Created at:1/16/2025
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ʻO ka Long QT syndrome kahi kūlana o ka naʻau e pili ana i ke ʻano o ka hana ʻana o ka ʻōnaehana uila o kou naʻau. Loaʻa iā ia kona inoa mai kahi ʻano kūikawā e ʻike ai nā kauka ma kahi hoʻokolohua electrocardiogram (ECG), kahi e lōʻihi ai kekahi ʻāpana o ke kaʻina o kou puʻuwai ma mua o ka maʻamau.
Hiki i kēia kūlana ke hoʻokumu i kou naʻau e pāʻani i kahi ʻano wikiwiki, haunaele i kapa ʻia ʻo torsades de pointes. ʻOiai paha he mea weliweli kēia, nui nā kānaka me ka long QT syndrome e ola ana i nā ola maʻamau, olakino me ka mālama kūpono a me ka ʻike.
Hana ʻia ka Long QT syndrome i ka wā e lōʻihi loa ai kou naʻau e hoʻomaha ma waena o nā pāʻani. E noʻonoʻo i kou naʻau e like me kahi kaapuni uila e pono ai e hoʻouka hou ma hope o kēlā me kēia pāʻani e noho mau i ka leo.
ʻO ka "QT interval" ke kuhikuhi nei i kahi ana ma ka palapala uila o kou naʻau. I ka wā e lōʻihi ai kēia manawa ma mua o ka maʻamau, hiki iā ia ke hana i kahi puka aniani kahi e hiki ai i nā ʻano naʻau weliweli ke kū mai. Hiki i kēia kūlana ke lilo i mea i hānau ʻia ʻoe a i ʻole e hoʻomohala ʻia ma muli o nā lāʻau lapaʻau a i ʻole nā kūlana olakino ʻē aʻe.
ʻO ka hapa nui o nā kānaka me ka long QT syndrome he ʻano maʻamau loa ko lākou naʻau. Aia wale nō ka pilikia me nā hōʻailona uila e hoʻonohonoho ana i kou puʻuwai.
Nui nā kānaka me ka long QT syndrome ʻaʻohe hōʻailona. I ka wā e kū mai ai nā hōʻailona, maʻamau lākou e kū mai ana ma muli o kou naʻau e pāʻani koke ana i kahi ʻano weliweli.
Eia nā hōʻailona nui āu e ʻike ai:
ʻO nā hāʻule pinepine ka hōʻailona i ʻike nui ʻia. Maʻamau lākou e kū mai ana i ka wā e pilikia ai kou kino, inā kino a i ʻole manaʻo paha. ʻIke kekahi poʻe i ko lākou mau hōʻailona e kū mai ana me ka hoʻoikaika kino, ka ʻauʻau, a i ʻole ka hoʻohoka ʻia e ka hola ʻala.
Loaʻa ka Long QT syndrome i ʻelua ʻano nui: congenital (hoʻoilina) a me ka loaʻa (hoʻomohala ʻia ma hope i ke ola). Ke hoʻomaopopo ʻana i ke ʻano āu e loaʻa ai e kōkua i ke alakaʻi ʻana i kāu ala lapaʻau.
Hana ʻia ka congenital long QT syndrome e nā mutations gene e hoʻoilina mai kou mau mākua. Aia ma kahi o 17 mau ʻano gene like ʻole, ʻo nā mea ʻekolu maʻamau loa:
Hoʻomohala ʻia ka Long QT syndrome loaʻa ma muli o nā kumu kūwaho e like me nā lāʻau lapaʻau, nā kūlana lapaʻau, a i ʻole nā kūlike electrolyte. Pinepine ka hoʻihoʻi ʻia ʻana o kēia ʻano i ka wā e hoʻoponopono ʻia ai ke kumu kumu.
Aia nā kumu o ka long QT syndrome ma muli o ka hoʻoilina a i ʻole ka loaʻa ʻana o ke ʻano. E ʻimi kākou i nā mea e alakaʻi ai i kēlā me kēia ʻano.
