E aʻo kākou e pili ana i nā protein ʻē i waiho ʻia i loko o ka puʻuwai? (Cardiac Amyloidosis)

Ke luhi nei ʻoe i kekahi manawa a i ʻole he pilikia iki kou hanu ʻana? ʻAʻole paha ʻoe e nānā nui i kēia mau mea. Akā i kekahi manawa hiki i kēia ke lilo i hōʻailona o ka pilikia o ka puʻuwai. I kēia lā e kamaʻilio mākou e pili ana i kahi kūlana e hoʻopilikia ai i ka puʻuwai, kahi āu i lohe ʻole ai , akā he mea kūpono loa ia e ʻike. Mai hopohopo, e kamaʻilio mākou e pili ana i kēia me ka maʻalahi a ma ke ʻano e hiki ai iā ʻoe ke hoʻomaopopo.

He aha kēia (Cardiac Amyloidosis)?

I ka ʻōlelo maʻalahi, ʻo ka `(Cardiac Amyloidosis)` he maʻi ia i hoʻokumu ʻia e nā protein ʻano ʻē i loko o ko kākou kino, e waiho ana i loko a puni ka puʻuwai. E noʻonoʻo iā ia e like me kahi poloka i loko o kahi paipu wai. ʻO kēia hōʻiliʻili ʻana o nā protein e hōʻemi ana i ka hiki o kou puʻuwai ke pamu i ke koko. A laila pono ka puʻuwai e hana ikaika. I ka hopena, hiki i kēia hoʻoikaika hou ke hoʻonāwaliwali a hōʻino i ka puʻuwai, e alakaʻi ana i ka `(Heart Failure)`.

Nui nā kumu no kēia. Hoʻoilina kekahi poʻe mai ko lākou mau mākua mai, ʻo ia hoʻi he genetic ia. Hiki i kekahi ke loaʻa i ke kūlana me ka ʻole o kahi loulou genetic. Hiki ke hana ʻia e nā maʻi ʻē aʻe, e like me ke kanesa. ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia kēia kūlana, i kapa ʻia ʻo "Amyloidosis", aia nā lāʻau lapaʻau maikaʻi no kekahi mau ʻano.

He aha nā ʻano o ka Amyloidosis Cardiac?

He mau kaulahao lōʻihi o nā molekala nā protein i loko o ko kākou kino. Kōkua kēia mau protein iā kākou e hana i nā mea he nui, me ka hāʻawi ʻana i ka ikehu, ka hoʻokō ʻana i nā hopena kemika i loko o ko kākou kino, a me ke kamaʻilio ʻana i nā leka ma waena o nā ʻōnaehana like ʻole. Akā, aia kekahi pilikia. Hiki i kā kākou mau cell ke hoʻohana wale i kēia mau kaulahao protein inā aia lākou i ke ʻano kūpono. ʻO nā maʻi pelu hewa protein, e like me ka amyloidosis, ke kumu o kēia mau protein e wili ʻia, pelu hewa ʻia, a hiki ʻole ke hana pono.

Ke huikau loa nā protein, huikau lākou a ʻaʻole hiki i ke kino ke hoʻohana iā lākou. Ke ʻole kahi e hele ai, hui pū kēia mau protein i huikau ʻia, pili pū, a paʻa i nā ʻāpana like ʻole o ke kino.

Hiki i ka Amyloidosis ke hoʻopilikia i nā ʻōnaehana he nui i loko o ke kino, ʻoiai ka puʻuwai. ʻEkolu mau ʻano nui o ka amyloidosis e hoʻopilikia i ka puʻuwai (hiki i nā ʻano ʻē aʻe ke hoʻopilikia i ka puʻuwai, akā ʻoi aku ka kakaikahi).

ʻAmyloidosis AL (Kaulahao Māmā)

Hoʻohana ko mākou kino i nā mea i kapa ʻia ʻo "nā kaulahao māmā" ma ke ʻano he ʻāpana o kāna ʻōnaehana pale. Mālama kēia iā mākou mai nā germs e like me nā virus a me nā bacteria. Hana ʻia ka "AL Amyloidosis" i ka wā e hana hewa ai nā cell i loko o ko mākou iwi iwi a hana i ka nui o kēia mau "kaulahao māmā." Hiki ke hana kēia ma kāna iho, a i ʻole hiki ke hana ʻia e kekahi mau ʻano maʻi kanesa koko a i ʻole nā ​​​​maʻi kanesa o ka ʻōnaehana pale. ʻIke pinepine ʻia kēia ʻano ma hope o 50 mau makahiki.

ʻO ka amyloidosis ATTR

ʻO Transthyretin (TTR) kahi protein e kōkua i ka hoʻoneʻe ʻana i kekahi mau kemika a puni i loko o ko kākou kino. Loaʻa kona inoa no ka mea lawe ia i ka hormone thyroid a me ka huaora A (retinol). Ua kapa ʻia ʻo ia ma mua ʻo Thyroxine-binding prealbumin (TBPA), akā ʻaʻole hoʻohana hou ʻia kēlā inoa.

