ATTR Amyloidosis: Nā ʻōuli, ʻike, a me nā hana

Anter

• Hōʻike Overview
• Nā Kumu
• Nā ʻano amyloidosis ATTR
• Nā ʻōuli
• ʻIkioma ATTR
• Nā hoʻoponopono
• Outlook

Hōʻike Overview

ʻO Amyloidosis kahi maʻi maʻi laha ʻole e hiki ke loaʻa ke kūkulu ʻia o nā protein amyloid i loko o ke kino. Hiki i kēia mau protein ke kūkulu i loko o nā kīʻaha koko, nā iwi, a me nā mea nui, e alakaʻi ana i ka nui o nā pilikia.

ʻAʻole hiki ke mālama ʻia kēia ʻano paʻakikī, akā hiki ke mālama ʻia ma o nā mālama ʻana. ʻIke ka hōʻoia ʻana a me ka mālama ʻana ma muli o ka paʻakikī o nā ʻōuli a me nā kumu ma waena o nā ʻano amyloidosis. Lōʻihi paha nā manawa e hōʻike.

E heluhelu e aʻo e pili ana i kekahi o nā ʻano maʻamau: amyloid transthyretin (ATTR) amyloidosis.

Nā Kumu

Pili ka ATTR amyloidosis i ka hana kūpono ʻole a me ke kūkulu ʻana o kekahi ʻano amyloid i kapa ʻia ʻo transthyretin (TTR).

Pono kou kino e loaʻa kahi nui kūlohelohe o TTR, i hana mua ʻia e ke ake. Ke komo i ke kahe o ke koko, kōkua ʻo TTR i ka halihali ʻana i nā hormone thyroid a me ka huaora A i loko o ke kino.

Hana ʻia kekahi ʻano TTR i ka lolo. ʻO ia ke kuleana no ka hana ʻana i ka wai cerebrospinal.

Nā ʻano amyloidosis ATTR

ʻO ATTR kekahi ʻano amyloidosis, akā aia kekahi mau subtypes o ATTR.

ʻO Hereditary, a ʻohana ATTR (hATTR a i ʻole ARRTm), holo i loko o nā ʻohana. Ma ka ʻaoʻao ʻē aʻe, ATTR i loaʻa (ʻaʻole hoʻoilina) ʻike ʻia ʻo "ʻano hihiu" ATTR (ATTRwt).

Pili pinepine ʻo ATTRwt me ka ʻelemakule, akā ʻaʻole naʻe me nā maʻi neurological ʻē aʻe.

Nā ʻōuli

ʻOkoʻa nā ʻōuli o ATTR, akā hiki ke hoʻopili ʻia:

• nāwaliwali, ʻoi aku i kou mau wāwae
• ʻōhū ka wāwae a me ka wāwae
• luhi loa
• ʻōlohelohe
• palpitations puʻuwai
• Kaumaha emi
• ʻōpū a me nā pilikia mimi
• libido haʻahaʻa
• nausea
• maʻi maʻi tunnel carpal

ʻO nā kānaka me ka amyloidosis ATTR ʻoi aku ka maʻalahi o ka maʻi puʻuwai, ʻo ia hoʻi me ka ATTR ʻano hihiu. Hoʻomaopopo paha ʻoe i nā hōʻailona e pili ana i ka naʻau, e like me:

• ʻeha ka umauma
• kuʻi ʻole a i ʻole kūpikipiki wikiwiki
• ʻūpā
• pehu
• pokole o ka hanu

ʻIkioma ATTR

ʻIke ka ATTR hiki ke lilo i mea paʻakikī i ka wā mua, ʻoiai ʻo ka nui o kāna mau hōʻailona e hoʻohālikelike i nā maʻi ʻē aʻe. Akā inā he moʻolelo ko kekahi o kou ʻohana o ATTR amyloidosis, pono kēia e kōkua i kāu kauka e hoʻāʻo no nā ʻano amiloidosis. Ma waho aʻe o kāu mau ʻōuli a me ka mōʻaukala olakino pilikino, hiki i kāu kauka ke kauoha i ka hoʻāʻo ʻana i nā kūpuna.