Hoʻopuka ʻia ka congenital long QT syndrome mai nā mutations gene e hoʻopilikia i nā protein i kapa ʻia ʻo nā ion channels. Hoʻokele kēia mau channels i ke kahe o nā hōʻailona uila i loko o nā sela o kou naʻau. I ka wā ʻaʻole lākou e hana pono, e lōʻihi ka hoʻomaha hou ʻana o kou naʻau ma mua o ka maʻamau.
Hiki i ka long QT syndrome loaʻa ke hoʻomohala mai kekahi mau kumu:
I kekahi manawa, loaʻa i nā kānaka kahi predisposition genetic māmā e lilo wale nō i pilikia i ka wā e hui pū ʻia me nā lāʻau lapaʻau a i ʻole nā mea hoʻonāukiuki ʻē aʻe. Wehewehe kēia i ke kumu e hoʻomohala ai kekahi poʻe i nā hōʻailona ma hope i ke ola ʻoiai me kahi ʻano hoʻoilina.
Pono ʻoe e ʻimi i ka mālama lapaʻau inā loaʻa iā ʻoe nā hāʻule, ʻoi aku hoʻi i ka wā e hoʻoikaika ai a i ʻole ke kaumaha manaʻo. ʻOiai hoʻokahi hāʻule wehewehe ʻole ʻia e pono ai ka loiloi, ʻoi aku hoʻi inā kū mai ia i ka wā e hana kino ai.
E hoʻokaʻaʻike koke i kāu kauka inā ʻike ʻoe i nā palpitations naʻau e like ʻole me ka hopohopo maʻamau, nā dizziness hou, a i ʻole inā ua haʻi ʻia iā ʻoe he ECG kūpono ʻole kāu. He mea nui nō hoʻi ka mōʻaukala ʻohana - inā loaʻa i nā hoahānau kokoke iā ʻoe ka long QT syndrome a i ʻole make koke i ka wā ʻōpio, e haʻi i kēia i kāu mea hoʻolako mālama ola.
E ʻimi i ka mālama pilikia koke inā hāʻule ʻoe a ʻaʻole ala koke, loaʻa iā ʻoe ka ʻeha umauma me ka hāʻule, a i ʻole e ʻike i ka mea e like me ka hoʻopaʻa ʻana. Hiki i kēia mau mea ke hōʻike i kahi ʻano naʻau weliweli e pono ai ka mālama koke.
Nui nā mea e hiki ke hoʻonui i kou hiki ke hoʻomohala i ka long QT syndrome a i ʻole e ʻike i nā pilikia inā loaʻa iā ʻoe. Ke hoʻomaopopo ʻana i kēia mau mea e kōkua iā ʻoe a me kāu kauka e hana i nā hoʻoholo ʻike e pili ana i kāu mālama.
No ke ʻano hoʻoilina, ʻo kāu kumu pilikia nui ka loaʻa ʻana o nā lālā ʻohana me ka long QT syndrome, ka hāʻule wehewehe ʻole ʻia, a i ʻole ka make naʻau koke i ka wā ʻōpio. Hiki i nā wahine me ka long QT syndrome hoʻoilina ke loaʻa ka pilikia nui i ka wā o kekahi mau loli hormonal, ʻoi aku hoʻi ma hope o ka hānau ʻana.
Nā kumu pilikia no ka long QT syndrome loaʻa:
He kuleana ko ka makahiki i nā ʻano hōʻailona. ʻO nā keiki a me nā ʻōpio me nā ʻano hoʻoilina pinepine ko lākou mau hōʻailona mua i ka wā o ka pubescence, ʻoiai paha e hoʻomohala nā mākua i nā ʻano loaʻa ma muli o nā lāʻau lapaʻau a i ʻole nā loli olakino.
