Lesi sifo esingatheleleki sibonakala ngokwakhiwa kwama-protein deposits ashintsha ukusebenza okuvamile kwezitho. Imvamisa iqhubekela phambili ibe yifomu elinzima futhi ingathinta zonke izitho zomzimba. Asazi ngokunembile ingqikithi yenani labantu abahlaselwe yilesi sifo esingavamile eFrance, kodwa kulinganiselwa ukuthi sithinta umuntu oyedwa kwabayikhulu. (1) Unyaka ngamunye eFrance, cishe izigameko ezi-100 ezintsha ze-AL amyloidosis zitholwa. uhlobo olungavamile lwesifo.

Ama-amyloidosis deposits angase avele cishe kunoma yisiphi izicubu nezitho zomzimba emzimbeni. I-Amyloidosis ivame ukuqhubekela esimweni esinzima ngokubhubhisa kancane kancane izitho ezithintekile: izinso, inhliziyo, umgudu wokugaya ukudla kanye nesibindi yizitho ezivame ukuthinteka. Kodwa i-amyloidosis ingathinta namathambo, amalunga, isikhumba, amehlo, ulimi ...

Ngakho-ke, uhla lwezimpawu lubanzi kakhulu: ukungakhululeki nokuphefumula lapho inhliziyo ibandakanyeka, i-edema yemilenze maqondana nezinso, ukwehla kwethoni yemisipha lapho kuthinteka izinzwa ze-peripheral, isifo sohudo / ukuqunjelwa kanye nokuvinjwa. izinso. umgudu wokugaya ukudla, njll. Izimpawu ezibucayi njengokuphelelwa umoya, isifo sohudo nokopha kufanele ziqaphe futhi ziholele ekuhloleni i-amyloidosis.

I-Amyloidosis ibangelwa ukushintsha ukwakheka kwamangqamuzana amaprotheni, okwenza ingancibiliki emzimbeni. Iphrotheni ibe isidala ama-molecular deposits: i-amyloid substance. Kulungile ukukhuluma nge-amyloidosis kune-amyloidosis, ngoba nakuba lesi sifo sinesizathu esifanayo, izinkulumo zaso zihluke kakhulu. Eqinisweni, cishe amaprotheni angamashumi amabili anesibopho sezinhlobo eziningi eziqondile. Kodwa-ke, kunezinhlobo ezintathu ze-amyloidosis: AL (immunoglobulinic), AA (ukuvuvukala) kanye ne-ATTR (transthyretin).

• I-AL amyloidosis iphumela ekuphindaphindeni kwamaseli athile amhlophe egazi (amaseli e-plasma). Akhiqiza amasosha omzimba (ama-immunoglobulins) azohlanganisa futhi enze amadiphozithi.
• I-AA amyloidosis yenzeka lapho ukuvuvukala okungapheli kubangela ukukhiqizwa okuphezulu kwamaprotheni e-SAA azokwakha i-amyloidosis ezicutshini.
• I-transthyretin protein ibandakanyeka ku-ATTR amyloidosis. Lolu hlobo lwesifo luwufuzo. Umzali othintekile unengozi engu-50% yokudlulisela uguquko enganeni yakhe.

I-Amyloidosis ayitheleleki. Idatha ye-Epidemiological ibonisa ngokucacile ukuthi ingozi iyanda ngokukhula (i-amyloidosis ivame ukutholakala eminyakeni engama-60-70). Abantu abanesifo esithathelwanayo noma esivuvukalayo, kanye nalabo asebene-dialysis ende, basengozini enkulu. Njengoba ezinye izinhlobo ze-amyloidosis, njenge-ATTR amyloidosis, zixhunyaniswa nokuguqulwa kwezakhi zofuzo, imindeni lapho ilungu elilodwa noma ngaphezulu liye lathinteka khona kufanele kube yisihloko sokuqapha okukhethekile.

Kuze kube manje, azikho izindlela zokuvimbela ukulwa ne-amyloidosis. Ukwelashwa konke kuhlanganisa ukunciphisa ukukhiqizwa kwephrotheni enobuthi efakwa futhi ihlanganiswe ezicutshini, kodwa ziyahlukahluka kuye ngohlobo lwe-amyloidosis:

• nge-chemotherapy esimweni se-AL amyloidosis. Izidakamizwa ezisetshenziswe embukisweni wakhe wokwelapha ” ukusebenza kahle kwangempela », Iqinisekisa i-National Society of Internal Medicine (SNFMI), ngenkathi icacisa ukuthi ukuhlasela kwenhliziyo kuhlala kubi.
• usebenzisa izidakamizwa ezinamandla zokulwa nokuvuvukala ukulwa nokuvuvukala okubangele i-AA amyloidosis.
• Nge-ATTR amyloidosis, ukufakelwa kabusha kungase kudingeke esikhundleni sesibindi esifakwe iphrotheni enobuthi.

I-French Association against Amyloidosis ishicilela ulwazi oluningi ngalesi sifo kanye nokwelashwa kwaso, okuhloselwe iziguli nezihlobo zazo.