane mwoyo amyloidosis, kamwe kamwe kuora kwepfungwa kunowanzokonzera.
Izvi zvakasiyana zvakasiyana nevanhu vanoona kufa kamwe kamwe kubva kune mamwe marudzi ehutano hwemwoyo, umo vane chironga chemwoyo (zvikurukuru ventricular tachycardia kana ventricular fibrillation ) inenge nguva dzose inokonzera. Somugumisiro, kuisa chinokonzerwa nechinokonzerwa nekugadziriswa kwegadziriro mukati mevanhu vane moyo wamyloidosis kazhinji hachiregi kupona. Apo vanhu vane mwoyo weymy amyloidosis chiitiko syncope, njodzi yekufa kamwe kamwe mumwedzi inoverengeka inotevera yakakwirira.
Ne moyo weymy amyloidosis, zvinyorwa zveamyloid zvinowanzoitika mukati memwoyo wekugadzira magetsi. ( Verenga pamusoro pemutambo wekutengesa .) Muna senile amyloidosis, TTR-rudzi rweprotini inowanzoita kuti tive ne bradycardia inokosha (inononoka mwoyo rhythm), uye inoda kuiswa kwepermanem pacemaker . Zvisinei, neAl-type amyloidosis bradycardia haisati iripo, uye kazhinji haina kutungamirira pacemaker.
Vanhu vane moyo yemyloidosis vanowanzotanga kuisa ropa ropa nyore nyore, zvose mumidziyo yeropa uye mumoyo, zvichiita kuti kuwedzere kuwedzerwa ngozi yekurohwa uye thromboembolism .
Peripheral neuropathy inowanzova dambudziko muvanhu vane AL amyloidosis.
Ko Cardiac Amyloidosis Inowanikwa Sei?
Vanachiremba vanofanirwa kufunga nezvekugona kwepfungwa yemamyloidosis pose apo munhu ane mwoyo kusakundikana kwezvikonzero zvisingatsanangurwi, kunyanya kana dyspnea uye edema ndiyo inonyanyooneka zviratidzo.
Mune munhu ane kukundikana kwemoyo mutsva, kuvapo kweropa rakaderera, kuwedzera kwechiropa, chirwere chepuropa neuropathy, kana puroteni mumuguta, kunofanirawo kuisa pfungwa mupfungwa yekukwanisa kwemoyo wemamyloidosis.
Iko electrocardiogram mumoyo wemamyloidosis inogona kuratidza madhigirii (kureva kuti, chiratidzo chegetsi chidiki kupfuura zvisizvo), asi kazhinji kacho ndiyo echocardiogram inopa mhinduro dzakanakisisa kune zvakakodzera kuongororwa.
Ikopicardiogram kazhinji inoratidza kuwedzera kwemutsipa wemwoyo wezvinhu zvose zviviri. Mukuwedzera, i-amyloid inogadzika inowanzogadzira pamusana pekufananidzira kwechiratidzo chinhu chinopenya "chinopenya" kubva mukati memutsipa wemwoyo. Ropa rinovhara mukati memo rinoonekwawo zvakanaka nguva dzose.
Kunyange zvazvo echocardiogram inowanzoendesa nzira inoenda kuongororwa, kuvhara pasi kuongororwa kweamyloidosis kunoda bhiopsy yenyama inoratidza amyloid deposits. Mune vanhu vane AL amyloidosis, biopsy inogona kuwanikwa kubva kumafupa emimba kana kubva kumapfupa emapfupa. Zvisinei, chirwere chemwoyo chinodiwa neTTR amyloidosis (uye dzimwe nguva kunyange neAl amyloidosis). Mwoyo unofara unowanzoitwa kwete kwete, asingaiti, uchishandisa nzira yecatheter.
Ko Cardiac Amyloidosis Inotariswa Sei?
Kazhinji, mwoyo wamyloidosis ine maitiro akaipa. Zvisinei, mumakore achangobva kusvika maitiro matsva ekurapa akave akavakwa nokuda kwemoyo wemamyloidosis, uye vanhu vane chirwere ichi vane chikonzero chekunzwa tariro yakawanda kudarika iyo ingangodaro makore mashomanana adarika.
Kurapa kwepfungwa yemamyloidosis kunogona kuonekwa muzvikamu zviviri: kurapa kwekushaya mwoyo, uye kurapwa kwemamiriro ezvinhu ari kuunza amyloid deposits.
Kurapwa Kwemwoyo Kusakundikana
Kurapa kukanganisa kwemwoyo kunokonzerwa nemwoyo wemamyloidosis zvakasiyana nekurapa mwoyo kusakonzerwa kunokonzerwa nemamwe mamiriro. Kunyange zvazvo beta-blockers neACE inhibitors ndezvekugadzirisa zvirwere zvakasiyana-siyana zvekushaya simba kwemwoyo, zvinodhaka izvi (pamwe chete ne calcium-channel blockers ) zvinogona kuita kuti kushaya mwoyo kwema-amyloid kuwedzere. Izvi zvisingakwanisi kuita kuti kurapwa kwemoyo kusakonzera dambudziko guru muamyloidosis.
