I-ATTR Amyloidosis: Izimpawu, Ukuxilongwa, kanye Nezokwelapha

-Delile

• Ukubuka konke
• Izimbangela
• Izinhlobo ze-ATTR amyloidosis
• Izimpawu
• Ukuxilongwa kwe-ATTR
• Ukwelashwa
• Umbono

Ukubuka konke

I-Amyloidosis yisifo esingajwayelekile esenzeka lapho kunenqwaba yamaprotheni e-amyloid emzimbeni. Lawa maprotheni angakha emithanjeni yegazi, emathanjeni, nasezithweni ezinkulu, okuholela ezinkingeni ezahlukahlukene.

Lesi simo esiyinkimbinkimbi aselapheki, kodwa singaphathwa ngezindlela zokwelashwa. Ukuxilongwa nokwelashwa kungaba yinselele ngoba izimpawu nezimbangela ziyahlukahluka phakathi kwezinhlobo ezahlukene ze-amyloidosis. Izimpawu nazo zingathatha isikhathi eside ukuzibonakalisa.

Funda ukuze ufunde ngolunye lwezinhlobo ezivame kakhulu: i-amyloid transthyretin (ATTR) i-amyloidosis.

Izimbangela

I-ATTR amyloidosis ihlobene nokukhiqizwa okungavamile nokwakhiwa kohlobo lwe-amyloid okuthiwa i-transthyretin (TTR).

Umzimba wakho uhloselwe ukuba nenani lemvelo le-TTR, elenziwa ikakhulukazi isibindi. Lapho ingena egazini, i-TTR isiza ukuhambisa ama-hormone e-thyroid novithamini A kuwo wonke umzimba.

Olunye uhlobo lwe-TTR lwenziwa ebuchosheni. Inesibopho sokwenza uketshezi lwe-cerebrospinal.

Izinhlobo ze-ATTR amyloidosis

I-ATTR wuhlobo olulodwa lwe-amyloidosis, kepha kukhona nama-subtypes we-ATTR.

Ifa, noma i-ATTR yomndeni (hATTR noma i-ARRTm), isebenza emindenini. Ngakolunye uhlangothi, i-ATTR etholakele (engeyona eyifa) yaziwa njenge- “wild-type” ATTR (ATTRwt).

I-ATTRwt ivame ukuhlotshaniswa nokuguga, kepha hhayi nezinye izifo zemizwa.

Izimpawu

Izimpawu ze-ATTR ziyahlukahluka, kepha zingafaka:

• ubuthakathaka, ikakhulukazi emilenzeni yakho
• ukuvuvukala kwemilenze neqakala
• ukukhathala ngokweqile
• ukuqwasha
• ukushaya kwenhliziyo
• ukuncipha komzimba
• Izinkinga zamathumbu nezomchamo
• libido ephansi
• isicanucanu
• umhubhe we-carpal syndrome

Abantu abane-ATTR amyloidosis nabo bathambekele kakhulu ezifweni zenhliziyo, ikakhulukazi nge-ATTR yohlobo lwasendle. Ungaqaphela ezinye izimpawu ezihlobene nenhliziyo, njenge:

• ubuhlungu besifuba
• ukushaya kwenhliziyo okungajwayelekile noma okusheshayo
• isiyezi
• ukuvuvukala
• ukuphelelwa umoya

Ukuxilongwa kwe-ATTR

Ukuthola i-ATTR kungaba yinselele ekuqaleni, ikakhulukazi njengoba izimpawu zayo eziningi zilingisa ezinye izifo. Kepha uma othile emndenini wakho enomlando we-ATTR amyloidosis, lokhu kufanele kusize ukuqondisa udokotela wakho ukuthi avivinye izinhlobo zofuzo ze-amyloidosis. Ngaphezu kwezimpawu zakho nomlando wezempilo yomuntu siqu, udokotela wakho anga-oda ukuhlolwa kofuzo.

Izinhlobo zasendle ze-ATTR kungaba nzima kakhulu ukuxilonga. Esinye sezizathu ukuthi izimpawu zifana nokuhluleka kwenhliziyo okucinana.

Uma i-ATTR isolwa futhi ungenawo umlando womndeni wesifo, udokotela wakho uzodinga ukuthola ubukhona bama-amyloid emzimbeni wakho.

Enye indlela yokwenza lokhu ngokusebenzisa ukuskena kwe-scintigraphy yenuzi. Lokhu kuskena kubheka okufakwa yi-TTR emathanjeni akho. Ukuhlolwa kwegazi nakho kunganquma ukuthi kukhona yini okufakwa egazini. Enye indlela yokuxilonga lolu hlobo lwe-ATTR ukuthatha isampula elincane (i-biopsy) lezicubu zenhliziyo.

Ukwelashwa

Kunezinhloso ezimbili zokwelashwa kwe-ATTR amyloidosis: misa ukukhula kwesifo ngokukhawulela ukufakwa kwe-TTR, futhi unciphise imiphumela i-ATTR enayo emzimbeni wakho.

Njengoba i-ATTR ithinta kakhulu inhliziyo, ukwelashwa kwalesi sifo kuvame ukugxila kule ndawo kuqala. Udokotela wakho angahle anikeze imishanguzo yokunciphisa isisu ukuze kunciphise ukuvuvukala, kanye nezinciphisi zegazi.

Ngenkathi izimpawu ze-ATTR zivame ukulingisa lezo zesifo senhliziyo, abantu abanalesi simo abakwazi ukuthatha kalula imithi ehloselwe ukwehluleka kwenhliziyo okucinana.

Lokhu kufaka phakathi i-calcium block blockers, i-beta-blockers, ne-ACE inhibitors. Eqinisweni, le mithi ingaba yingozi. Lesi ngesinye sezizathu eziningi zokuthi kungani ukuxilongwa okufanele kubalulekile kusukela ekuqaleni.

Ukufakelwa kwenhliziyo kunganconywa ezimweni ezinzima ze-ATTRwt. Lokhu kunjalo ikakhulukazi uma unokulimala kwenhliziyo okuningi.

Ngamacala wefa, ukufakelwa kwesibindi kungasiza ukumisa ukwakheka kwe-TTR. Kodwa-ke, lokhu kusiza kuphela ekuhlolweni kokuqala. Udokotela wakho futhi angabheka izindlela zokwelapha zofuzo.

Yize lungekho ikhambi noma ukwelashwa okulula, imishanguzo emisha eminingi njengamanje isezivivinyweni zokwelashwa, futhi intuthuko yokwelashwa iseduze. Khuluma nodokotela wakho ukuze ubone ukuthi isilingo somtholampilo sikulungele yini.

Umbono

Njengakwezinye izinhlobo ze-amyloidosis, alikho ikhambi le-ATTR. Ukwelashwa kungasiza ekunciphiseni ukukhula kwesifo, ngenkathi ukuphathwa kwezimpawu kungakhuphula izinga lakho lokuphila.

I-hATTR i-amyloidosis ine-prognosis engcono uma iqhathaniswa nezinye izinhlobo ze-amyloidosis ngoba iqhubeka kancane.

Njenganoma isiphi isimo sempilo, lapho uhlolwa kusenesikhathi utholakale utholakala nge-ATTR, kuba ngcono umbono jikelele. Abaphenyi baqhubeka ngokufunda kabanzi ngalesi simo, ngakho-ke ngokuzayo, kuzoba nemiphumela engcono kakhulu kuwo womabili ama-subtypes.