Ngatidzidzei nezveTransthyretin Amyloidosis (ATTR-CM), iyo inokanganisa mwoyo wako chinyararire.

Dzimwe nguva unonzwa sekuti pane chimwe chinhu chinoshamisa kana chakasiyana nezvemoyo wako here? Zvingave zvisiri nyaya hombe, asi zvinogona kuva dambudziko kana zvikasaonekwa zvakanaka. Nhasi tichataura nezvechirwere chemwoyo chakaoma zvishoma, asi chakakosha kuziva nezvemunhu wese. Ndiyo transthyretin amyloidosis, kana sekudanwa kwazvinoitwa navanachiremba, `(ATTR-CM)`.

Chii chinonzi transthyretin amyloidosis (ATTR-CM)?

Zvichitaurwa zviri nyore, `(ATTR-CM)` chirwere chinokanganisa mwoyo, icho chine chekuita neprotein iri mumuviri wedu. Ngatitsanangurei izvi zvishoma, handiti?

Chii chinonzi transthyretin (TTR)?

Transthyretin, kana kuti `(TTR)`, ipuroteni inokosha inowanikwa muropa redu, inonyanya kugadzirwa nechiropa chedu. Inoshanda sebasa rekutumira zvinhu mumuviri wedu. Ndiyo puroteni iyi `(TTR)` inotakura vhitamini A (inozivikanwawo seretinol) nehomoni thyroxine, inogadzirwa negland re thyroid, kuenda kunzvimbo dzakasiyana dzemuviri, kwadzinodiwa.

Chii chinonzi Amyloidosis?

Amyloidosis kuunganidzwa kwemapuroteni asina chimiro chakajairwa munhengo dzakasiyana dzemuviri wedu. Funga nezvazvo semurwi wezvinhu zvisina basa mumba medu. Kana mapuroteni akaiswa nenzira iyi mumoyo, tinoidaidza kuti cardiac amyloidosis.

Mapuroteni aya asina kujairika, semabhora madiki eshinda, anoumba maumbirwo eshinda anonzi 'fibrils' . Aya 'fibrils' anoguma ari mumoyo, kunyanya mu ventricle yekuruboshwe, iyo huru inopomba ropa kumuviri wese. Izvi zvinoita kuti moyo uome zvishoma nezvishoma. Pane kuva bhora rakapfava rerabha, rinoita sebhora rakaoma, rine matombo. Izvi zvinoita kuti zviomere moyo kudzika zvakanaka uye kupomba ropa.

Chii chinonzi Cardiomyopathy (CM)?

Kana moyo wakaoma uye wakaoma, tsandanyama yemoyo inorwara. Izvi ndizvo zvatinodaidza kuti cardiomyopathy (CM) mukurapa. Zvichitaurwa zviri nyore, tsandanyama yemoyo inoshaya simba. Izvi zvinoita kuti mwoyo usakwanise kupomba ropa rakakwana mumuviri. Izvi zvinowanzo tungamira kuchirwere chakakomba chinonzi heart failure.

Yeukai, pamusoro pechirwere ichi chinonzi `(ATTR-CM)`, kunewo rudzi rweamyloidosis inokonzerwa neimwe puroteni inonzi `(light chain)`. Inonzi `(AL) amyloidosis`. Ichirwere chakasiyana ne`(ATTR-CM)` chatiri kutaura nezvacho nhasi.

Chirwere ichi (ATTR-CM) chinozivikanwawo nemamwe mazita akati wandei. Semuenzaniso, unogona kunzwa chichinzi cardiac amyloidosis, amyloidosis ATTR, kana transthyretin cardiac amyloidosis kubva kuna vanachiremba kana kune dzimwe nzvimbo.

Ndedzipi mhando huru dze `(ATTR-CM)`?

Kune mhando mbiri huru dzechirwere ichi chinonzi "(ATTR-CM)``.