He ʻokoʻa loa nā hōʻailona o ka `ATTR Amyloidosis` . Hiki ke ʻike ʻia i ka wā ʻōpiopio o 20 mau makahiki a i ʻole a hiki i ka 70 mau makahiki. Aia ʻelua mau ʻano nui:

• ʻO ka ATTR Hoʻoilina: He kūlana hoʻoilina kēia. ʻO ke ʻano kēia, hoʻoilina ʻoe mai kou makuahine, makuakāne, a i ʻole nā ​​mea ʻelua.
• ʻAno hihiu ATTR: Hana ʻia kēia ke hoʻomaka wale ka protein TTR e hana hewa, me ka ʻole o kekahi hoʻololi ʻana o ka genetic. Hana pinepine ʻia kēia ma hope o 60 mau makahiki. Ua kapa ʻia ʻo ia ma mua ʻo Senile Systemic Amyloidosis a i ʻole Senile Cardiac Amyloidosis, akā ʻaʻole hoʻohana pinepine ʻia kēia mau inoa.

ʻO ka amyloidosis e pili ana i ka dialysis

ʻO ka poʻe i noho ma ka dialysis no nā makahiki ma muli o ka maʻi puʻupaʻa, ua ʻoi aku ka nui o ka pilikia o ka loaʻa ʻana o ka amyloidosis. ʻO kēia no ka mea, ʻaʻole i kānana ʻia kahi protein i kapa ʻia ʻo beta-2 microglobulin (beta-2 microglobulin) i ka wā o ke kaʻina hana dialysis. Hōʻiliʻili kēia protein, i ʻike ʻia ʻo beta-2Ms, i loko o ka puʻuwai a me nā ʻāpana ʻē aʻe o ke kino.

Nā ʻano laha ʻole (Cardiac Amyloidosis)

Ma waho aʻe o kēia, aia kekahi mau ʻano ʻē aʻe o ka "Cardiac Amyloidosis", akā he mea kakaikahi loa lākou.

ʻO wai ka mea i ʻoi aku ka paʻakikī ke hoʻomohala i kēia?

Aia kekahi mau kumu nui e hoʻopilikia ai i ka mea nāna i hoʻomohala i kēia maʻi:

• Makahiki: ʻOiai hiki i kekahi poʻe ke loaʻa i ka amyloidosis i ko lākou mau makahiki 20, he mea kakaikahi ia i ka poʻe ma lalo o 40 makahiki. Hoʻomaopopo pinepine ʻia ka maʻi ma hope o 50 makahiki, ʻoi aku hoʻi ma ke ʻano o ka amyloidosis ATTR wild-type.
• Kāne: ʻOi aku ka nui o ka loaʻa ʻana o ka amyloidosis cardiac i nā kāne ma mua o nā wahine. ʻOi aku hoʻi me ka amyloidosis ATTR wild-type, aia he 25 a 50 mau kāne i hoʻopilikia ʻia no kēlā me kēia wahine.
• ʻĀina hānau: ʻO kahi loli genetic e hoʻoulu ai i ka familial ATTR amyloidosis he mea maʻamau loa ia ma kekahi mau ʻāina. No ka laʻana, nā ʻāina e like me Pokukala, Iapana, Suedena, Pinilana, a me kekahi mau ʻāina ʻEulopa ʻē aʻe.
• Lāhui/ʻano lāhui: ʻO kekahi hoʻololi ʻano genetic e hoʻoulu ai i ka familial ATTR amyloidosis i loaʻa ma kahi o 4% o nā kānaka ʻeleʻele o ka hanauna Komohana o ʻApelika. Hoʻokomo pū kēia me nā ʻAmelika ʻApelika a me nā kānaka ʻeleʻele mai Latin America a me Caribbean.
• ʻO ka dialysis hoʻomau no nā makahiki: ʻO ka lōʻihi o kou noho ʻana ma ka dialysis, ʻo ka nui aʻe o kou loaʻa ʻana o kēia mau hōʻailona.

Pehea ka maʻamau o kēia maʻi?

Ma keʻano laulā, he maʻi laha ʻole ka ʻAmyloidosis. Eia nō naʻe, ʻoi aku ka maʻamau o kekahi mau ʻano o ka ʻAmyloidosis ma mua o nā mea ʻē aʻe.

• AL Amyloidosis: Ma ʻAmelika Hui Pū ʻIa wale nō, ua hōʻike ʻia ma kahi o 4,000 mau hihia hou o AL Amyloidosis i kēlā me kēia makahiki. ʻO ia hoʻi ma kahi o hoʻokahi i loko o 64,500 mau mākua.
• ʻO ka ʻAmyloidosis Hoʻoilina: Hiki ke ʻike ʻia kēia ʻano ma kahi o 4% o ka poʻe ʻeleʻele o ka hanauna Komohana o ʻApelika. I ka poʻe o ka hanauna ʻEulopa, ʻike ʻia ia ma kahi o hoʻokahi i loko o hoʻokahi haneli tausani. Eia nō naʻe, ma nā ʻāina i ʻōlelo ʻia ma luna, ʻoi aku ka kiʻekiʻe o kēia helu. No ka laʻana, ma Pokukala, ma kahi o hoʻokahi i loko o 538 mau kānaka i loaʻa i kēia ʻano.
• ʻO ka Amyloidosis ATTR ʻano hihiu: Manaʻo nā loea he maʻi i hōʻike ʻole ʻia kēia, me nā manaʻo e hōʻike ana e hiki ke hoʻopilikia i ka 20% o nā kāne ma luna o 80 mau makahiki.
• ʻO ka amyloidosis e pili ana i ka dialysis: Loaʻa kēia ma kahi o 20% o ka poʻe ma ka dialysis no ʻelua a ʻehā mau makahiki. Loaʻa ia i ka aneane o nā kānaka a pau ma ka dialysis no 13 mau makahiki a ʻoi aku paha.

