Ingaba iiproteni emzimbeni ziphazamisekile? Sithetha ngale amyloidosis!

Ngaba nawe uziva udiniwe, udiniwe, kwaye mhlawumbi unciphisa umzimba ngendlela engaqhelekanga kule mihla? Ngamanye amaxesha, utshintsho oluncinci emizimbeni yethu lunokukhokelela kwizinto ezinkulu. Namhlanje siza kuthetha ngesifo esenzeka xa uhlobo oluthile lweprotheyini emizimbeni yethu luphambana kancinci, lujike kakubi, luze lubekwe kwizitho zethu ezibalulekileyo. Oku sikubiza ngokuba yi-amyloidosis . Ungakhathazeki, siza kuthetha ngale nto ngokulula nangendlela onokuyiqonda.

Yintoni kanye kanye i-amyloidosis?

Ngamafutshane, i-amyloidosis yimeko apho iiproteni ze-amyloid (eziziiproteni ezingaqhelekanga) emizimbeni yethu zidibana, zihlangana, kwaye zenze amaqhekeza amancinci, njengeefayibha ezibizwa ngokuba yi-fibrils, ezihlala kwizitho ezibalulekileyo ezifana nentliziyo yethu, izintso, nesibindi. Xa zisenzeka, kwaye ukuba azinyangwa, ezi fibrils zeproteni ezingaqhelekanga zinokubangela umonakalo omkhulu kwizitho zethu.

Ngamanye amaxesha ezi proteni zingaqhelekanga zinokuqokelelana kwindawo encinci nje yelungu. Oku kubizwa ngokuba yi -localized amyloidosis . Oku kunokuchaphazela ulusu lwethu, inxalenye encinci yesinyi, okanye indlela yokuphefumla. Nangona kunjalo, rhoqo ezi fibrils zifakwa kwilungu lonke okanye kumalungu ahlukeneyo emzimbeni wonke. Oku kubizwa ngokuba yi -systemic amyloidosis .

Kukho iindaba ezimnandi. Oogqirha banokumisa ukuveliswa kweeproteni ezingaqhelekanga ezibangela i-amyloidosis. Kwezinye iintlobo ze-amyloidosis, inkqubo yethu yokuzikhusela yomzimba inokunceda ukususa ezi proteni. Oku kunokunciphisa iimpawu zakho kwaye kuphucule impilo yamalungu achaphazelekayo.

Ngaba kukho iintlobo ze-amyloidosis?

Ewe, oogqirha bahlula esi sifo ngokweeproteni ezichaphazelekileyo. Iintlobo eziphambili ezibonwayo zezi:

• I-AL amyloidosis : Kulapho umzimba wakho uvelisa inani elingaqhelekanga leeproteni zekhonkco lokukhanya (eziyinxalenye enkulu yee-antibodies). Ezi fibrils zihlala ziqokelelana entliziyweni (i-cardiac amyloidosis) kunye nezintso. I-AL amyloidosis inokuchaphazela nemithambo-luvo yakho, ulusu, kunye namalungu okugaya ukutya. Olu lolona hlobo luqhelekileyo lwe-amyloidosis.
• I-AA amyloidosis : Kule nto, iziqwenga zeprotheyini ye-serum A ziyaqokelelana kwizitho zakho zomzimba. Oku kunokwenzeka ukuba unesifo sokudumba ixesha elide, oko kuthetha isifo esibangela ukudumba emzimbeni (umzekelo, isifo samathambo, isifo samathumbu esidumbayo, okanye usulelo oluhlala ixesha elide). Oku kunokuchaphazela izintso zakho, izitho zokugaya ukutya, okanye intliziyo.
• I-ATTR amyloidosis: Kule meko, isibindi sakho senza umlinganiselo ongaqhelekanga weproteni ye-transthyretin . Le proteni ye-transthyretin engaqhelekanga ifakwa entliziyweni kwaye ngamanye amaxesha kwimithambo-luvo. Ezinye iintlobo zizuzwa njengelifa (i-familial amyloidosis), ngelixa ezinye zikhula kamva ebomini njengoko umntu ekhula (i-wild-type ATTR).

Ixhaphake kangakanani le sifo?

I-Amyloidosis sisifo esingaqhelekanga . Umzekelo, kwanaseMelika, oogqirha baqikelela ukuba kuphela amatyala amatsha e-AL amyloidosis ayi-1,275 ukuya kuma-3,200 afunyaniswa minyaka le. Ngoko ke, asisosifo siqhelekileyo.