ʻO ka mea nui e pili ana i ka long QT syndrome ʻo ka pilikia o ka hoʻomohala ʻana i kahi ʻano naʻau weliweli i kapa ʻia ʻo torsades de pointes. Hiki i kēia ʻano ke hoʻokumu i ka hāʻule a, i nā hihia kakaikahi, hiki ke holomua i ka cardiac arrest.
ʻO ka hapa nui o nā hanana o ka torsades de pointes he pōkole a kū hoʻokahi iho, ʻo ia ke kumu e ʻike ai nā kānaka i ka hāʻule akā laila e ola hou loa. Eia naʻe, ke hoʻomaopopo ʻana i nā pilikia hiki ke kōkua iā ʻoe e lawe i nā palekana kūpono.
Nā pilikia hiki:
ʻO ka ʻoiaʻiʻo maikaʻi, me ka mālama kūpono a me ka ʻike, ua emi nui ka pilikia o nā pilikia koʻikoʻi. Hiki i ka hapa nui o nā kānaka me ka long QT syndrome i mālama maikaʻi ʻia ke komo i nā hana maʻamau he nui me ka palekana.
Hoʻomaka ka diagnosis ʻana o ka long QT syndrome me ka electrocardiogram (ECG), kahi e ana ai i ka hana uila o kou naʻau. E ʻimi kāu kauka i kahi QT interval lōʻihi, ʻoiai i kekahi manawa ʻaʻole maopopo kēia ma kahi hoʻokolohua hoʻokahi.
Hiki i kāu kauka ke ʻōlelo aku i kekahi mau hoʻokolohua e loaʻa ai kahi kiʻi piha:
I kekahi manawa ʻaʻole pololei ka diagnosis. Pono paha kāu kauka e nānā i kāu mau hōʻailona, mōʻaukala ʻohana, a me ka papa inoa lāʻau lapaʻau me nā hopena hoʻokolohua. Hiki iā lākou ke hana i nā hoʻokolohua kūikawā e hoʻāʻo i ka ʻōnaehana uila o kou naʻau me ka palekana.
Kūkulu ka lapaʻau no ka long QT syndrome i ka pale ʻana i nā leo naʻau weliweli a me ka mālama ʻana i kāu mau hōʻailona. Aia ke ala ma muli o kāu ʻano kūikawā, ka nui o nā hōʻailona, a me nā kumu pilikia pilikino.
No ka long QT syndrome loaʻa, pinepine ka lapaʻau e komo pū ana i ka hoʻoponopono ʻana i ke kumu kumu. Hiki paha kēia ke manaʻo i ka hoʻōki ʻana a i ʻole ka hoʻololi ʻana i nā lāʻau lapaʻau, ka hoʻoponopono ʻana i nā kūlike electrolyte, a i ʻole ka mālama ʻana i nā kūlana e like me nā maʻi thyroid.
Nā koho lapaʻau no ka long QT syndrome hoʻoilina:
ʻO nā beta-blockers pinepine ka lapaʻau mua ma muli o ka kōkua ʻana i ka hoʻomau ʻana i kou leo naʻau. E hana pū kāu kauka me ʻoe e loaʻa ai ka lāʻau kūpono a me ka nui e kaohi i kāu mau hōʻailona ʻoiai e hoʻemi ana i nā hopena ʻaoʻao.
ʻO ka mālama ʻana i ka long QT syndrome ma ka home e komo pū ana me ka ʻike ʻana i kāu mau mea hoʻonāukiuki a me ka lawe ʻana i nā ʻanuʻu e pale aku ai iā lākou. ʻAʻole kēia manaʻo ʻaʻole hiki iā ʻoe ke ola i kahi ola ikaika, akā ʻo ia wale nō e hana ʻoe i nā koho ʻike e pili ana i nā hana.
Eia nā ʻano mālama home nui:
E makaʻala i ke ʻano o kou manaʻo i ka wā o nā hana like ʻole. Inā ʻike ʻoe i nā hōʻailona me nā hoʻoikaika kino kūikawā a i ʻole nā kūlana, e kūkākūkā i nā hoʻololi me kāu kauka ma mua o ka pale ʻana i nā hana a pau.