Pehea ka hopena o kēia maʻi i ke kino?

ʻO ka Cardiac Amyloidosis kahi maʻi e hoʻololi i ke ʻano o kou puʻuwai a hoʻopilikia i kona hiki ke pamu. Hana ʻia ia ma nā ʻano penei:

• Mānoanoa o nā paia o ka puʻuwai: Ke waiho ʻia nā protein `(Amyloid)` i loko o ka ʻiʻo o ka puʻuwai, mānoanoa nā paia o ka puʻuwai a hoʻonui ʻia ka puʻuwai. Ke hana ʻia kēia, pono ka puʻuwai e hana ikaika e pamu i ke koko ma ke kino holoʻokoʻa. ʻO kēia hoʻoikaika hou aʻe e alakaʻi ai i ka `(Heart Failure)` .
• Hiki i ka cardiac amyloidosis ke hana i nā ʻano puʻuwai maʻamau ʻole (arrhythmias), a i ʻole nā ​​hōʻailona uila nāwaliwali i loko o ka puʻuwai. ʻO ka atrial fibrillation kahi ʻano maʻamau o ka arrhythmia e pili ana me ka amyloidosis.
• Nā waihona Amyloid: He mau protein waxy a sticky nā protein Amyloid e hui pū maʻalahi a hana i nā puʻupuʻu nui. Inā paʻa kēia mau puʻupuʻu i loko o ka valve puʻuwai, hiki ke ālai ʻia ka valve, e kāohi ana i ke kahe koko.

He aha nā hōʻailona?

ʻAʻole nā ​​kānaka a pau i loaʻa i kahi hoʻololi genetic e hiki ai ke hana i ka amyloidosis e loaʻa i ka maʻi. ʻAʻole loa e loaʻa i kekahi poʻe. Loaʻa i kekahi, akā ʻaʻole ia he koʻikoʻi.

ʻO ka maʻamau, pili nā hōʻailona o ka Cardiac Amyloidosis i ka puʻuwai, akā hiki ke pili pū i nā ʻāpana koʻikoʻi ʻē aʻe e like me ke akepaʻa a me nā puʻupaʻa. I nā pae hope loa o ka Cardiac Amyloidosis, hiki ke ʻike ʻia nā hōʻailona o ka hāʻule nui o ka puʻuwai (i helu ʻia ma lalo nei).

ʻO nā hōʻailona kūpono paha:

• Pōkole o ka hanu: Hiki ke hana kēia i ka wā e hana ana ʻoe a i ʻole ʻoe e moe ana.
• Ka pehu ʻana ma muli o ka hōʻiliʻili ʻana o ka wai: ʻIke pinepine ʻia kēia ma nā wāwae, nā kuʻekuʻe wāwae, ka ʻūhā, a me ka ʻōpū.
• Luhi:Manaʻo nui i ka luhi no kekahi mau lā a ʻoi aku paha.
• Nā Palpitations o ka Puʻuwai: He manaʻo maikaʻi ʻole āu e manaʻo ai me he mea lā e kuʻi ana kou puʻuwai me ka ʻole o kou hoʻoikaika ʻana.
• ʻO nā aʻa koko ma ka ʻāʻī: Hana ʻia kēia ke hāʻule ka puʻuwai, no ka mea, he paʻakikī ke pamu pono. Hoʻokomo pū kēia kaomi i ke kaomi ma luna o nā aʻa koko ma ka ʻāʻī, e hoʻonui ai iā lākou.
• Hepatomegaly: He pilikia maʻamau kēia o ka hāʻule ʻana o ka puʻuwai, a ʻo ia ke kumu like e hoʻonui ai i nā aʻa koko ma ka ʻāʻī. ʻO ka piʻi ʻana o ke kaomi mai ka puʻuwai e kau pū i ke kaomi ma luna o nā kīʻaha koko i loko o ke akepaʻa, e hoʻoulu ai i ka pehu ʻana o ke kino.
• Nā pilikia puʻupaʻa: Nā mea e like me ka mumū o nā puʻupaʻa a i ʻole nā ​​​​loli i ka hana o nā puʻupaʻa.

ʻO nā mea ʻē aʻe e hiki ke lilo i mau hōʻailona o ka "Cardiac Amyloidosis" ʻaʻole pili i ka puʻuwai, ke akepaʻa, a i ʻole nā ​​puʻupaʻa:

• ʻEha ʻano ʻē.
• Alelo pehu.
• ʻO ka Carpal Tunnel Syndrome (Pulmonary Allerve Compression - Hiki i kēia ke lilo i hōʻailona mua, pinepine i nā makahiki ma mua o ka puka ʻana o ka maʻi).
• ʻO ka Lumbar Spinal Stenosis (ka haiki ʻana o ka hakahaka a puni ke kaula spinal ma ke kua haʻahaʻa).
• Nā pilikia ʻike.
• Nā pilikia lohe a me ke kuli.
• ʻO ka maneʻo a i ʻole ke ʻāʻī ʻana i nā lima a i ʻole nā ​​wāwae.

