Transthyretin Amyloid Cardiomyopathy (ATTR-CM): Nā Hōʻailona, ​​Nā Hana, a me nā mea hou aʻe

Anter

• He aha nā hōʻailona o ATTR-CM?
• He aha ke kumu o ATTR-CM?
• Hoʻoilina (ʻohana) ATTR
• ʻO ATTR ʻano ʻāhiu
• Pehea e ʻike ʻia ai ka ATTR-CM?
• Pehea e mālama ʻia ai ʻo ATTR-CM?
• He aha nā mea weliweli?
• He aha ke kuanaʻike inā loaʻa iā ATTR-CM?
• Ke laina lalo

ʻO Transthyretin amyloidosis (ATTR) kahi kūlana kahi i waiho ʻia kahi protein i kapa ʻia ʻo amyloid i loko o kou puʻuwai, a i loko hoʻi o kou aʻa a me nā lālā ʻē aʻe. Hiki iā ia ke alakaʻi i kahi maʻi puʻuwai i kapa ʻia ʻo transthyretin amyloid cardiomyopathy (ATTR-CM).

ʻO Transthyretin kahi ʻano kikoʻī o ka protein amyloid i waiho ʻia i loko o kou puʻuwai inā loaʻa iā ATTR-CM. Hāpai maʻamau i ka huaora A a me ka hormone thyroid ma loko o ke kino.

ʻElua ʻano amyloidosis transthyretin: ʻano hihiu a me nā hoʻoilina.

ʻO ka ATTR ʻano ʻāhiu (ʻo ia hoʻi ʻo ka senile amyloidosis) ʻaʻole i hoʻokumu ʻia e kahi hoʻololi kūlohelohe. ʻO ka protein i waiho ʻia i loko o kona ʻano non-mutated.

I ka hoʻoilina hoʻoilina ATTR, hana hewa ʻia ka protein (misfolded). A laila puʻupuʻu pū kekahi a ʻoi aku paha ka hopena ma nā kino o kou kino.

He aha nā hōʻailona o ATTR-CM?

Hoʻopili ka ventricle hema o kou puʻuwai i ke koko ma o kou kino. Hiki i ka ATTR-CM ke hoʻopili i nā paia o kēia keʻena o ka puʻuwai.

Hoʻopaʻa nā waihona amyloid i nā paia, no laila ʻaʻole hiki iā lākou ke hoʻomaha a kaomi maʻamau.

ʻO kēia ka hiki ʻole i kou puʻuwai ke hoʻopihapiha pono (hoʻemi i ka hana diastolic) me ke koko a i ʻole ka pauma ʻana i ke koko ma o kou kino (hoʻemi ʻia ka hana systolic). Kāhea ʻia kēia he cardiomyopathy kaohi, kahi ʻano o ka puʻuwai puʻuwai.

Nā ʻōuli o kēia ʻano kīnā ʻole o ka naʻau:

• ka hanu ʻana (dyspnea), keu hoʻi i ka wā e moe ana i lalo a i ʻole me ka hoʻoikaika
• pehu i kou mau wāwae (peripheral edema)
• ʻeha ka umauma
• lāʻau ikiʻai lāʻau ikiʻai wale (arrhythmia)
• palpitations
• luhi
• hoʻonui ake akea a me ka pelupelu (hepatosplenomegaly)
• wai i loko o kou ʻōpū (ascites)
• ʻai ʻilihune
• ʻālohilohi, keu hoʻi i ke kū ʻana
• maʻule (syncope)

ʻO kahi hōʻailona kūikawā e kū i kekahi manawa ke koko kiʻekiʻe e holo mālie. Hana ʻia kēia no ka lilo ʻana o ka puʻuwai i mea kūpono ʻole, ʻaʻole hiki ke pauma i lawa e hoʻonui i kou kiʻekiʻe o ke koko.

ʻO nā ʻōuli ʻē aʻe āu e loaʻa ai mai nā waihona amyloid i nā ʻāpana ʻē aʻe o ke kino ʻē aʻe o kou puʻuwai pū kekahi:

• maʻi maʻi tunnel carpal
• ke ahi a me ka helu ʻana i kou mau lima a me kou mau wāwae (peripheral neuropathy)
• ʻeha hope mai ka lāʻau stenosis

Inā ʻeha kou umauma, e kāhea koke iā 911.

E ʻimi koke i ka lāʻau inā ʻoe e hoʻomohala i kēia mau ʻōuli:

• e hoʻonui nei i ka hanu
• ʻōhū nui o ka wāwae a i ʻole ka loaʻa wikiwiki ʻana
• wikiwiki a i ʻole kōmi o ka puʻuwai
• hoʻomaha iki a i ʻole ka lohi o ka naʻau
• ʻūpā
• maule ana

He aha ke kumu o ATTR-CM?

ʻElua ʻano o ka ATTR, a he kumu kū hoʻokahi ko kēlā me kēia.

Hoʻoilina (ʻohana) ATTR

I kēia ʻano, hehi hewa ʻole ka transthyretin ma muli o ka hoʻololi kūlohelohe. Hiki ke hoʻoili ʻia mai ka makua a ke keiki ma o nā gen.

