Paramyloidosis: yintoni kwaye yintoni iimpawu

Umxholo

• Ziintoni iimpawu
• Izizathu ezinokubangela
• Unyango lwenziwa njani

I-Paramyloidosis, ekwabizwa ngokuba sisifo seenyawo okanye iFamilial Amyloidotic Polyneuropathy, sisifo esinqabileyo esinganyangekiyo, esinemvelaphi yemfuza, esibonakaliswa kukuveliswa kwemicu ye-amyloid sisibindi, efakwa kwizicwili kunye nemithambo-luvo, iyitshabalalise kancinci.

Esi sifo sibizwa ngokuba sisifo seenyawo kuba kusezinyaweni apho iimpawu zibonakala khona okokuqala kwaye, kancinci kancinci, ziyavela nakwezinye iindawo zomzimba.

Kwi-paramyloidosis, ukungahambi kakuhle kwemithambo-luvo kubangela ukuba iindawo ezingafakwanga yimithambo-luvo zichaphazeleke, zikhokelela kutshintsho kubuntununtunu kubushushu, ukubanda, iintlungu, ukungathambisi nokungcangcazela. Ukongeza, amandla emoto nawo ayachaphazeleka kwaye izihlunu ziphulukana nobunzima bemisipha, zifumana i-atrophy enkulu kunye nelahleko yamandla, ekhokelela kubunzima ekuhambeni nasekusebenziseni amalungu.

Ziintoni iimpawu

Iparamyloidosis ichaphazela inkqubo yemithambo-luvo, ekhokelela ekuveleni:

• Iingxaki zentliziyo, ezinje ngoxinzelelo lwegazi, arrhythmias kunye nezithintelo zeatrioventricular;
• Ukungasebenzi kakuhle kwe-erectile;
• Iingxaki zesisu, ezinjengokuqhinwa, urhudo, ukungabinako ukubamba ilindle kunye nokugabha kunye nokugabha, ngenxa yobunzima bokukhupha isisu;
• Ukungasebenzi kakuhle komchamo, njengokugcinwa komchamo kunye nokungavisisani kunye notshintsho kumanqanaba okucoca ngokuqaqamba;
• Ukuphazamiseka kwamehlo, njengokuwohloka komfundi kunye nokungaboni.

Ukongeza, kwinqanaba lesifo, umntu unokufumana ubunzima ekunciphiseni ukuhamba, efuna isitulo esinamavili okanye ukuhlala ebhedini.

Esi sifo sihlala sibonakala phakathi kweminyaka engama-20 nengama-40, sikhokelela ekufeni kwiminyaka eli-10 ukuya kweli-15 emva kokuqala kweempawu zokuqala.

Izizathu ezinokubangela

Iparamyloidosis sisifo esizimeleyo esingena nyango kwaye sibangelwa kukutshintsha kwemvelo kwiprotheyini ye-TTR, equlathe ukufakwa kwizicwili kunye nemithambo yento ye-fibrillar eveliswa sisibindi, ebizwa ngokuba yi-amyloid.

Ukubekwa kwale nto kwizihlunu kukhokelela ekunciphiseni okuqhubekayo kobuntununtunu ukukhuthaza amandla emoto.

Unyango lwenziwa njani

Olona nyango lusebenzayo lweparamyloidosis kukufakelwa kwesibindi, okwaziyo ukucothisa ukuqhubela phambili kwesi sifo kancinci. Ukusetyenziswa kwamachiza okunyanga ukubonwa komzimba kubonisiwe ukunqanda umzimba womntu ukwala ilungu elitsha, kodwa iziphumo ezingathandekiyo zinokuvela.

Ukongeza, ugqirha unokucebisa amayeza, anegama elithi Tafamidis, elinceda ukucothisa inkqubela phambili yesi sifo.