Amyloidosis yentliziyo

I-amyloidosis yentliziyo sisifo esibangelwa ziidiphozithi zeproteni engaqhelekanga (i-amyloid) kwinyama yentliziyo. Ezi dipozithi zenza ukuba kube nzima ukuba intliziyo isebenze ngokufanelekileyo.

I-Amyloidosis liqela lezifo apho iziqhuma zeeproteni ezibizwa ngokuba ziiamyloid zakha kwizicubu zomzimba. Ixesha elingaphezulu, ezi proteni zitshintsha indawo yesiqhelo, zikhokelela ekusileleni kwelungu elibandakanyekayo. Zininzi iindlela ze-amyloidosis.

I-amyloidosis yentliziyo ("istiff heart syndrome") yenzeka xa iidipozithi ze-amyloid zithatha indawo yesihlunu sentliziyo esiqhelekileyo. Luhlobo oluqhelekileyo lokuthintela i-cardiomyopathy. I-amyloidosis yentliziyo inokuchaphazela indlela imiqondiso yombane ehamba ngayo entliziyweni (inkqubo yokuqhuba). Oku kunokukhokelela ekubetheni kwentliziyo okungaqhelekanga (arrhythmias) kunye neempawu zentliziyo ezingalunganga (ibhloko yentliziyo).

Le meko inokufunyanwa njengelifa. Oku kubizwa ngokuba yintsapho yentliziyo i-amyloidosis. Inokuphinda ikhule njengesiphumo sesinye isifo esifana nohlobo lwethambo kunye nomhlaza wegazi, okanye njengesiphumo senye ingxaki yezonyango ebangela ukudumba. I-amyloidosis yentliziyo ixhaphake kakhulu emadodeni kunabafazi. Esi sifo sinqabile kubantu abangaphantsi kweminyaka engama-40.

Abanye abantu banokungabikho mpawu. Xa zikhona, iimpawu zinokubandakanya:

• Ukuchama kakhulu ebusuku
• Ukudinwa, ukunciphisa amandla omthambo
• Iipalpitations (imvakalelo yokubetha kwentliziyo)
• Ukuphefumla okufutshane kunye nomsebenzi
• Ukudumba kwesisu, imilenze, amaqatha, okanye elinye ilungu lomzimba
• Ingxaki yokuphefumla ngelixa ulele phantsi

Iimpawu ze-amyloidosis yentliziyo inokuhambelana nenani leemeko ezahlukeneyo. Oku kunokwenza ukuba kube nzima ukufumanisa ingxaki.

Iimpawu zinokubandakanya:

• Izandi ezingaqhelekanga kwimiphunga (ukuqhekeka kwemiphunga) okanye intliziyo iyakrokra
• Uxinzelelo lwegazi olusezantsi okanye olwehlayo xa usukuma
• Imithambo eyandisiweyo yentamo
• Isibindi esidumbileyo

Olu vavanyo lulandelayo lunokwenziwa:

• Isifuba okanye isisu se-CT scan (siqwalasele "umgangatho wegolide" ukunceda ukufumanisa le meko)
• I-Coronary angiography
• I-Electrocardiogram (ECG)
• Echocardiogram
• Imagnetic resonance imaging (iMRI)
• Iskena sentliziyo yenyukliya (MUGA, RNV)
• Ipositron emission tomography (iPET)

I-ECG inokubonisa iingxaki ngentliziyo okanye imiqondiso yentliziyo. Ingabonisa imiqondiso esezantsi (ebizwa ngokuba yi "voltage esezantsi").

I-biopsy ye-cardiac isetyenziselwa ukuqinisekisa ukuxilongwa. I-biopsy yenye indawo, enje ngesisu, izintso, okanye umongo wethambo, ihlala isenziwa ngokunjalo.

Umboneleli wakho wezempilo unokukuxelela ukuba wenze utshintsho kwindlela otya ngayo, kubandakanya ukunciphisa ityuwa kunye nolwelo.