Hoʻomaka ka hoʻomaka ʻana o nā ʻōuli ma kāu mau 50, akā hiki iā lākou ke hoʻomaka i ka manawa o kāu 20s.

ʻO ATTR ʻano ʻāhiu

ʻO ke kuhi hewa ʻana o ka protein he hanana maʻamau. Loaʻa i kāu kino nā ʻano hana e wehe i kēia mau protein i mua o ka hopena ʻana i kahi pilikia.

Ke ʻelemakule nei ʻoe, lilo kēia mau mīkini i mea ʻoi aku ka maikaʻi, a hiki i nā protein i hoʻopili hewa ʻia ke ʻōpala a hana i nā waihona. ʻO ia ka mea e hana ai i ka ʻano ATTR ʻano hihiu.

ʻO ka ʻano ATTR ʻāhiu ʻaʻole ia he hoʻololi kūkaʻi, no laila ʻaʻole hiki ke hoʻoili ʻia i waena o nā gen.

Hoʻomaka ka hoʻomaka ʻana o nā ʻōuli ma kāu mau makahiki he 60 a 70 paha.

Pehea e ʻike ʻia ai ka ATTR-CM?

Hiki ke paʻakikī ka ʻike ʻana ma muli o ka like o nā ʻōuli me nā ʻano puʻuwai puʻuwai ʻē aʻe. Hoʻohana ʻia nā hoʻokolohua i hoʻohana pinepine ʻia no ka hōʻoia

• electrocardiogram e hoʻoholo inā mānoanoa nā paia o ka puʻuwai mai nā waihona (maʻa mau ka uila uila)
• echocardiogram e nānā i nā paia mānoanoa a nānā i ka hana o ka puʻuwai a nānā i nā hiʻohiʻona hoʻomaha hoʻomaha a i ʻole nā ​​hōʻailona o ka hoʻonui ʻana o kaomi i ka puʻuwai.
• naʻau MRI e nānā no ka amyloid i ka paia puʻuwai
• ʻimi biopsy puʻuwai puʻuwai e nānā no nā waihona amyloid ma lalo o kahi microscope
• nā noiʻi genetika e ʻimi nei i ka ATTR hoʻoilina

Pehea e mālama ʻia ai ʻo ATTR-CM?

Hana ʻia ʻo Transthyretin e kou ake. No kēia kumu, mālama ʻia ka hoʻoilina ATTR-CM me ka hoʻoliʻiliʻi ate inā hiki. Ma muli o ka hōʻino pinepine ʻia o ka puʻuwai ke ʻike ʻia ke ʻano, hana pinepine ʻia kahi transplant puʻuwai i ka manawa like.

I ka 2019, ʻae ʻia ʻelua mau lāʻau no ka mālama ʻana o ATTR_CM: tafamidis meglumine (Vyndaqel) a me tafamidis (Vyndamax) capsule.

Hiki ke mālama ʻia kekahi o nā ʻōuli o ka cardiomyopathy me nā diuretics e hemo ai ka nui o ka wai.

ʻO nā lāʻau ʻē aʻe i maʻa mau ʻia e mālama i ka puʻuwai puʻuwai, e like me beta-blockers a me digoxin (Lanoxin), hiki ke hōʻeha i kēia ʻano a ʻaʻole pono e hoʻohana maʻamau.

He aha nā mea weliweli?

ʻO nā kumu pilikia no ka hoʻoilina ATTR-CM me:

• kahi moʻolelo ʻohana o ke kūlana
• kāne kāne
• makahiki ma luna o 50
• He kūpuna ʻApelika

Nā kumuhana kūpilikiʻi no ka ATTR-CM ʻano hihiu me:

• makahiki ma luna o 65
• kāne kāne

He aha ke kuanaʻike inā loaʻa iā ATTR-CM?

Me ka ʻole o ke ake a me ka puʻuwai, e ʻoi aku ana ka ATTR-CM i ka hala ʻana o ka manawa. Ma ka awelika, ola ka poʻe me ATTR-CM ma hope o ka hōʻailona.

Hiki i ke ʻano ke loaʻa i kahi hopena hoʻonui i kou ola o ka maikaʻi, akā ʻo ka mālama ʻana i kāu mau hōʻailona me ka lāʻau hiki ke kōkua nui.

Ke laina lalo

Hoʻokumu ʻia ʻo ATTR-CM e ka hoʻololi kūlohelohe a i ʻole pili i ka makahiki. Alakaʻi ia i nā ʻōuli o ka puʻuwai ʻole.

ʻIke ka paʻakikī ma muli o ke ʻano like o nā ʻano puʻuwai puʻuwai ʻē aʻe. E hele a holomua ʻoi aku ka maikaʻi ma mua o ka manawa akā hiki ke mālama ʻia me ke ake a me ka puʻuwai a me ka lāʻau e kōkua i ka mālama ʻana i nā hōʻailona.

Inā ʻike ʻoe i kekahi o nā ʻōuli o ATTR-CM i helu ʻia ma mua, e kāhea i kāu kauka.