He aha nā kumu o ka Cardiac Amyloidosis?

Nui nā kumu like ʻole o nā ʻano amyloidosis like ʻole, akā pili ka hapa nui o lākou i kā mākou DNA .

E noʻonoʻo e like me ka puke kuke ʻohana. Aia i loko nā kuhikuhi no ke ʻano o ka hana ʻana o kā mākou mau cell, pehea e hana ai i kekahi mau protein. ʻO ka hoʻoili ʻana i ka DNA mai nā mākua a i nā keiki ua like ia me kou mau mākua e kākau lima ana i kēlā puke meaʻai iā ʻoe.

Ua like nā hoʻololi ʻana o ka genetic me nā typos i loko o kāu puke kuhikuhi DNA. Hiki i kahi leka nui ke hoʻopilikia i kahi kuhikuhi. Ua like ia me nā maʻi e like me ka amyloidosis. Ke hana nei kāu mau cell i ka ikaika e like me kā lākou hiki, akā ʻike wale lākou pehea e hahai ai i ke kuhikuhi. Hiki i kēia mau typos ke hana iā ʻoe ma nā ʻano ʻelua.

Hoʻoilina

He maʻi ʻohana pinepine ka Cardiac Amyloidosis. ʻO ia hoʻi, hoʻoilina ʻoe iā ia. Hana ʻia kēia ke loaʻa i kekahi a i ʻole ʻelua o kou mau mākua kahi mutation i kā lākou DNA, a ua kope ʻia ka hewa i loko o kēlā "puke kuhikuhi" i kāu.

Loaʻa

ʻO nā maʻi i loaʻa ʻia he mau maʻi ia āu e hoʻoilina ʻole ai, akā e ulu ana i kekahi manawa o kou ola. ʻO ka amyloidosis ATTR ʻano hihiu a me ka amyloidosis e pili ana i ka dialysis nā laʻana o kēia mau maʻi i loaʻa. Akā, ʻaʻole pili kekahi o kēia mau mea i nā mutations i loko o kāu DNA.

• Loaʻa ka amyloidosis ATTR ʻano hihiu no ka mea hana pololei kou kino i nā protein, akā i ka hala ʻana o ka manawa, lilo kēlā mau protein i mea paʻa ʻole (ʻo ia ke kumu e pili ai kēia maʻi i ka ʻelemakule). I ka hopena, pelu wale ka protein i ka hewa.
• ʻO ka amyloidosis e pili ana i ka dialysis ke hōʻiliʻili ʻia kahi protein maʻamau i hiki ʻole ke kānana ʻia e ka dialysis, e like me kou mau puʻupaʻa, i loko o kou kino. I ka hala ʻana o ka manawa, hōʻiliʻili kēlā protein a hiki i kahi e hoʻoulu ai i nā pilikia.

He mea laha anei kēia?

ʻAʻole, ʻaʻole he maʻi lele ka ʻAmyloidosis ma kekahi ʻano.

Pehea e ʻike ai ke kauka i kēia? (Diagnosis)

E kānalua mua ke kauka i ka '(Cardiac Amyloidosis)' ma muli o kāu mau hōʻailona, ​​​​kahi hoʻokolokolo kino (nānā ke kauka, manaʻo, a hoʻolohe i kou kino no nā hōʻailona o ka maʻi), a me kou mōʻaukala olakino ʻohana. A laila e hana lākou i nā hoʻokolohua diagnostics, nā hoʻokolohua kiʻi, a me nā hoʻokolohua lab e hōʻoia inā loaʻa iā ʻoe ka '(Cardiac Amyloidosis)' a i ʻole.

Inā loaʻa i kekahi o kou ʻohana kahi ʻano ʻohana o ka amyloidosis, ʻaʻole paha ʻoe e pono i kekahi mau hoʻokolohua a i ʻole pono paha e loaʻa nā hoʻokolohua ʻē aʻe. Hiki ke hana ʻia nā hoʻāʻo genetic e ʻike inā he mau hoʻololi genetic kou e hiki ai ke hoʻoulu i ka amyloidosis cardiac.

He aha nā hoʻokolohua e hana ʻia e ʻike ai i kēia maʻi?

Inā kānalua kāu kauka he "Cardiac Amyloidosis" kou, hiki iā lākou ke kauoha i hoʻokahi a ʻoi aku paha o nā hoʻokolohua ma lalo nei.

Hoʻāʻo ʻana ma ka hale hana

Lawe kēia mau hoʻokolohua i nā laʻana o nā ʻiʻo, ke koko, a i ʻole nā ​​​​wai kino ʻē aʻe. Hoʻohana ʻia kahi kala kūikawā i kapa ʻia ʻo Congo red i nā hoʻokolohua he nui. Hoʻāla kēia kala i nā wahi i waiho ʻia ai ka amyloid e ʻālohilohi i ka ʻōmaʻomaʻo ma lalo o kekahi mau kūlana kukui.