Zithini iimpawu zesi sifo?

Iimpawu ze-amyloidosis zinokwahluka kumntu nomntu, kuxhomekeke kuhlobo lweproteni engaqhelekanga kunye nendawo apho ii-fibrils zibekwe khona.

Khawucinge nje, ukuba ezi proteni zibekwe entliziyweni (`(cardiac amyloidosis)`), ke iimpawu ezenzekayo bubuthathaka, ubunzima bokuphefumla, ukutyhafa (oku kungaba luphawu lwesigqi sentliziyo esingaqhelekanga) okanye ukungasebenzi kakuhle kwentliziyo kunye nokudumba kwemilenze.

Kwakhona, ukuba ezi proteni zibekwe kwizintso (i-renal amyloidosis), iimpawu ezinje ngokudumba kweenyawo nemilenze kunye nokubona amaqamza kumchamo zinokubakho.

Kukho iimpawu ezininzi eziqhelekileyo ezinokubonwa kwi-amyloidosis:

• Ndiziva ndidiniwe ngendlela engaqhelekanga.
• Ukunciphisa umzimba ngaphandle kwesizathu.
• Ubuthathaka okanye ukuba ndindisholo kwamalungu omzimba, ukungakwazi ukubamba into ngendlela efanelekileyo.
• Ukuziva ngathi izandla azinanto.
• Utshintsho lolusu: Oku kuthetha izinto ezifana nolusu olukrweleka lula kunye namabala amfusa (purpura) ajikeleze amehlo.

Kutheni le nto i-amyloidosis isenzeka?

I-Amyloidosis yenzeka xa iiproteni emizimbeni yethu zisongwa ngendlela engafanelekanga kwaye zinamathela. Ziyahlangana, zenze amaqhekeza, okanye ii-fibrils, kwaye zihlale kwizitho zomzimba nakwizicubu. Oogqirha bakubiza oku ngokuthi "yingxaki yokusongwa kweproteni ngendlela engafanelekanga." Iiproteni ekufuneka zilungelelaniswe, ngamatyathanga amade, ziyabhideka kwaye zingakwazi ukwaphulwa ngokufanelekileyo ngumzimba, nto leyo ebangela iingxaki.

Izinto ezinokubangela oku zezi:

• Utshintsho lwezakhi zofuzo (utshintsho) : Ungazuza ilifa utshintsho lwezakhi zofuzo (utshintsho) olwenza iiproteni ze-amyloid ezingaqhelekanga. Okanye, olu tshintsho lwezakhi zofuzo lunokwenzeka ngexesha lobomi bomntu, ngezizathu ezingaziwayo.
• Ezinye iimeko zempilo ezisisiseko: Ngamanye amaxesha i-amyloidosis inokubangelwa yimeko eyahlukileyo yezonyango. Oku kwenzeka kwi-AA amyloidosis. Oku kuqheleke kakhulu kubantu abanezifo ezihlala ixesha elide okanye izifo ezingapheliyo ezifana ne-rheumatoid arthritis.

Ngubani onokuba nethuba elingcono lokuphuhlisa oku? (Izinto Ezinobungozi)

Kukho izinto ezininzi ezinokubangela umngcipheko onokuchaphazela uphuhliso lwe-amyloidosis:

• Ubudala : Esi sifo sidla ngokufunyaniswa kubantu abaneminyaka engama-60 nangaphezulu .
• Isini : Amadoda anamathuba amaninzi okufumana esi sifo.
• Uhlanga : Kufunyenwe ukuba abantu abamnyama eMelika banamathuba amaninzi okufumana imfuza yofuzo ebangela uhlobo lwelifa lwe-'ATTR amyloidosis'. (Akukho datha ithile ingako malunga noku kwilizwe lethu, kodwa ngokubanzi kuthiwa ezinye iintlanga zinotyekelo oluphezulu kwiintlobo ezithile.)
• Ezinye iimeko zonyango : Ukuba nesifo sokudumba esingapheliyo kwandisa umngcipheko wokuba ne-AA amyloidosis. Kwakhona, phakathi kwe-12% kunye ne-15% yabantu abane -multiple myeloma, uhlobo lomhlaza wegazi, bafumana i-AL amyloidosis.
• I-Dialysis yexesha elide : Abantu abanesifo sezintso esingapheliyo abasele besebenzisa i-dialysis ixesha elide banokufumana iintlobo ezithile ze-amyloidosis.
• I-Amyloidosis kusapho : Ezinye iintlobo ze-amyloidosis zinokungena kwiintsapho. Oku kuthetha ukuba zinokufunyanwa njengelifa.