Kuya kufuneka uthathe iipilisi zamanzi (i-diuretics) ukunceda umzimba wakho ukuba ulahle ulwelo olugqithisileyo. Umboneleli unokukuxelela ukuba uzilinganise yonke imihla. Ukufumana ubunzima beepawundi ezi-3 okanye nangaphezulu (ikhilogremu enye okanye nangaphezulu) ngaphezulu kweentsuku ezi-1 ukuya kwezi-2 kunokuthetha ukuba kukho ulwelo oluninzi emzimbeni.

Amayeza afaka i-digoxin, i-calcium-channel blockers, kunye ne-beta-blockers inokusetyenziswa kubantu abane-atrial fibrillation. Nangona kunjalo, amayeza kufuneka asetyenziswe ngononophelo, kwaye idosi kufuneka ibekwe esweni ngononophelo. Abantu abane-amyloidosis yentliziyo banokuba novakalelo ngakumbi kwiziphumo ebezingalindelekanga zala machiza.

Olunye unyango lunokubandakanya:

• Unyango ngamayeza
• I-cardioverter-defibrillator enokufakwa (AICD)
• I-Pacemaker, ukuba kukho iingxaki ngeempawu zentliziyo
• I-Prednisone, iyeza elichasayo

Ukufakelwa kwentliziyo kunokuqwalaselwa kubantu abaneentlobo ezithile ze-amyloidosis ezinesifo sentliziyo esifanelekileyo. Abantu abane-amyloidosis yelifa banokufuna ukufakelwa kwesibindi.

Kwixesha elidlulileyo, i-cardiac amyloidosis bekucingelwa ukuba ayisosifo esinganyangekiyo kwaye sibulala ngokukhawuleza. Nangona kunjalo, intsimi itshintsha ngokukhawuleza. Iindidi ezahlukeneyo ze-amyloidosis zinokuchaphazela intliziyo ngeendlela ezahlukeneyo. Ezinye iintlobo zinzima kunezinye. Abantu abaninzi ngoku banokulindela ukusinda kwaye bafumane umgangatho wobomi obulungileyo iminyaka eliqela emva kokuxilongwa.

Iingxaki zinokubandakanya:

• I-Atrial fibrillation okanye i-ventricular arrhythmias
• Ukusilela kwentliziyo
• Ulwakhiwo lwamanzi kwisisu (ascites)
• Ukonyusa ubuntununtunu kwi-digoxin
• Uxinzelelo lwegazi oluphantsi kunye nesiyezi ekuchameni kakhulu (ngenxa yeyeza)
• Isifo sesinus
• Iimpawu zesifo sokuqhutywa kwentliziyo (Iimpawu zokuziphatha ezinxulumene nokuqhutywa ngokungaqhelekanga kweempembelelo kwisisipha sentliziyo)

Tsalela umnikezeli wakho ukuba unale ngxaki kwaye uphuhlise iimpawu ezintsha ezinje:

• Ukuba nesiyezi xa utshintsha indawo
• Ubunzima obugqithisileyo (fluid) inzuzo
• Ukunciphisa umzimba ngokweqile
• Ukuphelelwa ngamandla
• Iingxaki zokuphefumla ezinzima

IAmyloidosis - intliziyo; I-amyloidosis yentliziyo ephambili - uhlobo lwe-AL; Amyloidosis esecaleni kwentliziyo - uhlobo lweAA; Ukuqina kwentliziyo; I-senile amyloidosis

• Icandelo lentliziyo phakathi embindini
• Ukuchaneka kwentliziyo
• Ipayipi yebheyiphu

UFalk RH, uHershberger RE. I-cardiomyopathies edibeneyo, ethintelayo, kunye ne-infiltrative. Ku: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, ii-eds. Isifo sentliziyo seBraunwald: Incwadi yesifundo seMpilo yeNtliziyo. Ngomhla we-11. IPhiladelphia, PA: Elsevier; I-2019: isahluko 77.

UMcKenna WJ, uElliott PM. Izifo ze-myocardium kunye ne-endocardium. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 54.