Kuyini Ukuphila okulindelwe nge-ATTR Amyloidosis?

-Delile

• Isikhathi sokuphila namazinga okusinda
• Izici ezithinta amathuba okusinda
• Izinhlobo ze-ATTR amyloidosis
• Umndeni we-ATTR amyloidosis
• Uhlobo lwe-ATTR amyloidosis
• Ezinye izinhlobo ze-amyloidosis
• Izinketho zokwelashwa
• Ukuthatha

Ku-amyloidosis, amaprotheni angajwayelekile emzimbeni aguqula isimo bese ehlangana ndawonye ukwakha amafibril amyloid. Leyo micu yakha izicubu nezitho, ezingabavimba ukuthi basebenze kahle.

I-ATTR amyloidosis ingenye yezinhlobo ezivame kakhulu ze-amyloidosis. Kuyaziwa nangokuthi i-transthyretin amyloidosis. Kubandakanya iphrotheni eyaziwa ngokuthi i-transthyretin (TTR), ekhiqizwa esibindini.

Kubantu abane-ATTR amyloidosis, i-TTR yakha ama-clumps angakha izinzwa, inhliziyo, noma ezinye izitho zomzimba. Lokhu kungadala ukwehluleka komzimba okubeka engcupheni impilo.

Funda ukuze ufunde ukuthi lesi simo singathinta kanjani isikhathi sokuphila somuntu nezinto ezithonya amazinga okusinda, kanye nolwazi lwangemuva mayelana nezinhlobo ezahlukene ze-ATTR amyloidosis nokuthi ziphathwa kanjani.

Isikhathi sokuphila namazinga okusinda

Isikhathi sokuphila namazinga okusinda ayahlukahluka ngokuya ngohlobo lwe-ATTR amyloidosis umuntu anayo. Izinhlobo ezimbili eziyinhloko ezomndeni nezinhlobo zasendle.

Ngokwesilinganiso, abantu abane-ATTR amyloidosis yomndeni baphila iminyaka engu-7 kuya kwengu-12 ngemuva kokuthola ukuxilongwa kwabo, ngokwe-Genetic and Rare Diseases Information Center.

Ucwaningo olushicilelwe ephephabhukwini i-Circulation lithole ukuthi abantu abane-ATTR amyloidosis yohlobo lwasendle baphila isilinganiso seminyaka engaba ngu-4 ngemuva kokuxilongwa. Izinga lokusinda leminyaka emihlanu phakathi kwabahlanganyeli bokufunda lalingamaphesenti angama-36.

I-ATTR amyloidosis ivame ukudala imicu ye-amyloid yakhele enhliziyweni. Lokhu kungadala isigqi senhliziyo esingajwayelekile nokuhluleka kwenhliziyo okusongela impilo.

Alikho ikhambi elaziwayo le-ATTR amyloidosis. Kodwa-ke, ukuxilongwa nokwelashwa kusenesikhathi kungasiza ukubambezela ukukhula kwesifo.

Izici ezithinta amathuba okusinda

Izici eziningana zingathinta amazinga okusinda kanye nesikhathi sokuphila kubantu abane-ATTR amyloidosis, kufaka phakathi:

• uhlobo lwe-ATTR amyloidosis abanayo
• yiziphi izitho ezithintekayo
• lapho izimpawu zabo ziqala
• baqala kanjani ukwelashwa kusenesikhathi
• imiphi imishanguzo abayitholayo
• impilo yabo iyonke

Ucwaningo oluningi luyadingeka ukuze kufundwe ukuthi izindlela ezahlukahlukene zokwelashwa zingathinta kanjani amazinga okusinda kanye nesikhathi sokuphila kubantu abanalesi simo.

Izinhlobo ze-ATTR amyloidosis

Uhlobo lwe-ATTR amyloidosis umuntu analo luzothinta umbono wakhe wesikhathi eside.

Uma uhlala ne-ATTR amyloidosis, kodwa awunasiqiniseko sokuthi hlobo luni, buza udokotela wakho. Izinhlobo ezimbili eziyinhloko ezomndeni nezinhlobo zasendle.

Ezinye izinhlobo ze-amyloidosis nazo zingakhula lapho amaprotheni ngaphandle kwe-TTR ehlangana nama-amyloid fibrils.

Umndeni we-ATTR amyloidosis

I-ATTR amyloidosis eyaziwa nangokuthi ifa le-ATTR amyloidosis. Kubangelwa ukuguqulwa kwezakhi zofuzo okungadluliselwa kusuka kumzali kuye enganeni.