Zeziphi iingxaki ezinokubangela oku?

Ezi ntsalela ze-amyloid zinokuthintela amalungu achaphazelekayo ukuba angasebenzi kakuhle. Iingxaki ezinokubakho ziquka:

• Ukusebenza kwentliziyo kuyehla, ekugqibeleni intliziyo iyeke ukusebenza.
• Isifo sezintso kunye nokungaphumeleli kwezintso ekugqibeleni .
• I-Neuropathy (ebangela ukungaziva mnandi kunye nobuthathaka).

Kodwa khumbula, ukuba ufuna unyango kwiqela elifanelekileyo lezonyango, unganciphisa amathuba ezi ngxaki.

Sichongwa njani esi sifo ngokuchanekileyo? (Ukuxilongwa)

Oogqirha benza i-biopsy ukuze babone iproteni ebangela i-amyloidosis. Oku kuquka ukuthatha iqhekeza elincinci lesicubu kwilungu elichaphazelekayo okanye kwindawo apho ezi fibrils zihlala ziqokelelana khona (umzekelo, kumafutha angaphantsi kwesikhumba sesisu sakho okanye kumongo wakho wethambo).

Ukongeza, kukho ezinye iimvavanyo onokuzenza:

• Uvavanyo lwegazi nomchamo : Olu vavanyo lunokujonga amanqanaba eeprotheyini angaqhelekanga egazini okanye emchameni wakho. Lunokusetyenziswa ukubona indlela amalungu omzimba achaphazelekileyo asebenza ngayo.
• Uvavanyo lokufota: Olu vavanyo lunokunceda oogqirha babone umonakalo kwizitho ezichaphazelekayo. Ngokuxhomekeke apho zikhoyo iifibrils, unokufuna i-echocardiogram , ejonga intliziyo, i- MRI , ebonisa imifanekiso eneenkcukacha zezitho kunye nezicubu zomzimba, okanye ezinye iimvavanyo.
• Uvavanyo lwemfuza : Ukuba ugqirha wakho ukrokrela ukuba une-amyloidosis ngenxa yokuguquka kwemfuza, olu vavanyo lunokwenziwa.

Ngaba kukho amanqanaba esi sifo?

Ewe, oogqirha basebenzisa i-amyloidosis staging ukuze bafumanise ukuba isifo sisasazeke kangakanani kwaye sinzima kangakanani. Kukho iindlela ezahlukeneyo zokulinganisa iintlobo ezahlukeneyo zesifo. Ukuze ahlele isifo, ugqirha wakho unokuqwalasela oku kulandelayo:

• Ubungakanani bomonakalo kwizitho zomzimba.
• Uvavanyo lwegazi lubonisa ubungakanani beproteni engaqhelekanga xa kuthelekiswa neproteni eqhelekileyo ye-amyloid.
• Iimpawu zakho kunye nobunzima bazo.

Kubaluleke kakhulu ukuthetha nogqirha wakho malunga nenqanaba le-amyloidosis yakho kunye nendlela eliya kuyichaphazela ngayo i-prognosis yakho.

Zeziphi iindlela zonyango ezikhoyo?

Oogqirha balawula i-amyloidosis ngokunyanga imeko engaphantsi. Unyango lunokucothisa ukuqhubela phambili kwe-amyloidosis kwaye luthintele ukwakheka kwee-fibrils ezintsha. Amayeza anokunceda ekunciphiseni iimpawu zakho. Kwi-AL amyloidosis, xa unyango luthintela ukwakheka kweediphozithi ezintsha ze-amyloid, inkqubo yakho yomzimba yokuzikhusela inokususa ezo sele zikhona. Uphando lusaqhubeka lokufumana iindlela zokuphucula inkqubo yokususa i-amyloid kwizitho zakho zomzimba.