1.ATTR-CM yemhuri/yenhaka:

Rudzi urwu rumwe chete. Zvichitaurwa zviri nyore, runopfuudzwa kubva kuchizvarwa kuenda kune chimwe chizvarwa . Rudzi urwu runokonzerwa nekuchinja kwemajini edu, kushanduka kwemajini. Tinoti izvi ndezvemhuri kana kuti zvenhaka zveATTR-CM. Mumamiriro ezvinhu aya, mapuroteni asina kujairika anogona kuiswa kwete mumoyo chete, asiwo mutsinga uye dzimwe nguva muitsvo. Kune kushanduka kwemajini kwakasiyana kunokanganisa izvi pakati pemapoka akasiyana emarudzi pasi rose.

2. ATTR-CM yemhando yemusango:

Rumwe rudzi irworwo rwemhuka dzesango (wild-type ATTR-CM). Hapana chikonzero chaicho chemajini chakawanikwa cheizvi. Zvinongoerekana zvaitika, pasina chikonzero chakajeka. Rudzi urwu runonyanya kukanganisa mwoyo netsinga.

Chirwere ichi che `(ATTR-CM)` chakajairika zvakadii?

Kutaura chokwadi, kunyangwe nyanzvi dzezvekurapa hadzigone kutaura kuti vangani vane chirwere ichi che '(ATTR-CM)'. Zvisinei, zvinofungidzirwa kuti chirwere ichi chingave chakajairika munharaunda kupfuura zvinofungwa pari zvino . Muzviitiko zvakawanda, chirwere ichi hachiongororwi nemazvo, kureva kuti, '(hachisati chaongororwa zvakakwana)'.

Kusiyana kwemajini kunogona kukanganisa mamwe mapoka emadzinza kupfuura mamwe. Semuenzaniso, kuchinja kwemajini uku kunoonekwa zvakanyanya muvanhu vatema muUnited States. Zvisinei, chinhu chakakosha ndechekuti havasi vese vane kuchinja uku vanozorwara nechirwere ichi.

Ndezvipi zvinokonzera `(ATTR-CM)`?

Chikonzero chikuru cheATTR-CM yemhuri idambudziko, kana shanduko, mujini rinogadzira puroteni TTR yatakambotaura nezvayo. Kashoma, munhu anogona kuchinja majini aya kekutanga pasina mumwe munhu mumhuri akambova nechirwere ichi. Izvi zvinonzi mutation ye de novo.

Vanachiremba havasati vaziva kuti chii chinokonzera chirwere cheWild-type ATTR-CM. Zvisinei, chinowanzoitika muvarume vane makore anopfuura 65. Saka, zvinofungidzirwa kuti kukwegura uye, kusvika pamwero wakati, murume kana mukadzi zvinogona kuva zvinhu zvinokonzera njodzi.

Chero chikonzero chipi zvacho, chinozoitika ndechekuti miviri yedu inotanga kugadzira mapuroteni asina kujairika, asina kujairika e`(TTR)`. Mapuroteni aya asina kujairika anotyoka nyore nyore, anobatana, uye 'anopetana'. Aya ndiwo maitiro aanopetana, achiumba tumapundu tudiki tunonzi `amyloid fibrils'. Aya anofamba mumuviri wese kuburikidza neropa uye anoiswa munhengo dzakasiyana-siyana, dzakadai semwoyo netsinga. Nekufamba kwenguva, nhengo idzi dzinotanga kukuvara.

Ndezvipi zviratidzo zvechirwere che `(ATTR-CM)`?

Zviratidzo zvechirwere ichi `(ATTR-CM)` zvinogona kusiyana zvikuru kubva pamunhu kusvika pamunhu. Zvakare, zviratidzo zvinosiyana zvichienderana nerudzi rwechirwere.

• Vamwe vanhu vane ATTR-CM yemhando yemusango vanogona kunge vasina zviratidzo.Kana zviratidzo zvikaonekwa, zvinowanzotanga kuonekwa mushure memakore makumi matanhatu nemashanu.
• Zviratidzo zvemhuri zveATTR-CM zvinowanzotanga kuonekwa mushure memakore makumi mashanu. Zvisinei, dzimwe nguva zviratidzo zvinogona kutanga kare, muma20, kana kutozosvika padanho repamusoro, muma80.

