E aʻo kākou e pili ana i ka maʻi Tangier, kahi e kū mai ana i nā poʻe me nā pae haʻahaʻa o ka cholesterol maikaʻi (HDL) i loko o ke kino.

Ua lohe paha ʻoe i kahi ʻano cholesterol i kapa ʻia ʻo ka cholesterol maikaʻi (HDL / high-density lipoprotein) ? ʻAe, ʻae... I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi laha ʻole loa e kū mai ana i ka wā e haʻahaʻa loa ai ka pae o kēia cholesterol maikaʻi i loko o ko mākou kino. Ua kapa ʻia kēia ʻo Tangier Disease . ʻAʻole paha ʻoe i kamaʻāina i kēia inoa, akā he mea nui loa ia e ʻike e pili ana i ia mea.

He aha ka maʻi Tangier? E hoʻomaopopo maʻalahi kākou!

ʻAe, e hoʻomaka kākou mai ka hoʻomaka. He maʻi laha ʻole ka maʻi Tangier a mākou e hoʻoilina ai mai ko mākou mau mākua mai, ʻo ia hoʻi, he maʻi hoʻoilina genetic . ʻO ka poʻe me kēia maʻi he haʻahaʻa loa ko lākou mau pae o ka cholesterol maikaʻi (HDL) i loko o ko lākou kino. Ua ʻike anei ʻoe, ʻaʻole i kapa ʻia kēia cholesterol HDL he "cholesterol maikaʻi" no ka mea ʻole. ʻO lākou ka poʻe e kōkua i ka mālama ʻana i ko mākou kino ma ka wehe ʻana i ka cholesterol maikaʻi ʻole (LDL / low-density lipoprotein) e hōʻiliʻili pinepine ana i loko o ko mākou mau kīʻaha koko, ʻo ia hoʻi, i loko o ko mākou mau aʻa .

I kēia manawa, e noʻonoʻo i ka mea e hana ʻia ke emi kēia cholesterol maikaʻi (HDL). ʻO kēlā cholesterol maikaʻi ʻole (LDL) a me nā ʻano momona ʻē aʻe (lipids) e hoʻomaka maʻalahi e waiho i loko o ko kākou mau aʻa. E like me ka lepo e paʻa ana i loko o kahi paipu wai. Hiki i kēia ke hana i nā pilikia no ko kākou puʻuwai , ka hōʻeha puʻuwai , a i ʻole ke alakaʻi i nā kūlana e like me ka hahau ʻana . ʻAʻole wale ia, hiki i kēia maʻi ke hoʻopilikia i nā ʻōpū ʻē aʻe he nui i loko o ko kākou kino.

No ke aha i kapa ʻia ai ʻo "Tangier"?

He moʻolelo hoihoi loa kēia. Ua kapa ʻia kēia maʻi ʻo "Tangier" ma muli o ka Mokupuni ʻo Tangier , aia ma kēlā ʻaoʻao o ke kai mai ka mokuʻāina ʻo Virginia o ʻAmelika Hui Pū ʻIa. Ua ʻike mua ʻia kēia maʻi i waena o ka poʻe i noho ma ia mokupuni. Eia naʻe, ʻaʻole ia he manaʻo he mea kēia i kaupalena ʻia i kēlā mokupuni. Aia kekahi poʻe me kēia maʻi ma nā ʻāina ʻē aʻe o ka honua.

Pehea ka maʻamau o ka maʻi Tangier?

ʻOiaʻiʻo, he maʻi laha ʻole ka maʻi Tangier. He kakaikahi wale nō ka poʻe i ʻike ʻia ma ka honua holoʻokoʻa, ma kahi o 100. ʻO ia ke kumu ʻaʻole i kamaʻilio nui ʻia. Akā, ʻoiai he mea laha ʻole ia, he mea nui e makaʻala i kēia maʻi, ʻeā?

Pehea ka hopena o ka maʻi Tangier i ko mākou kino?

Eia ka mea e hana ʻia. Ke loaʻa iā kākou ka maʻi diabetes, haʻahaʻa nā pae cholesterol maikaʻi (HDL) o ko kākou kino, no laila ʻaʻole hiki ke lawe pono ʻia nā momona (lipids) i ko kākou akepaʻa . ʻO ke akepaʻa ka mea e wehe i kēia momona makemake ʻole ʻia mai ke kino. No laila, hoʻomaka mālie kēia momona hiki ʻole ke wehe ʻia e hōʻiliʻili ma nā ʻāpana like ʻole o ko kākou kino. Ke hōʻiliʻili kēia momona, hiki i kēlā ʻōkana ke lilo i mea nui iki.