Hiki i nā ʻano hihiu o ATTR ke lilo i mea paʻakikī e kuhi. ʻO kekahi kumu no ka mea like nā ʻōuli me ke kī ʻole o ka puʻuwai congestive.

Inā manaʻo ʻia ʻo ATTR a ʻaʻohe ou moʻolelo ʻohana o ka maʻi, pono e ʻike kāu kauka i ke kū ʻana o nā amyloids i kou kino.

ʻO kekahi ala o ka hana ʻana i kēia ma o kahi scan scintigraphy nukelea. Nānā kēia scanner i nā waihona TTR i loko o kou mau iwi. Hiki i kahi hōʻoia koko ke hoʻoholo inā loaʻa nā waihona i ke kahe o ke koko. ʻO kekahi ala e kuhikuhi ai i kēia ʻano ATTR ma o ka lawe ʻana i kahi mea liʻiliʻi (biopsy) o ka puʻuwai puʻuwai.

Nā hoʻoponopono

ʻElua mau pahuhopu no ka ATTR amyloidosis lapaʻau: e hoʻōki i ka holomua o ka maʻi ma ka palena ʻana i nā waihona TTR, a hoʻoliʻiliʻi i nā hopena a ATTR i loaʻa i kou kino.

Ma muli o ka hopena o ka ATTR i ka puʻuwai, mālama pinepine ʻia nā mālama ʻana no ka maʻi i kēia wahi. Kau kauka paha i ke kauka diuretics e hōʻemi i ka pehu ʻana, a me nā mea lahilahi o ke koko.

ʻOiai e hoʻohālikelike pinepine nā ʻōuli o ATTR i nā maʻi puʻuwai, ʻaʻole hiki i ka poʻe me kēia ʻano ke lawe maʻalahi i nā lāʻau i manaʻo ʻia no ka holo pono ʻole o ka puʻuwai.

Pākuʻi ʻia kēia mau mea me ka aʻa ʻaʻa calcium, nā beta-blockers, a me nā mea kāohi ACE. ʻO ka ʻoiaʻiʻo, hiki i kēia mau lāʻau ke hōʻeha. ʻO kēia kekahi o nā kumu he nui i koʻikoʻi ai kahi hōʻailona kūpono mai ka hoʻomaka.

Kūkā ʻia kahi transplant puʻuwai no nā hihia koʻikoʻi o ATTRwt. ʻO kēia ka hihia inā nui ka hōʻeha o kou puʻuwai.

Me nā hihia hoʻoilina, hiki i kahi transplant ke akepaʻa ke kōkua i ka hoʻōki ʻana i ke kūkulu ʻana o TTR. Eia naʻe, kōkua wale kēia i nā diagnoses mua. E noʻonoʻo paha kāu kauka i nā hoʻoponopono genetic.

ʻOiai ʻaʻohe lāʻau hoʻōla a mālama maʻalahi paha, nui nā lāʻau hou i kēia manawa i nā hoʻokolohua hoʻokolohua, a ke holo nei nā holomua i ka lāʻau. E kamaʻilio me kāu kauka e ʻike inā kūpono kahi hoʻokolohua hoʻokolohua nāu.

Outlook

E like me nā ʻano amyloidosis ʻē aʻe, ʻaʻohe lāʻau no ATTR. Hiki i ke kōkua ke hōʻemi i ka holomua o ka maʻi, ʻoiai ʻo ka mālama ʻana i nā ʻōuli ke hoʻomaikaʻi i kou nohona holoʻokoʻa.

Loaʻa ka wānana maikaʻi ʻo hATTR amyloidosis i ka hoʻohālikelike ʻia me nā ʻano amyloidosis ʻē aʻe no ka mea e hele mālie ana.

E like me nā ʻano olakino, ʻo ka mea ma mua e hoʻāʻo ʻia a ʻike ʻia no ATTR, ʻo ka ʻoi aku ka maikaʻi o ka nānā holoʻokoʻa. Ke aʻo mau nei nā mea noiʻi e pili ana i kēia ʻano, no laila i ka wā e hiki mai ana, e loaʻa nā hopena maikaʻi aʻe no nā subtypes ʻelua.