Kazhinji, zviratidzo izvi zve `(ATTR-CM)` zvakafanana zvikuru nezviratidzo zve `(Heart Failure)`. Funga kana wanga uchinzwa zvinhu izvi nguva pfupi yadarika:

• Kushaya mweya: Kunzwa kushaya mweya, kunyanya kana wakarara pamubhedha, kana kunyangwe uchiita zvinhu zvishoma, zvakaita sekufamba chinhambwe chipfupi.
• Kunzwa kuguta uye kuzvimba .
• Kuvhiringidzika , kunetseka kufunga zvakajeka.
• Kukosora, kunzwa segoroko (kunyanya kana wakarara pasi).
• Kuzvimba kwemakumbo, zvitsitsinho, netsoka (edema) (sekunge zvazara nemvura).
• Arrhythmia kurova kwemoyo kusina kurongeka, dzimwe nguva kunonzi kurova kwemoyo nekukurumidza, kunyanya chirwere chinonzi atrial fibrillation.
• Kurova kwemoyo kunoratidza kurova kwemoyo nekukurumidza, zvichireva kuti mwoyo unonzwa sekunge uri kurova/kurova .
• Kuneta ndiko kunzwa kuneta uye kuneta nguva dzese pasina chikonzero .
• Kunzwa dzungu, kana kuti sekunge uchafenda .

Ndezvipi zvinetso zvinogona kuitika nekuda kwe `(ATTR-CM)`?

Transthyretin amyloidosis (ATTR-CM) inogona kukonzera matambudziko emoyo, kunyanya kutadza kushanda zvakanaka kwemoyo uye atrial fibrillation (Afib).

Uyezve, kana aya ma amyloid deposits akaungana mutsinga, matambudziko anotevera anogona kuitika:

• Carpal tunnel syndrome: Iyi inyaya apo tsinga iri muruoko inomanikidzwa. Kazhinji, minwe yemaoko ese inova isina simba uye inorwadza, uye dzimwe nguva kurwadziwa kunomutsa iwe husiku.
• Kuona madodzi matema achiyangarara pamberi pemaziso (maziso anoyangarara).
• Kurwadziwa kwetsinga dzemumakumbo: Uku kukuvadzwa kwetsinga dzemumakumbo. Izvi zvinogona kukonzera kusanzwa, kukwenya, kurwadziwa, uye pamwe kurasikirwa nekunzwa mumakumbo.

Dzimwe nguva zvinhu izvi zvinogona kuungana mumusana, zvichikonzera spinal stenosis. Zvinogonawo kuungana mumatishu akasiyana-siyana emuviri, zvichikonzera zvirwere zvakaita sekuputika kwematendon.

Chimwe chinhu chakakosha ndechekuti amyloidosis dzimwe nguva inoperekedzwa nechirwere chinonzi aortic stenosis. Uku kutetepa kwevharuvhu yeaortic inoburitsa ropa kubva mukamuri guru remoyo. Kana chiremba wako akakuona uine aortic stenosis, chiremba wako anogonawo kukuyedza amyloidosis.Izvi zvinonyanya kukosha kana uine zvimwe zviratidzo, zvakaita sekushaya kwemoyo kusina kunaka uye carpal tunnel syndrome.

Chirwere che `(ATTR-CM)` chinoonekwa sei?

Kutaura zvazviri, kuongorora chirwere ichi che "ATTR-CM" dzimwe nguva kunogona kuoma. Nekuti, semuenzaniso, rudzi rwechirwere chenhaka runogona kuratidza zviratidzo zvakafanana nechirwere chemwoyo chinokonzerwa neBP yakakwira. Saka, vamwe vanhu vanogona kutowana "chirwere chisina kururama" pakutanga.

Kune rumwe rutivi, mhando yemhando yemusango hairatidzi zviratidzo zviri pachena nguva dzose. Nokudaro, chirwere ichi chingasaonekwa kusvika paitika dambudziko rakakura sekushaya simba kwemoyo.