• Biopsy ʻiʻo: Ma kēia, lawe nā kauka i kahi laʻana liʻiliʻi o ka ʻiʻo mai kahi wahi, e like me ka ʻiʻo puʻuwai, a hoʻāʻo iā ia i loko o kahi keʻena hoʻokolohua. Hiki ke lawe ʻia nā laʻana ʻiʻo mai nā wahi ʻē aʻe, akā inā hāʻawi kahi laʻana mai kahi wahi ʻaʻole puʻuwai i kahi hopena maikaʻi, pono e hōʻoia ʻia me nā hoʻokolohua kiʻi.
• Hoʻāʻo koko: I kekahi mau ʻano amyloidosis cardiac, ke kaʻapuni nei nā protein amyloid i loko o ke koko. Hiki i nā hoʻokolohua lab ke ʻike i kēia mau protein.
• Hoʻāʻo ʻana i ka mimi: I kekahi mau hihia, loaʻa paha nā protein amyloid i loko o ka mimi. Hiki i ka hoʻāʻo ʻana no ke alo o kēia mau protein i loko o ka mimi ke kōkua i ka ʻike ʻana.

Nā hoʻokolohua kiʻi

Ma muli o ka hiki i ka amyloidosis cardiac ke hoʻonui i kou puʻuwai a hoʻololi i ke ʻano, hiki i nā hoʻokolohua kiʻi ke kōkua i ka ʻike ʻana i ke kūlana. ʻO nā hoʻokolohua e hiki ke hana ʻia:

• Ka helu ʻikepili o ke kaʻa:Ua like kēia hoʻāʻo me kahi ʻōpeʻapeʻa e hoʻohana ana i ke kani e ʻimi i kona ala. Hoʻohana ia i nā nalu kani alapine kiʻekiʻe. Hoʻokomo ʻia kahi mea e hoʻopuka ana i ke kani ma ka ʻili o ka umauma, a hiki i nā kauka ke "ʻike" i ka puʻuwai ma ka nānā ʻana i ke ʻano o ka lele ʻana o nā nalu kani mai nā ʻano like ʻole i loko o ke kino. He mea pono loa kēia no ka ʻike ʻana inā he mānoanoa loa nā ʻāpana o ka puʻuwai.
• Scintigraphy: No kēia hoʻāʻo, hoʻokomo nā kauka i kahi tracer, kahi mea radioactive iki (akā ʻaʻole ia he mea ʻino), i loko o kou koko. Hōʻiliʻili ka tracer ma nā wahi i hōʻiliʻili ʻia ai ka protein amyloid. A laila hiki ke ʻike maʻalahi ʻia kēia mau wahi me kahi hoʻāʻo kiʻi i kapa ʻia ʻo SPECT scan (Single-Photon Emission Computed Tomography) .
• ʻO ke Kiʻi Resonance Magnetic (MRI): Hoʻohana kēia hoʻāʻo i kahi magnet ikaika loa a me ka ʻenehana kamepiula e hana i kahi kiʻi o ka puʻuwai. He kikoʻī loa ka MRI, no laila hiki i nā kauka ke ʻike i ka mānoanoa a i ʻole nā ​​​​loli ʻē aʻe i loko o ka puʻuwai.

Electrocardiogram (ECG a i ʻole EKG)

I loko o kahi ECG, ua hoʻopili ʻia kekahi mau sensor (ʻo ka maʻamau he 12) i ka ʻili o kou umauma. ʻIke nā sensor i ke au uila e kahe ana ma kou puʻuwai a hōʻike i ka ikaika o kēlā hōʻailona uila ma ke ʻano he nalu ma ka pepa a i ʻole kahi pale. Hiki i nā loea i aʻo ʻia, e like me nā kauka, ke kālailai i ka nalu e ʻike inā he mau loli ʻē i ke ʻano o kekahi mau ʻāpana o kou puʻuwai e alakaʻi ai i ka uila.

Nā hoʻokolohua genetic

Ma muli o nā hopena o nā hoʻokolohua ʻē aʻe, hiki i kāu kauka ke ʻōlelo aku i ka hoʻāʻo genetic. Hiki i kēia mau hoʻokolohua ke ʻike i nā mutations i loko o kāu DNA e hiki ke kumu o kāu amyloidosis.

Aia kekahi lāʻau lapaʻau no ka Amyloidosis?

Hiki ke mālama ʻia nā ʻano he nui o ka `(Cardiac Amyloidosis)`, akā ʻaʻole hiki ke ho'ōla piha ʻia ka maʻi. ʻOkoʻa nō hoʻi ka lāʻau lapaʻau ma muli o ke ʻano o ka `(Amyloidosis). Ma ke ʻano laulā, he mea nui loa ka ʻike mua ʻana . Inā loaʻa koke ka `(Amyloidosis)`, hiki ke kaupalena ʻia ka hōʻino lōʻihi i ka puʻuwai. Inā ʻaʻole i ʻike ʻia a mālama ʻia i ka wā mua, hiki ke loaʻa ka hōʻino mau loa. ʻO ke ala wale nō e hoʻoponopono ai i ka hōʻino mau loa o ka puʻuwai he `(Heart Transplant)`.