Hoʻokomo pinepine ʻia ka momona ma kēia ʻano:

• ʻAke: Hiki ke hoʻonui ʻia ke ake.
• ʻŌpū:Hiki i ka ʻōpū ke pehu a hoʻonui ʻia.
• ʻO nā tonsils: ʻO nā tonsils i loko o ko kākou puʻu. Hiki iā lākou ke lilo i kala melemele-ʻalani ke piha lākou i ka momona.
• Nā kiko lymph: He ʻāpana kēia o kā mākou ʻōnaehana pale. Hiki iā lākou ke pehu.
• Puʻuwai: Hiki i kēia ke hoʻopilikia i ka hana o ka puʻuwai.
• Lolo: I kekahi manawa hiki ke hoʻopilikia ʻia ka lolo.

Ma waho aʻe o kēia, hiki ke loaʻa kekahi mau pilikia ʻē aʻe:

• Nā pilikia aʻalolo: Hiki ke loaʻa nā pilikia aʻalolo, e like me ka helu ʻana i nā lālā.
• Nā pae triglyceride kiʻekiʻe: He ʻano momona kēia i loaʻa i ke koko, e like me ka cholesterol.
• Nā pilikia ʻike: Aia paha he ao ʻē i nā maka, a i kekahi manawa nalowale ka ʻike.

He aha nā hōʻailona o ka maʻi Tangier?

He mea nui loa kēia. Hiki ke ʻokoʻa nā hōʻailona o ka maʻi Tangier mai kēlā kanaka a kēia kanaka. Aia ia i kahi i waiho ʻia ai ka momona i loko o ke kino, e like me kaʻu i ʻōlelo ai ma mua. Hiki i kekahi poʻe ke hōʻike i nā hōʻailona i ka wā hānau, aʻo kekahi poʻe ʻaʻole paha e hōʻike i nā hōʻailona nui a hiki i ko lākou mau makahiki 60-65.

Eia kekahi mau hōʻailona maʻamau:

• ʻEha ʻōpū a nausea paha.
• Nā waihona momona i loko o nā aʻa (atherosclerosis): Hoʻonui kēia i ka pilikia o ka maʻi puʻuwai.
• ʻIli maloʻo.
• ʻO ka ʻōwili ʻana o ka lihilihi i loko (Ectropion): He hōʻailona ʻano ʻē kēia.
• Anemia: ʻO ke ʻano kēia o ka nele o ke koko.
• Ka nāwaliwali o nā ʻiʻo ma nā lima, nā wāwae, nā lima, a i ʻole nā ​​​​​​wāwae.
• Nā ʻōpuʻu lymph i pehu no ka manawa lōʻihi me ka ʻole o ka maʻi.

E hoʻomanaʻo, i kekahi manawa hiki ke kuhihewa ʻia kēia mau hōʻailona no nā maʻi ʻē aʻe, ʻo ia ke kumu he mea nui e loaʻa kahi hōʻailona pololei.

He aha ke kumu o ka maʻi Tangier? E kamaʻilio kākou e pili ana i nā genes!

ʻO ka maʻi Tangier ke kumu o kahi kīnā i loko o kahi gene i kapa ʻia ʻo ka gene ABCA1 a mākou e hoʻoilina ai mai ko mākou mau mākua. I ka ʻōlelo maʻalahi, aia i loko o ka gene ABCA1 nā kuhikuhi e wehe i ka cholesterol mai ko mākou mau cell a hoʻohui iā ia i ka cholesterol maikaʻi (HDL). Lawe ʻo HDL i kēia cholesterol i ke akepaʻa, kahi e hoʻokuʻu ʻia ai.

I kēia manawa, inā he kīnā a he mutation paha i loko o kēia gene ABCA1, ʻaʻole hana pono kēlā mau kuhikuhi. A laila ʻaʻole e hana pono ʻia ka wehe ʻana o ka cholesterol mai nā cell a me ka hoʻokumu ʻana o HDL. ʻO ka hopena, ʻo ia ka emi nui ʻana o nā pae HDL.

Penei ke ʻano o ka hoʻoilina ʻana:

• Inā hoʻoilina nā mākua ʻelua i ka gene ABCA1 hemahema, e loaʻa i ke keiki ka maʻi Tangier.
• Inā hoʻokahi wale nō makua e hoʻoilina i ka gene kīnā, e lilo ke keiki i mea lawe. ʻAʻole paha lākou e hōʻike i nā hōʻailona, ​​​​akā, ʻoi aku ka haʻahaʻa o ko lākou pae HDL ma mua o ka maʻamau.