Kune nzira dzakawanda dzekuongorora chirwere ichi dzinoshandiswa navanachiremba:

• Kuongororwa kweECG (Electrocardiogram): Izvi zvinoongorora mashandiro emagetsi emwoyo.
• Kuongororwa kwemifananidzo yemoyo: Echocardiogram (iyi inotarisa chimiro uye mashandiro emoyo), MRI scan, PET scan, nezvimwewo.
• Kuongororwa kwemapfupa / Kuongororwa kwemapfupa: Izvi zvinogona kutarisa kana paine amyloid mumuviri.
• Kuongororwa kwemoyo: Izvi zvinosanganisira kutora chidimbu chidiki chenyama kubva pamoyo wochiongorora uchishandisa maikorosikopu kuti uone kana paine amyloid deposits.
• Kuongororwa kweropa: Izvi zvinogona kuona shanduko kana shanduko mujini re`(TTR)`.

Ndeapi marapirwo e `(ATTR-CM)`?

Uyu ndiwo mubvunzo unonyanya kukosha kuvanhu vazhinji. Kutaura chokwadi, hapana mushonga wakakwana weATTR-CM parizvino. Zvakare, hapana nzira chaiyo yekubvisa tambo dzeamyloid dzakatoiswa mumuviri.

Asi, musatya! Iye zvino kune mishonga inogona kuderedza kuunganidzwa kwemapuroteni matsva aya anokuvadza uye kuderedza kufambira mberi kwechirwere. Iyi inyaradzo huru.

Pamusoro pezvo, kurapwa kwakasiyana kunopihwa kudzora zviratidzo zvinokonzerwa nemigumisiro yechirwere ichi, zvakaita sekushaya simba kwemoyo, kusagadzikana kwepfungwa, uye kushaya hope.

• Kune mimwe mishonga chaiyo inopihwa yeATTR-CM yemhuri. Iyi inoshanda nekusunga puroteni yeTTR, kuidzikamisa, uye kudzivirira puroteni kuti isapete. Mienzaniso inosanganisira Tafamidis (Vyndaqel®, Vyndamax®) uye Diflunisal (Dolobid®). Diflunisal chaizvoizvo iNSAID (mushonga usiri westeroidal unorwisa kuzvimba). Dzimwe nguva vanachiremba vanoishandisa 'isina label', zvichireva kuti haina kubvumidzwa zvakananga pachirwere ichi, asi nekuti inobvumidzwa kune mamwe mamiriro ezvinhu uye inogona kubatsira pachirwere ichi.
• Kune mimwe mishonga, yakaita seInotersen (Tegsedi®) nePatisiran (Onpattro®), inoshanda nekunonotsa kugadzirwa kwemapuroteni aya ane amyloid asina kunaka muchiropa.

Kashoma, kana chirwere ichi chawedzera, zvinotevera zvinogona kudiwa:

• Kuisirwa chiropa.
• Kuisirwa itsvo.
• Mudziyo wekubatsira ventricle yekuruboshwe (LVAD) (muchina mudiki unobatsira moyo kupomba ropa) wakanaka, kana kuti senzira yekupedzisira, kuisirwa moyo.

Chirwere ichi `(ATTR-CM)` chinogona kudzivirirwa here?

Kana uine gene mutation `(TTR)` inokonzera `familial' `(Familial) ATTR-CM`, vana vako vane mukana we50% wekugara nhaka gene mutation. Izvi zvinoreva kuti, havasi vana vese, asi mukana mumwe chete pavaviri wekuva nemwana. Zvisinei, chinhu chakakosha kurangarira ndechekuti havasi vana vese vanogara nhaka gene mutation iyi vachakura `(ATTR-CM)`.

Kana uine dambudziko remajini rakadai, zvakanaka kutaura nenyanzvi yemajini paunenge uchironga kuva nevana. Unogona kudzidza nezvesarudzo dzakadai sekuongorora majini usati waisa mwana muchibereko (PGD). Munzira iyi, maembryo akagadzirwa kuburikidza ne in vitro fertilization (IVF) anoongororwa kuti aone kana jini racho risina kunaka asati aiswa muchibereko. Maembryo ane hutano asina kukanganisa anogona kusarudzwa uye kuiswa muchibereko kuti aderedze njodzi yekuti mwana agare nhaka chirwere ichi.

Ndeipi tarisiro yeramangwana yevarwere ve `(ATTR-CM)`?

