I-Paramyloidosis: yini futhi yiziphi izimpawu

-Delile

• Ziyini izimpawu
• Izimbangela ezingaba khona
• Yelashwa kanjani

I-Paramyloidosis, esibizwa nangokuthi isifo sezinyawo noma i-Familial Amyloidotic Polyneuropathy, yisifo esingajwayelekile esingenalo ikhambi, esivela kofuzo, esivezwa ukukhiqizwa kwemicu ye-amyloid yisibindi, efakwa ezicutshini nasemithanjeni yezinzwa, ibabhubhise kancane.

Lesi sifo sibizwa ngokuthi yisifo sezinyawo ngoba sisezinyaweni lapho izimpawu zivela khona okokuqala kuthi, kancane kancane, zivele kwezinye izindawo zomzimba.

Ku-paramyloidosis, ukukhubazeka kwemizwa yezimbangela kubangela ukuthi izindawo ezithonywe yilezi zinzwa zithinteke, okuholele kuzinguquko ekuzweleni ekushiseni, emakhazeni, ebuhlungwini, ethintekayo nasekudlidlizeleni. Ngaphezu kwalokho, amandla emoto nawo ayathinteka futhi imisipha ilahlekelwa yisisindo semisipha, ihlupheke kakhulu futhi iphelelwe ngamandla, okuholela ebunzimeni bokuhamba nokusebenzisa izitho.

Ziyini izimpawu

I-Paramyloidosis ithinta uhlelo lwe-peripheral nervous, okuholela ekuveleni kwe:

• Izinkinga zenhliziyo, ezifana nomfutho wegazi ophansi, i-arrhythmias kanye nokuvinjelwa kwama-atrioventricular;
• Ukungasebenzi kwe-Erectile;
• Izinkinga zamathumbu, njengokuqunjelwa, isifo sohudo, ukungasebenzi komgogodla kanye nesicanucanu nokuhlanza, ngenxa yobunzima bokukhipha isisu;
• Ukungasebenzi kahle komchamo, njengokugcinwa komchamo nokungavinjelwa kanye nezinguquko kumazinga wokuhlunga we-glomerular;
• Ukuphazamiseka kwamehlo, njengokuwohloka komfundi nobumpumputhe obulandelayo.

Ngaphezu kwalokho, esigabeni esibulalayo sesifo, umuntu angahle ahlushwe ukuhamba okuncishisiwe, okudinga isihlalo sabakhubazekile noma ukulala embhedeni.

Lesi sifo sivame ukuvela phakathi kweminyaka engama-20 nengama-40, siholele ekufeni eminyakeni eyi-10 kuya kwengu-15 ngemuva kokuqala kwezimpawu zokuqala.

Izimbangela ezingaba khona

I-Paramyloidosis yisifo esizuzwe njengefa se-autosomal esingenalo ikhambi futhi sidalwa ukuguqulwa kwezakhi zofuzo kuphrotheni ye-TTR, equkethe ukufakwa kwezicubu nezinzwa zento ye-fibrillar ekhiqizwa isibindi, ebizwa nge-amyloid.

Ukufakwa kwalesi sici ezicutshini kuholela ekwehleni okuqhubekayo kokuzwela kukhuthazo namandla emoto.

Yelashwa kanjani

Ukwelashwa okuphumelela kakhulu kwe-paramyloidosis ukufakelwa kwesibindi, okwazi ukubamba kancane ukukhula kwesifo. Ukusetshenziswa kwemithi yokuzivikela emzimbeni kukhonjiswa ukuvimbela umzimba womuntu ukwenqaba isitho esisha, kepha kungavela imiphumela emibi.

Ngaphezu kwalokho, udokotela futhi angancoma umuthi, onegama elithi Tafamidis, elisiza ukubambezela ukuqhubeka kwesifo.