I kēia manawa, e noʻonoʻo ʻoe he mau mea lawe kēia gene kīnā nā mākua ʻelua, ʻo ia hoʻi, ʻaʻole paha he mau hōʻailona ko lāua, akā loaʻa iā lāua ka gene. Ke loaʻa i kēlā mau mākua kahi keiki:

• Aia he 25% (1/4) ka manawa e loaʻa ai i ke keiki ka maʻi Tangier.
• Aia he 50% (1/2) ka manawa e lilo ai ke keiki i mea lawe i ka maʻi.
• Aia he 25% (1/4) ka manawa kūpono ʻaʻole e hoʻoilina ke keiki i kekahi mau genes kīnā a e olakino maikaʻi ʻo ia.

Ua like kēia me ka hoʻolei ʻana i kahi ʻāpana kālā a loaʻa nā poʻo a i ʻole nā ​​huelo. Ua like nā mea hiki ke loaʻa no kēlā me kēia hāpai ʻana.

Pehea e ʻike ai nā kauka i ka maʻi Tangier?

I mea e ʻike pono ai inā loaʻa iā ʻoe ka maʻi Tangier, pono ʻoe e ʻike i ke kauka. E hana mua ʻo ia i kahi hoʻokolokolo kino a nīnau e pili ana i kāu mau hōʻailona. A laila, e kauoha lākou i kekahi mau hoʻokolohua koko . Nānā nui kēia mau hoʻokolohua koko i kou pae cholesterol HDL a me ka haʻahaʻa o kāu mau pae o kahi protein i kapa ʻia ʻo apolipoprotein A1 (ApoA1) . He haʻahaʻa loa kēia mau mea ʻelua i ka poʻe me ka maʻi Tangier.

Hiki ke hana ʻia nā hoʻāʻo genetic e hōʻoia i ka maʻi. Hiki iā ia ke nānā no kahi mutation kikoʻī i loko o ka gene ABCA1. Eia nō naʻe, ʻaʻole paha e loaʻa nā hoʻāʻo genetic ma nā wahi āpau. I kēlā mau hihia, hiki i kāu kauka ke lawe i kahi ʻāpana liʻiliʻi o ka ʻiʻo (biopsy) mai kahi ʻāpana o kou kino, e like me kou tonsils, ʻili, a i ʻole ke akepaʻa. Hiki ke hoʻohana ʻia ke ʻano o ka momona i loko o kēia mau ʻiʻo e loaʻa ai kahi manaʻo o ka maʻi.

He aha ka protein i nele i ka maʻi Tangier?

I ka maʻi Tangier, ua hoʻemi nui ʻia kahi protein i kapa ʻia ʻo apolipoprotein A1 (ApoA1) . ʻO kēia ApoA1 kahi ʻāpana nui o ka molekole cholesterol HDL. No laila, ke emi ka ApoA1, e emi pū nā pae HDL.

He aha nā hoʻokolohua i hoʻohana ʻia no ka hōʻoia ʻana a me ka hahai ʻana?

Hiki paha i nā kauka ke hana i kekahi mau hoʻokolohua ʻē aʻe e ʻike ai i ka maʻi a nānā i nā hopena o ka maʻi ma ke kino.

• Nā hoʻāʻo hana aʻalolo a me nā ʻiʻo (Electromyograms / EMGs): E nānā no ka hōʻino ʻana o nā aʻalolo a me ka nāwaliwali o nā ʻiʻo.
• Hoʻokolokolo maka: E nānā no kekahi ʻike maka pōwehiwehi a pilikia paha.
• ʻO ke ultrasound ma kou ʻōpū / ʻōpū: E nānā i ke kūlana o nā ʻōpū e like me ke akepaʻa a me ka spleen.
• ʻO ke ultrasound ma kou mau aʻa carotid: E nānā i nā waihona momona ma nā aʻa nui ma kou ʻāʻī.
• ʻO ke CT angiogram o kou puʻuwai:E nānā i nā mea i ālai ʻia i loko o nā aʻa e hoʻolako ana i ke koko i ka puʻuwai.
• Echocardiogram: He scan e nānā i ka hana a me ke ʻano o ka puʻuwai.
• Hoʻāʻo koʻikoʻi hoʻoikaika kino: E ʻike pehea e pane ai ka puʻuwai i ka hoʻoikaika kino.

Pehea e mālama ʻia ai ka maʻi Tangier?

ʻO kaʻoiaʻiʻo, ʻaʻohe lāʻau lapaʻau kikoʻī e hiki ke ho'ōla loa i ka maʻi Tangier. No ka mea he maʻi genetic ia, nui nā mea hiki iā ʻoe ke hana e kāohi i nā hōʻailona a hōʻemi i nā pilikia.

I kekahi manawa, pono e wehe ʻia kahi ʻōkana i hōʻino ʻia e nā waihona momona, no ka laʻana me nā tonsils a i ʻole ka spleen, ma ke ʻoki kino .

Hiki i ka meaʻai a me ka mea inu ke kōkua?