ʻAmyloidosis AL

Ua like loa ka mālama ʻana no ka AL Amyloidosis me nā ʻano maʻamau o ka mālama ʻana i ka maʻi kanesa. ʻO lākou kēia:

• Chemotherapy: E like me ke kanesa, hiki i ka chemotherapy ke hoʻopau i nā pūnaewele plasma hana ʻole.
• Hoʻoili pūnaewele kumu: Hana pinepine ʻia kēia lāʻau lapaʻau me ka chemotherapy, a hoʻololi i nā pūnaewele plasma hana hewa me nā pūnaewele hou. Lawe pinepine ʻia kēia mau pūnaewele kumu mai ou aku (autologous). No ka mea, ʻo ʻoe iho lākou, ʻike kou kino iā lākou a ʻaʻole hōʻole a pane maikaʻi ʻole paha iā lākou.
• Hoʻōla lāʻau pale:Hiki i kāu ʻōnaehana pale ke ʻike a luku i nā cell i hana hewa. I nā maʻi e like me ke kanesa a i ʻole AL Amyloidosis, ʻaʻole ʻike kāu ʻōnaehana pale i kēlā mau cell he hewa. Aʻo ka Immunotherapy i kāu ʻōnaehana pale e ʻike a luku i nā cell hewa.

ʻO ka amyloidosis ATTR

ʻO kēia ʻano "(Amyloidosis)" ke hana ʻia nā protein e hiki ke haki maʻalahi i kou ake. Pono kēia pilikia e hoʻōki i nā lāʻau lapaʻau āpau. Aia i loko o ka papa inoa ma lalo nā lāʻau lapaʻau i ʻāpono ʻia e ke aupuni a me nā mea e hana ʻia nei i ka wā noiʻi.

• Hoʻoili akepaʻa: I ka wā ma mua, ʻo ka hoʻoili akepaʻa wale nō ke ala e pale ai i kou akepaʻa mai ka hana ʻana i nā protein hewa. ʻOiai ʻaʻole kēia wale nō ke koho, he ʻano hana pono a maikaʻi nō hoʻi ia.
• Nā mea hoʻopau leo ​​genetic: ʻO ATTR Amyloidosis ke kumu o kahi hoʻololi genetic i loko o kāu DNA, e like me ka typo i loko o kahi puke meaʻai. ʻO nā mea hoʻopau leo ​​genetic nā lāʻau lapaʻau e hana e like me kahi kāleka meaʻai no ka manawa pōkole. Ma kahi o kāu mau cell e hahai ana i ka meaʻai hewa, hāʻawi lākou iā lākou i nā ʻōlelo aʻoaʻo hou i hiki iā lākou ke hana pololei i ka protein.
• Nā mea hoʻopaʻa: Hoʻōki kēia mau lāʻau i nā molekala TTR mai ka pelu hewa ʻana a i ʻole ka haki ʻana.
• Nā mea hoʻopaʻa Fibril: Ke hoʻomaka nā protein amyloid e hui pū, hana lākou i nā ʻano like me ka fiber i kapa ʻia he fibrils. Hoʻōki kēia mau lāʻau i ka hoʻokumu ʻia ʻana o nā fibrils.

ʻO ka amyloidosis e pili ana i ka dialysis

ʻO kēia ʻano `(Amyloidosis)` e ulu ana i loko o ka poʻe e pono ai `(dialysis)` no ka mea, hōʻiliʻili ka protein `(beta-2M)`. ʻO ka maʻamau, kānana a wehe kou mau puʻupaʻa i kēlā protein, akā ʻaʻole hiki i ka `(dialysis)` maʻamau ke hana i kēlā. ʻO ia ke kumu he mau makahiki no ka poʻe ma `(dialysis)` e loaʻa ai kēia maʻi. I kēia manawa, aia ʻelua mau ala e mālama ai:

• Hoʻoili puʻupaʻa: ʻO ke ʻano o ka puʻupaʻa olakino, ʻaʻole pono ʻoe i ka dialysis, a hiki i kahi puʻupaʻa i hoʻoili ʻia ke kānana i ka protein beta-2M keu. Hiki i kahi hoʻoili puʻupaʻa ke pale aku i ka amyloidosis e pili ana i ka dialysis mai ka lilo ʻana i mea ʻino, akā ʻaʻole maopopo i nā mea noiʻi inā e wehe pū ia i ka protein amyloid i hōʻiliʻili ʻia i loko o ke kino.
• Nā kānana kūikawā: ʻOiai ʻaʻole hiki i nā kānana dialysis maʻamau ke wehe i nā protein beta-2M, aia nā kānana kūikawā e hiki ke wehe hapa iā lākou. ʻO ka mea pōʻino, ʻaʻole kēia mau kānana e kānana i nā protein āpau, a ʻaʻole lākou e kānana i nā mea āpau. ʻO ke ʻano kēia, ʻoiai ma hope o nā makahiki o ka dialysis, hiki i ka amyloidosis e pili ana i ka dialysis ke ulu i ka hopena.