Pono nā lālā ʻohana e aʻo i ka CPR maʻamau a ʻike pehea e ʻike ai inā loaʻa iā ʻoe kahi hanana koʻikoʻi. Ke kōkua ka hoʻolālā ʻana i nā mea a pau e ʻike i ka hilinaʻi a me ka mākaukau.
Ke hoʻomākaukau nei no kāu hoʻokolohua e kōkua i ka hōʻoia ʻana e loaʻa iā ʻoe ka mea nui loa mai kāu kipa. E lawe mai i kahi papa inoa piha o nā lāʻau lapaʻau, nā mea hoʻohui, a me nā lāʻau lapaʻau ma luna o ka counter āu e lawe ai, ʻoiai hiki i kekahi ke hoʻopilikia i kou leo naʻau.
E kākau i kāu mau hōʻailona, me ka wā e kū mai ai, ka mea āu i hana ai, a me ka lōʻihi o ko lākou mau lōʻihi. E hoʻomaopopo i nā ʻano āu i ʻike ai, e like me nā hōʻailona e kū mai ana i ka wā e hoʻoikaika ai, ke kaumaha, a i ʻole i kekahi manawa o ka lā.
E hōʻiliʻili i kāu mōʻaukala lapaʻau ʻohana, ʻoi aku hoʻi i nā ʻike e pili ana i nā pilikia naʻau, nā make koke, a i ʻole nā hāʻule i nā hoahānau. He mea nui kēia ʻike no ka hoʻomaopopo ʻana i kāu pilikia a me nā pono lapaʻau.
E hoʻomākaukau i nā nīnau e pili ana i kāu mau hana i kēlā me kēia lā, nā lāʻau lapaʻau, a me nā hopohopo āu e loaʻa ai. E nīnau e pili ana i nā palena hana, ka hoʻolālā pilikia, a me ka wā e ʻimi ai i ka mālama koke.
ʻAʻole hiki iā ʻoe ke pale aku i ka long QT syndrome hoʻoilina ʻoiai ua hoʻoholo ʻia e kāu mau gene. Eia naʻe, hiki iā ʻoe ke lawe i nā ʻanuʻu e pale aku ai i ka long QT syndrome loaʻa a hoʻemi i nā pilikia inā loaʻa iā ʻoe kekahi ʻano.
Nā ʻano palekana e komo pū ana me ka makaʻala e pili ana i nā lāʻau lapaʻau e hiki ke hoʻolōʻihi i ka QT interval. E haʻi mau i nā mea hoʻolako mālama ola e pili ana i kou kūlana ma mua o ka loaʻa ʻana o nā kuhikuhi hou, me nā antibiotics a i ʻole nā antidepressants.
E mālama i ka olakino maikaʻi ma ka ʻai ʻana i ka meaʻai kaulike waiwai i ka potassium a me ka magnesium, e noho hydrated pono, a mālama i nā kūlana olakino ʻē aʻe e like me ka maʻi diabetes a i ʻole nā pilikia thyroid. E pale aku i ka inu nui ʻana o ka waiʻona a me nā lāʻau lapaʻau kū kānāwai ʻole, e hiki ke hoʻopilikia i kou leo naʻau.
Inā loaʻa iā ʻoe ka mōʻaukala ʻohana o ka long QT syndrome a i ʻole nā make koke wehewehe ʻole ʻia, e noʻonoʻo i ka ʻōlelo aʻo gene a me ka hoʻokolohua. ʻAe ka ʻike mua ʻana i ka nānā pono a me ka pale ʻana i nā pilikia.
ʻO ka Long QT syndrome kahi kūlana hiki ke mālama ʻia e hoʻopilikia ana i ka ʻōnaehana uila o kou naʻau. ʻOiai e pono ai ka nānā mau a me ka mālama, hiki i ka hapa nui o nā kānaka me kēia kūlana ke ola i nā ola piha, ikaika me ka mālama kūpono a me ka ʻike.