Transthyretin amyloidosis (ATTR-CM) chirwere chinoramba chichienderera mberi chinogona kutungamira kumatambudziko akakomba. Asi musazvidya mwoyo! Nemishonga mitsva yakaita seTafamidis, pamwe nemishonga mitsva iri kuyedzwa pari zvino, makore ekurarama uye mhando yehupenyu zviri kuvandudzika. Imwe ongororo yakaratidza kuti varwere vakagamuchira mushonga weTafamidis kwemwedzi makumi matatu vakawana kuwedzera kwe13% mukurarama.

Chinhu chinonyanya kukosha ndechekugara uchiona chiremba wemwoyo uye kutevedzera mazano avo kuti uve nechokwadi chekuti uri kuwana mishonga yakakodzera yemoyo wako.

Unofanira kuona chiremba rini?

Kana uine chimwe chezviratidzo zvinotevera kana kupfuura, zvakakosha kuti uone chiremba nekukurumidza uye utsvage mazano usingazviregerere:

• Kuvhiringidzika kamwe kamwe kana matambudziko ekurangarira.
• Kuona zvinhu zvakaita semavanga matema zvichiyangarara pamberi pemaziso kunonzi "Eye floaters".
• Kurova kwemoyo kusina kurongeka kana kunzwa kwekurova kwemoyo nekukurumidza (kurova kwemoyo).
• Kutadza kufema zvakanaka.
• Pasina chikonzero chakajekaKuzvimba uye kuwedzera uremu.

Mibvunzo ipi yaunofanira kubvunza chiremba wako?

Kana ukaonekwa uine chirwere ichi (ATTR-CM), kana kuti uchifungidzira kuti unacho, zvakanaka kubvunza chiremba wako mibvunzo yakaita seiyi:

• Rudzii rweTransthyretin Amyloidosis rwandinarwo? (Rwedzinza kana kuti rwemusango?)
• Chii chiri kukonzera mamiriro ezvinhu aya kwandiri?
• Ndine here shanduko, kana chikanganiso, mujini rekuti `(TTR)`?
• Ndedzipi nzira dzekurapa dzamunondikurudzira?
• Migumisiro yemishonga iyi ndeipi?
• Ndichada chimwe chinhu chakaita sekuisirwa nhengo mune ramangwana here?
• Ndezvipi zviratidzo zvematambudziko andinofanira kunyanya kunetseka nazvo?

Zvimwe zvezvinhu zvakakosha zvatinofanira kurangarira kubva munyaya iyi ndeizvi:

Transthyretin amyloidosis, kana kuti ``ATTR-CM``, chirwere apo puroteni ``Transthyretin`` inopetwa yozvigadzika mumoyo se``fibrils`` diki. Izvi zvinogona kukonzera kuti makamuri emoyo akobvu uye asashande zvakanaka, zvichikonzera chirwere chinonzi ``Cardiomyopathy``.

Vamwe vanhu vanogona kunge vaine chirwere ichi chinoitika mumhuri (Familial ATTR-CM). Izvi zvinoreva kuti pane mhedzisiro yemajini. Kune vamwe, chirwere ichi chinogona kukura nekukura pasina chikonzero chakajeka (Wild-type ATTR-CM).

Kunyange hazvo pasina mushonga uchiripo, kune mishonga inoshanda uye nzira dzekurapa dzinogona kubatsira kudzora mapuroteni matsva, kuderedza zviratidzo, uye kudzivirira zvirwere zvakakomba zvakaita sekurwadziwa kwemoyo. Kashoma, kana chirwere chikaenderera mberi, chimwe chinhu chakadai sekuisirwa nhengo yemuviri chingave chinodiwa.

Chinhu chakakosha ndechekuti kana uine zviratidzo zvisingawanzoitika kana kusagadzikana kwakabatana nemoyo wako, usazviregeredza nekuti 'zvakangoitika' uye tsvaga rubatsiro rwechiremba nekukasika. Nekuti, sechirwere chipi zvacho, kana izvi zvikaonekwa nekukurumidza, mikana yekutanga kurapwa inowedzera, kuderedza matambudziko, uye kurarama hupenyu hwakajairika.

` Transthyretin amyloidosis, ATTR-CM, chirwere chemwoyo, amyloidosis, kutadza kwemoyo, kuiswa kwemapuroteni, zvirwere zvenhaka