Nā lāʻau lapaʻau ʻaʻole kikoʻī

Aia kekahi mau ʻano lapaʻau a me nā lāʻau lapaʻau ʻē aʻe e hiki ke kōkua i ka poʻe me ka Cardiac Amyloidosis. Hoʻōla pinepine kēia i nā hōʻailona o ka maʻi a hiki ke komo pū me:

• Hoʻoili puʻuwai: ʻO ka poʻe me ka hōʻino nui o ka puʻuwai ma muli o ka amyloidosis cardiac i kekahi manawa e pono ai kahi hoʻoili puʻuwai. Hana pinepine ʻia kēia ʻoki ma hope o ka hoʻoponopono ʻia ʻana o ke kumu o ka amyloidosis a i ʻole ka mālama ʻia ʻana.
• Nā lāʻau lapaʻau anti-arrhythmia: Kōkua kēia mau lāʻau lapaʻau i ka pale ʻana i nā puʻuwai puʻuwai kūpono ʻole, a hiki i kekahi o ia mau mea ke hoʻoweliweli.
• Nā mea hana i hoʻokomo ʻia: ʻO kēia mau mea hana e komo pū me nā pacemakers a me nā implantable cardioverter defibrillators (ICDs) . Hoʻokele lākou i kou helu puʻuwai ma ka hāʻawi ʻana i nā haʻalulu uila a me ka pale ʻana i nā arrhythmias weliweli.
• Nā lāʻau lapaʻau diuretic: Kōkua kūikawā kēia mau lāʻau lapaʻau i ka paʻa ʻana o ka wai (kahi hōʻailona maʻamau o ka hāʻule ʻana o ka puʻuwai) ma ka hoʻomaikaʻi ʻana i kāu hana puʻupaʻa. Eia nō naʻe, ʻaʻole pono ʻoe e lawe i kēia mau lāʻau lapaʻau inā loaʻa iā ʻoe kahi maʻi e like me ka maʻi puʻupaʻa koʻikoʻi.
• Doxycycline : He mea ʻawahia hoʻi nā kaulahao māmā ma AL amyloidosis i kou mau hunaola puʻuwai. No nā kumu i maopopo ʻole ai i nā loea, hiki i ka antibiotic doxycycline ke pale aku i kēlā mau hopena ʻawahia.

Aia kekahi mau hopena ʻaoʻao o ka lāʻau lapaʻau?

ʻOkoʻa loa nā pilikia a me nā hopena ʻaoʻao o ka mālama ʻana no kēia maʻi. Aia ma lalo iho kekahi mau mea hiki ke hana maʻamau. Eia nō naʻe, hiki i kāu kauka ke wehewehe maikaʻi aku i nā pilikia, nā hopena ʻaoʻao, a me nā pilikia ʻē aʻe iā ʻoe. ʻO kēia no ka mea ua ʻike pono lākou i kou kūlana, no laila hiki iā lākou ke hoʻopilikino i kā lākou ʻike a me nā wehewehe ʻana i kou kūlana kikoʻī.

ʻEhia ka manawa e ʻoi aku ai koʻu maikaʻi ma hope o ka lāʻau lapaʻau?

ʻO ka maʻamau, ʻo ka mālama ʻana no ka Cardiac Amyloidosis ka mea i kaupalena ʻia i ka mālama ʻana i kāu mau hōʻailona. Hiki i kekahi mau lāʻau lapaʻau ke hāʻawi i kahi hōʻoluʻolu, akā he manawa pōkole nā ​​​​hopena. I kekahi mau hihia, hiki i nā lāʻau lapaʻau ke hana i nā hopena ʻaoʻao e hiki ai ke hoʻonui iā ʻoe no kekahi manawa, akā hiki i kāu kauka ke hāʻawi iā ʻoe i nā lāʻau lapaʻau a me ke alakaʻi e kōkua i ka hōʻemi ʻana i ka koʻikoʻi o kēlā mau hopena ʻaoʻao.

Hiki ke pale ʻia kēia? A i ʻole hiki ke hoʻēmi ʻia ka pilikia?

ʻO ka mea pōʻino, ʻaʻole hiki ke pale ʻia ka amyloidosis (me hoʻokahi ʻokoʻa, e nānā ma lalo). ʻOiai ʻaʻole ʻike ʻia ke kumu o ka amyloidosis i hoʻoilina ʻole ʻia, ʻaʻohe ala e pale aku ai. ʻAʻole hiki ke pale ʻia ka amyloidosis hoʻoilina, no ka mea, aia ia i loko o ka DNA āu e hoʻoilina ai mai kou mau mākua mai.

ʻO ka ʻokoʻa wale nō ka amyloidosis cardiac, kahi e pili ana me ka dialysis. Hiki ke pale ʻia kēia kūlana inā ʻaʻole ʻoe e hana i ka dialysis no ka manawa lōʻihi, a i ʻole inā ʻoe e hoʻohana i nā kānana kūikawā e hoʻōki i ka hōʻiliʻili ʻana o nā protein beta-2M. Eia nō naʻe, ʻaʻole maʻamau kēia mau kānana e hoʻōki loa i ka hōʻiliʻili ʻana, ʻo ia hoʻi he ala pinepine lākou e hoʻopaneʻe ai i ka hoʻomaka ʻana o ka maʻi.

He aha ka nānā ʻana no kēia maʻi?

He maʻi holomua ka Cardiac Amyloidosis, me nā hōʻailona o ka hāʻule ʻana o ka puʻuwai e lilo i mea ʻino aʻe. I ka hopena, hiki i ka maʻi ke hoʻonāwaliwali i kou puʻuwai a hiki i kahi e hiki ʻole ai ke hana hou. Inā ʻaʻole mālama ʻia, ʻoi aku ka maikaʻi ʻole o ka nānā ʻana.

