Paramyloidosis: he aha ia a he aha nā hōʻailona

Anter

• He aha nā hōʻailona
• Nā kumu kūpono
• Pehea e hana ai i ka lāʻau

ʻO Paramyloidosis, i kapa ʻia hoʻi ka maʻi wāwae a i ʻole Familial Amyloidotic Polyneuropathy, kahi maʻi ʻano ʻole i ola ʻole, no ke kumu aʻa, i hōʻike ʻia e ka hana ʻana o nā fibers amyloid e ke akepaʻa, i waiho ʻia i nā aʻa a me nā aʻa, e luku mālie iā lākou.

Ua kapa ʻia kēia maʻi ka maʻi o nā wāwae no ka mea aia i ka wāwae ke ʻike ʻia nā ʻōuli no ka manawa mua a, i ka liʻiliʻi iki, ʻike ʻia ma nā wahi ʻē aʻe o ke kino.

I ka paramyloidosis, kahi hōʻino o nā aʻa āpau ke kumu o nā wahi i hoʻopili ʻia e kēia mau ʻalalā e hoʻopili ʻia, e alakaʻi ana i nā loli i ka ʻike i ka wela, ke anuanu, ʻeha, tactile a me ka haʻalulu. Hoʻohui ʻia, pili pū ka mana kaʻa a nalowale nā ​​mākala i ko lākou puʻupuʻu, ke kaumaha nei i kahi atrophy nui a me ka nalo ʻana o ka ikaika, e alakaʻi ai i ka paʻakikī i ka hele a me ka hoʻohana ʻana i nā lālā.

He aha nā hōʻailona

Hoʻopili ka Paramyloidosis i ka ʻōnaehana nūhou ākea, e alakaʻi ana i ka puka ʻana o:

• Nā pilikia puʻuwai, e like me ke kahe o ke koko, arrhythmia a me nā ālai atrioventricular;
• ʻO ka hana Erectile;
• Nā pilikia Gastrointestinal, e like me ka constipation, diarrhea, fecal incontinence a me ka nausea a me ka luaʻi, ma muli o ka paʻakikī i ka haʻalele ʻana i ka gastric;
• ʻO nā hana urinary, e like me ka paʻa ʻana o ka urinary a me ka hoʻomau ʻole a me nā loli i nā helu filtration glomerular;
• Nā maʻi o ka maka, e like me ka hōʻino ʻana o nā haumāna a me nā makapō āpau.

Eia hou, i kahi pae hopena o ka maʻi, pilikia paha ke kanaka i ka hoʻoliʻiliʻi hoʻoliʻiliʻi, e koi ana i kahi noho huila a i ʻole e noho ana i kahi moena.

Hōʻike maʻamau ka maʻi ma waena o nā makahiki 20 a 40, e alakaʻi ana i ka make 10 a 15 mau makahiki ma hope o ka hoʻomaka ʻana o nā ʻōuli mua.

Nā kumu kūpono

ʻO Paramyloidosis kahi maʻi autosomal mana i hoʻoilina i loaʻa ʻole ka lāʻau lapaʻau a kumu ʻia e kahi hoʻololi kūlohelohe i ka protein TTR, kahi o ka waiho ʻana i nā aʻa a me nā aʻa o kahi mea fibrillar i hana ʻia e ke akepaʻa, i kapa ʻia ʻo amyloid.

ʻO ka waiho ʻana o kēia mea i loko o nā aʻa ke alakaʻi i kahi hōʻemi holomua o ka ʻike i nā stimuli a me ka hiki i ka motika.

Pehea e hana ai i ka lāʻau

ʻO ka lapaʻau kūpono loa no ka paramyloidosis ka transplantation o ke ake, i hiki ai ke hoʻolohi i ka holomua o ka maʻi iki. Hōʻike ʻia ka hoʻohana ʻana o nā lāʻau immunosuppressive i mea e pale aku ai i ke kino o kēlā me kēia mea mai ka hōʻole ʻana i ka mea hou, akā e ala aʻe paha nā hopena ʻoluʻolu ʻole.

Eia hou, hiki i ke kauka ke koi aku i kahi lāʻau, me ka inoa ʻo Tafamidis, kahi e kōkua ai e hoʻolohi i ka holomua o ka maʻi.