ʻO ka mea nui loa ka hana pū ʻana me kāu hui mālama ola e hoʻomaopopo i kāu ʻano kūikawā a me nā kumu pilikia. Me ka hui kūpono o nā lāʻau lapaʻau, ka ʻike ʻano ola, a me ka nānā mau, hiki iā ʻoe ke hoʻemi nui i kou pilikia o nā pilikia.
E hoʻomanaʻo ʻaʻole e hoʻomaopopo kou mau palena i ka loaʻa ʻana o ka long QT syndrome - ʻo ia wale nō ke manaʻo e hana ʻoe i nā hoʻoholo ʻike e pili ana i kāu mau hana a me ka mālama ola. E komo mau i kāu hoʻolālā lapaʻau, e kamaʻilio ākea me kāu mau kauka, a mai kānalua e nīnau i nā nīnau e pili ana i kou kūlana.
Hiki i nā kānaka he nui me ka long QT syndrome ke hoʻoikaika kino me ka palekana, akā pono paha e hoʻololi ʻia ke ʻano a me ka nui ma muli o kāu kūlana kūikawā. E kōkua kāu kauka e hoʻoholo i nā hana kūpono no ʻoe. Hiki ke kaupalena ʻia ka ʻauʻau no kekahi mau ʻano o ka long QT syndrome hoʻoilina, ʻoiai paha e paipai ʻia nā hoʻoikaika kino ʻē aʻe me nā palekana kūpono.
He hoʻoilina kekahi mau ʻano o ka long QT syndrome, i hoʻoili ʻia ma o nā ʻohana ma o nā mutations gene. Inā loaʻa iā ʻoe ka long QT syndrome hoʻoilina, loaʻa i kēlā me kēia o kāu mau keiki ka manawa 50% e hoʻoilina i ke kūlana. Eia naʻe, ʻaʻole hōʻoia ka loaʻa ʻana o ka mutation gene e hoʻomohala nā hōʻailona, a ʻaʻole hoʻoili ʻia nā ʻano loaʻa i nā keiki.
Nui nā lāʻau lapaʻau e hiki ke hoʻopilikia i ka long QT syndrome, me kekahi mau antibiotics, antidepressants, antihistamines, a me nā lāʻau naʻau. E haʻi mau i nā mea hoʻolako mālama ola e pili ana i kou kūlana ma mua o ka loaʻa ʻana o nā kuhikuhi hou. E mālama i kahi papa inoa hou o nā lāʻau lapaʻau e pale aku ai, a e noʻonoʻo e komo i nā mea hoʻonaninani ʻike lapaʻau.
ʻAʻole hiki ke hoʻōla ʻia ka long QT syndrome hoʻoilina ʻoiai ua hana ʻia e nā mutations gene, akā hiki ke mālama maikaʻi ʻia me ka lapaʻau. Hiki ke hoʻihoʻi ʻia ka long QT syndrome loaʻa inā hiki ke hoʻoponopono ʻia ke kumu kumu (e like me ka lāʻau lapaʻau a i ʻole ka kūlike electrolyte). ʻOiai me nā ʻano hoʻoilina, ʻae ka mālama kūpono i ka hapa nui o nā kānaka e ola i nā ola maʻamau.
Hiki i ka long QT syndrome ke koʻikoʻi inā waiho ʻole ʻia, hiki ke alakaʻi i nā leo naʻau weliweli. Eia naʻe, me ka diagnosis kūpono a me ka mālama, loaʻa i ka hapa nui o nā kānaka me kēia kūlana nā hopena maikaʻi loa. ʻO ka mea nui ka hana pū ʻana me kāu hui mālama ola e mālama i kāu mau kumu pilikia kūikawā a hahai mau i kāu hoʻolālā lapaʻau.