Me ka ʻole o ka lāʻau lapaʻau, ʻo ka awelika o ka manawa ola no ka poʻe me AL Amyloidosis ma lalo o ʻewalu mahina. No ka poʻe me nā hihia koʻikoʻi loa, hiki ke lilo i ʻekolu a ʻeono mahina. No ka poʻe me ATTR Amyloidosis, ʻo ka awelika o ka manawa ola he ʻelua a ʻelima mau makahiki. No ka poʻe me nā ʻohana i mālama ʻole ʻia me ATTR Amyloidosis, ʻoi aku ka ʻino o ke kūlana, me ka awelika o ka manawa ola o ʻelua a ʻekolu mau makahiki.

Pehea e hiki ai iaʻu ke mālama iaʻu iho a hoʻokele i kaʻu mau hōʻailona?

ʻO ka mea pōʻino, ʻaʻole nui nā mea hiki iā ʻoe ke hana e kōkua iā ʻoe iho ma hope koke iho o kou loaʻa ʻana o ka amyloidosis. Inā loaʻa i kekahi o kou ʻohana ka maʻi, pono ʻoe e kamaʻilio me kāu kauka e pili ana i ka hoʻāʻo genetic. ʻOiai ʻaʻole nā ​​​​kānaka āpau me kahi mutation i pili i ka cardiac amyloidosis e loaʻa i ka maʻi, he mea nui e ʻike inā hiki iā ʻoe ke loaʻa. Ma kēlā ala, hiki iā ʻoe ke makaʻala i nā hōʻailona a hoʻomaka i ka mālama ʻana i ka wā mua.

Inā loaʻa iā ʻoe ka `(Cardiac Amyloidosis)`, he mea nui loa ia e hahai i nā ʻōlelo a kāu kauka e pili ana i nā lāʻau lapaʻau a me ka mālama ʻana. Hana ka nui o kēia mau lāʻau lapaʻau ma nā ʻano kikoʻī loa, a no lākou e hana pono ai, he mea nui e lawe iā lākou e like me ka ʻōlelo a kāu kauka iā ʻoe.

Āhea wau e ʻike ai i ke kauka a e ʻimi paha i ka mālama wikiwiki?

Ma hope o kou ʻike ʻia ʻana me ka Cardiac Amyloidosis, e hoʻonohonoho kāu kauka i nā kipa hahai maʻamau. Ma muli o ke koʻikoʻi o kou kūlana, pono paha ʻoe e ʻike pinepine i kāu kauka.

E haʻi aku nō hoʻi kāu kauka iā ʻoe i nā hōʻailona e pono ai ke nānā ʻia e ke kauka. Eia nō naʻe, ma ke ʻano laulā, e kāhea i kāu kauka inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona:

• Manaʻo poniuniu a nāwaliwali paha i ka wā e noho ana a kū ana paha: Ua kapa ʻia kēia he "Orthostatic Hypotension." Hana ʻia kēia no ka mea ʻo ke koko haʻahaʻa e hōʻemi ana i ka nui o ke koko e hele ana i ka lolo ke kū ʻoe.
• Ka pehu ʻana o nā lālā a i ʻole ka ʻōpū: ʻO kēia ma muli o ka paʻa ʻana o ka wai.
• ʻO ka pohō kaumaha koke a koʻikoʻi: He hōʻailona koʻikoʻi kēia, ʻoiai inā ʻaʻole ʻoe e hoʻāʻo nei e pohō kaumaha.

Āhea wau e hele ai i ka ER (Emergency Room)?

Ma muli o ka hiki i ka amyloidosis cardiac ke hoʻopilikia nui i kāu hana puʻuwai, hōʻike kekahi mau hōʻailona e pono koke ʻoe i ke kōkua lapaʻau.He mea pono ia. Eia kekahi o lākou:

• Manaʻo o nā palpitations o ka puʻuwai.
• Ka puʻuwai wikiwiki, lohi, a i ʻole kūlana ʻole.
• Ka paʻakikī o ka hanu ʻana no kekahi kumu.

ʻO ka mea hope loa, kekahi mau mea nui (Leka Lawe-Home)

He ʻano paʻakikī iki ka Cardiac Amyloidosis, akā he mea nui loa ia e ʻike i ia mea.

E hoʻomanaʻo, ʻoiai hiki i kēia maʻi ke holomua mālie, hiki i ka ʻike mua ʻana a me ka mālama kūpono ke hele lōʻihi i ka hoʻolōʻihi ʻana i kou ola a me ka hoʻomaikaʻi ʻana i kou ʻano ola.

I kekahi manawa hiki i nā hoʻoili kino ke ho'ōla i ke kūlana. Mahalo i nā lāʻau lapaʻau a me nā lāʻau lapaʻau hou, hiki i nā poʻe he nui me kēia maʻi ke kaohi i ko lākou mau hōʻailona a ola no nā makahiki ma hope o ka ulu ʻana o ka maʻi.

Inā he kānalua a nīnau paha kāu e pili ana i kēia, e kamaʻilio pono me kāu kauka . Hiki iā lākou ke hāʻawi iā ʻoe i ka ʻōlelo aʻo kūpono. Mai makaʻu, e ʻike, e nīnau i nā nīnau. ʻAʻole ʻoe hoʻokahi.

ʻAmyloidosis Puʻuwai , Amyloidosis Puʻuwai, Waiho ʻana o ka Protein, Maʻi Puʻuwai, Nā Hōʻailona Maʻi Puʻuwai, Lapaʻau Amyloidosis, AL Amyloidosis, ATTR Amyloidosis, Maʻi Puʻuwai