Ezinye zeendlela zonyango lwe-amyloidosis zezi:

• Unyango lweKhemotherapy : Olu nyango lutshabalalisa iiseli zeplasma ezingaqhelekanga ezenza iiproteni zechain yokukhanya ezineengxaki ezibangela i-AL amyloidosis. Abantu abaninzi basebenzisa i -steroid kunye namayeza ekhemotherapy.
• Unyango olujoliswe kulo : Olu nyango lujolise kwiiproteni ezithile, ii-genes, okanye izicubu ezibangela i-amyloidosis. Lubandakanya amayeza athintela iiproteni ukuba zingasondeli kakuhle.
• Ukufakelwa amalungu omzimba : Ukuba izintso, isibindi, okanye intliziyo yonakele kangangokuba ayisakwazi ukusebenza kakuhle, oogqirha banokucebisa ukufakelwa amalungu omzimba. Olu lunyango lweentlobo ze-amyloidosis ezizuzwe njengelifa.

Yintoni ekufuneka ixhalatyelwe ngumntu one-amyloidosis?

Ugqirha wakho unokunyanga iimpawu zakho, alawule ukusasazeka kwesifo, kwaye kwezinye iimeko, ancede nasekubuyiseleni esi sifo. Nangona kunjalo, ezinye iintlobo ze-amyloidosis, ukuba azinyangwa, zinokubangela umonakalo osongela ubomi kumalungu omzimba. Yiyo loo nto kubaluleke kakhulu ukuqaphela isifo kwangethuba kwaye uqale unyango ngokukhawuleza.

Ufuna ukuzinyamekela njani nawe?

Ngenxa yokuba kukho iintlobo ezahlukeneyo ze-amyloidosis, akukho ndlela inye yokulawula esi sifo. Buza ugqirha wakho ukuba zeziphi iindlela ezifanelekileyo kuwe.

Oku kungabandakanya:

• Ukunyamekela impilo yakho yomzimba ngokutya ukutya okunesondlo kunye nokuzilolonga rhoqo .
• Beka impilo yakho yengqondo kwindawo yokuqala . Buza ugqirha wakho malunga namaqela enkxaso kubantu abane-amyloidosis. Inokuba yindlela entle yokujongana noxinzelelo kunye nokuba wedwa kokuphila nesifo esingaqhelekanga, kunye nokunxibelelana nabanye abadlula kwinto efanayo nawe.

Ufanele uye nini kugqirha?

Ekubeni iimpawu zahlukile kakhulu, kunokuba nzima ukwazi ukuba uza kubonana nini nogqirha. Kodwa ngokubanzi, ukuba uneempawu ongaziqondiyo kwaye ongazipheliyo emva kwexesha elithile, ngokuqinisekileyo kuya kufuneka ubonane nogqirha. Isenokungabi yi-amyloidosis, kodwa ukuba kunjalo, kokukhona usazi ngokukhawuleza, kokukhona kungcono.

Yeyiphi imibuzo omele uyibuze ugqirha?

Nantsi eminye imibuzo onokuyibuza ugqirha wakho:

• Luhlobo luni lwe-amyloidosis endinalo?
• Kutheni ndifumene i-amyloidosis?
• Luhlobo luni lonyango endiludingayo?
• Zithini iziphumo ebezingalindelekanga zonyango?
• Ithini ingqikelelo yam emva konyango?

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

I-Amyloidosis sisifo esinzima kakhulu kuba sichaphazela wonke umntu ngendlela eyahlukileyo. Kwezinye iimeko, sinokulawuleka. Kwezinye iimeko, sinokuba yingozi ebomini. Oku kunokuba ngamava adidayo kwaye agqithisileyo. Kwakhona, kuba sisifo esingaqhelekanga, ungaziva uwedwa xa ufuna uncedo kakhulu.

Eyona nto ibalulekileyo kukukhumbula ukuba awuwedwa. Ugqirha wakho unokukuchazela iindlela zonyango ezilungileyo kuwe, kuxhomekeke kuhlobo lwe-amyloidosis onayo. Banokukubonisa nezixhobo kunye namaqela enkxaso anokukunceda. Iqela lakho lezonyango linokukukhokela ekufumaneni unyango olungcono kunye nenkxaso oyifunayo ukulwa nesi sifo.

I- Amyloidosis, iiproteni, iiproteni ezingaqhelekanga, umonakalo wamalungu omzimba, isifo sentliziyo, isifo sezintso, izifo zemithambo-luvo