Lokhu kuguqulwa kofuzo kubangela ukuthi i-TTR ingazinzi kakhulu kunokujwayelekile. Lokhu kuphakamisa amathuba okuthi i-TTR izokwakha ama-amyloid fibrils.

Izinguquko eziningi ezahlukahlukene zofuzo zingadala i-ATTR amyloidosis yomndeni. Ngokuya ngokushintshwa kwezakhi zofuzo umuntu anakho, lesi simo singathinta izinzwa zabo, inhliziyo yabo, noma kokubili.

Izimpawu ze-ATTR amyloidosis yomndeni ziqala ebudaleni futhi ziba zimbi ngokuhamba kwesikhathi.

Uhlobo lwe-ATTR amyloidosis

Uhlobo lwe-ATTR amyloidosis aludalwa ukuguqulwa kwezakhi zofuzo okwaziwayo. Esikhundleni salokho, kukhula ngenxa yezinqubo zokuguga.

Kulolu hlobo lwe-ATTR amyloidosis, i-TTR iyazinza kancane ngeminyaka futhi iqala ukwakha imicu yefrimu yama-amyloid. Labo fibrils bavame ukufakwa enhliziyweni.

Lolu hlobo lwe-ATTR amyloidosis luvame ukuthinta abesilisa abaneminyaka engaphezu kwengama-70 ubudala.

Ezinye izinhlobo ze-amyloidosis

Ezinye izinhlobo ze-amyloidosis zikhona, kufaka phakathi i-AL ne-AA amyloidosis. Lezi zinhlobo zifaka amaprotheni ahlukile kune-ATTR amyloidosis.

I-AL amyloidosis yaziwa nangokuthi i-amyloidosis eyinhloko. Kubandakanya izingxenye ezingavumelekile zama-antibody, ezaziwa ngamaketanga alula.

I-AA amyloidosis ibizwa nangokuthi i-amyloidosis yesibili. Kubandakanya iphrotheni eyaziwa ngokuthi yi-serum amyloid A. Kuvame ukubangelwa ukutheleleka noma isifo sokuvuvukala, njenge-rheumatoid arthritis.

Izinketho zokwelashwa

Uma une-ATTR amyloidosis, uhlelo lokwelashwa lwakho olunconywayo luzoncika ohlotsheni oluthile onalo, kanye nezitho ezithintekayo nezimpawu ezikhulayo.

Ngokuya ngokutholakala kwakho, bangahle bakunikeze okukodwa noma okuningi kokulandelayo:

• ukufakelwa kwesibindi, esetshenziselwa ukwelapha ezinye izimo ze-ATTR amyloidosis yomndeni
• Izithulisi ze-ATTR, isigaba semithi esiza ukunciphisa ukukhiqizwa kwe-TTR kubantu abane-ATTR amyloidosis yomndeni
• Izithambisi ze-ATTR, isigaba semithi engasiza ukumisa i-TTR ekwakheni imicu yama-amyloid kubantu abane-amyloidosis yohlobo lomndeni noma lwasendle

Odokotela bakho bangaphinde bancome ezinye izindlela zokwelapha ukusiza ukuphatha izimpawu ezingaba khona kanye nezinkinga ze-ATTR amyloidosis.

Isibonelo, lezi zindlela zokwesekwa zingafaka ushintsho ekudleni, isisu, noma ukuhlinzwa ukusiza ukwelapha ukwehluleka kwenhliziyo.

Ezinye izindlela zokwelashwa kwe-ATTR amyloidosis nazo ziyafundwa ekuhlolweni kwemitholampilo, kufaka phakathi imishanguzo engasiza ukusula imicu ye-amyloid emzimbeni.

Ukuthatha

Uma une-ATTR amyloidosis, khuluma nodokotela wakho ukuze ufunde kabanzi ngezinketho zakho zokwelashwa nangombono wesikhathi eside.

Ukuxilongwa nokwelashwa kusenesikhathi kungasiza ekunciphiseni ukukhula kwesifo, kuqede izimpawu, futhi kuthuthukise isikhathi sakho sokuphila.

Uhlelo lokwelashwa olunconywayo lukadokotela wakho luzoya ngohlobo oluthile lwesifo onaso, kanye nezitho ezithintekile.

Ukwelashwa okusha kungabuye kutholakale ngokuzayo ukusiza ukuthuthukisa amazinga okusinda kanye nekhwalithi yempilo kubantu abakulesi simo.

Udokotela wakho angakusiza ufunde ngentuthuko yokwelashwa yakamuva.