Kushandiswa kwe loop diuretics , yakadai sefurosemide (Lasix), ndiyo inonyanya kushandiswa mune zvechiremba mumoyo wemamyloidosis. Iyi mishonga inowanzobudirira mukuderedza idema yakaoma iyo inowanzotarisana nemamiriro ezvinhu aya, uye inokwanisawo kubvisa dyspnea (iyo imwe chiratidzo inowanzo) zvikuru. Loop diuretics inowanzoshandiswa mukukwirira kwepamusoro, uye inogona kupiwa intravenously kana ichidiwa.
Beta blockers haafaniri kushandiswa mumoyo wemamyloidosis. Iko kukwanisa kwepombi kukupura kwakanyanya zvishoma mumamiriro ezvinhu aya, uye panguva imwechete mwoyo haugoni kuzadza neropa zvakanyatsonaka. Somugumisiro, kuwedzerwa kwepfungwa yemwoyo kunofanirwa kuchengetedza chirwere chakakwana chemoyo mukati memwoyo wemamyloidosis. Izvi zvinoreva kuti beta blockers, nekudzikisa kutengesa kwemoyo, zvinogona kukonzera kuderedza kusinganzwisisiki kune vanhu ava. Calcium blockers inogonawo kuderedza mwoyo, uye inofanirawo kudziviswa.
Mune vanhu vane AL-type amyloidosis, ACE inhibitors inogona kuita kuti kuderedzwa kukuru (uye zvichida kunouraya) kuwedzere kweropa-zvichida nokuti mari yemamyloid mumatope emhepo inoita kuti mishonga yemasipirini isabhadhara kudonha mumhepo iyo ACE inhibitor inowanzokonzera. Iri doro rakanyanya muropa rinowanzoonekwa muvanhu vane TTR amyloidosis, uye kune ava vanhu ACE inhibitors vanogona kuedzwa nekuchenjerera.
Kuchinja kwemwoyo hakusi nzira yevanhu vane AL-type amyloidosis, nokuti kazhinji vane chirwere chikuru mune dzimwe nhengo dzakawanda. Apo vanhu vane TTR-type amyloidosis kazhinji vane zvirwere zvishoma kumwoyo, ivo vanowanzove vakwegura kuti varege kufungidzirwa sevakakodzeri vanoda kukanganisa mwoyo. Kudyara kunogona kuva sarudzo yevashoma vashoma vane TTR-rudzi rwemwoyo amyloidosis.
Kurapa kweDambudziko Rinokonzera Amyloidosis
Primary, AL-type Amyloidosis. Urwu rwemamyloidosis inowanzokonzerwa nemakumbo asingasviki emasero e plasma anobudisa zvakawanda zveA-type amyloid. Somugumisiro, mumakore achangopfuura chemotherapeutic regimens yakagadzirwa kuti iedze kuuraya chipfeko chisina kukodzera chemasero. High-dose melphalan inoteverwa nemafuta emapfupa kupemha ndiyo mushonga unonyanya kurudzirwa. Zvinosuruvarisa kuti, vanhu vane AL amyloidosis vane hupombwe hwemwoyo vanowanzove vasina hutano hwakakwana kuti vashandise rudzi urwu rwehutano hunotyisa. Mimwe chemotherapy inoshandiswa inogona kushandiswa muvanhu ava, zvisinei, uye zvingasarudzwa zvishoma zvinowanikwa mune vazhinji vavo. Kana AL amyloidosis inogona kuongororwa uye inoporesa isati yava yakawanda, mhinduro yakagadziriswa yakawanda.
Secondary Amyloidosis. Ndivo vashomanana chete vevanhu vane mwoyo yemyloidosis vane chiitiko ichi. Zvisinei, kushungurudzwa kwechisimba chechirwere chinokonzera kupisa kunogona kuderedza kufambira mberi kweamyloidosis.
Senile Amyloidosis. Muvanhu vane moyo wamyloidosis inokonzerwa neTTR amyloid, mapuroteni akawanda anogadzirwa muchiropa. Icho chinowedzera kuti TTR-type amyloidosis ndeyemhando mbiri. Mune imwe yemhando idzi, kusiyana kwakasiyana-siyana kunoonekwa nevanhu vaduku, chiropa chekushanda chinobvisa chinyorwa che TRR-type amyloid protein uye inogumisa kufambira mberi kweamyloidosis. Zvinosuruvarisa, kune vanhu vakwegura vane zvakanyanya kufanana, senile TTR-type amyloidosis, chiropa chekushanda hachibatsiri kufambira mberi kwechirwere.
Zvinodhaka zviri pasi pekutsvakurudza izvo zvine chinangwa che "kusimbisa" puroteni yeTTR kuitira kuti irege kuzogadzirisa sezvinoitwa amyloid. Zvisinei, inotanga kusvika kuti uzive kana madhiragi aya achanyatsogadzirisa zvakaguma zveTTR-rudzi rwemwoyo amyloidosis.
Pasi Pasi
Cardiac amyloidosis chirwere chakakomba chinokonzera zviratidzo zvikuru, uye zvinowedzera kuderedza upenyu hurefu. Mimwe mamiriro ezvinhu anokwanisa kubudisa amyloidosis, uye zvakanakisisa kurapwa-uye pane imwe nzvimbo kufungidzira - kunobva kune rudzi rweamyloid protein inowanikwa mumatumbu.
Pasinei nemashoko akaoma, kufambira mberi kwakanyanya kuri kuitwa mukunzwisisa marudzi akasiyana-siyana emoyo wemamyloidosis, uye mukunyengedza nzira dzakanakisisa dzokurapa kune mumwe nomumwe wavo.
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