ʻAe, hiki maoli nō ia. Aia kekahi mau meaʻai e hiki ke kōkua i ka hoʻonui iki ʻana i ko mākou pae cholesterol maikaʻi (HDL), a kōkua pū kekahi i ka hoʻēmi ʻana i ko mākou pae cholesterol maikaʻi ʻole (LDL).

• ʻAvokado
• ʻAila ʻoliva
• Nā legumes e like me nā pī a me nā chickpeas (Beans)
• Nā palaoa piha (e.g., palaoa piha, laiki palaoa piha)
• Nā iʻa momona ( e.g. salmon, mackerel, herring)
• Nā nati ( e.g. pīnati, ʻalemona, cashews - akā me ka paʻakai ʻole)
• nā huaʻai i loaʻa ka nui o ka fiber (e.g. guava, mango, banana)
• Nā hua Chia a me nā hua flax

ʻO ka hoʻāʻo ʻana e hoʻokomo i kēia mau meaʻai i kāu papaʻai i nā manawa a pau e kōkua i ka mālama ʻana i nā pae cholesterol maikaʻi.

He aha nā lāʻau lapaʻau a me nā hoʻololi o ke ʻano o ka nohona?

Hiki i kāu kauka ke kuhikuhi i nā lāʻau hoʻohaʻahaʻa cholesterol , e like me nā statins.

Paipai pū ia e hana i kekahi mau hoʻololi nohona e hiki ke kōkua i ka hoʻonui ʻana i nā pae HDL:

• Hoʻomaʻamaʻa pinepine: Hoʻokomo pū kēia me ka hele wāwae a holo paha no ka liʻiliʻi he 30 mau minuke i ka lā.
• ʻAʻole hoʻohana i nā huahana paka: Pono ʻoe e hoʻōki loa i ka puhi paka.
• Ke noho nei i ke kaumaha olakino.
• E ʻai i nā momona monounsaturated ma kahi o nā momona saturated: Loaʻa nā momona saturated i nā mea e like me ka ʻaila niu, ka ʻaila pama, a me nā ʻaila holoholona. Loaʻa nā momona monounsaturated i ka ʻaila ʻoliva a me nā avocados.

He aha nā hopena ʻaoʻao o ka lāʻau lapaʻau?

Hiki i nā lāʻau hoʻohaʻahaʻa kolesterol ke hana i nā hopena ʻaoʻao liʻiliʻi i kekahi manawa. ʻAʻole loaʻa i nā kānaka a pau, akā he mea maikaʻi ke ʻike iā lākou.

• Nā ʻeha poʻo
• ʻEha ʻiʻo
• Ka paʻa ʻana o ka ʻōpū
• ʻŌpū ʻeha

Inā hana ʻia kekahi mea e like me kēia, pono ʻoe e haʻi i kāu kauka. A laila hiki iā ia ke hoʻololi i ka lāʻau lapaʻau a hāʻawi paha iā ʻoe i nā ʻōlelo aʻoaʻo ʻē aʻe.

Hiki ke pale ʻia ka maʻi Tangier?

ʻOiai he maʻi hoʻoilina kēia, ʻaʻole hiki iā mākou ke pale aku i kona ulu ʻana. ʻO ke ʻano kēia, inā loaʻa iā ʻoe kēia kīnā i loko o kou mau genes, ʻaʻole hiki ke hoʻololi ʻia. Eia nō naʻe, inā loaʻa iā ʻoe kēia maʻi, a inā he mea lawe ʻoe, hiki iā ʻoe ke kūkākūkā me kahi kākāʻōlelo hoʻoilina e ʻike i ka pilikia o ka hoʻoilina ʻana o kāu mau keiki. A laila, me ka noʻonoʻo ʻana i ke kūlana hoʻoilina o kekahi makua, hiki iā ʻoe ke loaʻa kahi manaʻo maopopo o ka hiki i kāu mau keiki ke loaʻa.

Inā loaʻa iaʻu ka maʻi Tangier, he aha ka mea e hiki mai ana i ka wā e hiki mai ana?

ʻO ka wānana no ka poʻe me ka maʻi Tangier ma ke ʻano laulā he maikaʻi, akā pili ia i ka holomua o kāu mau hōʻailona. E like me nā maʻi āpau, ʻoi aku ka maikaʻi o ka ʻike mua ʻana , ma mua o ka lilo ʻana o nā hōʻailona i mea koʻikoʻi.

ʻOiai ʻaʻohe lāʻau lapaʻau paʻa no kēia, aia ka manaʻolana e kōkua nā mea e like me ka gene therapy i ka wā e hiki mai ana. Ke hoʻomau nei ka noiʻi.

Pehea ka lōʻihi o ke ola ʻana o ka maʻi Tangier?

ʻOiai he maʻi hoʻoilina kēia, no ke ola holoʻokoʻa ia. Eia nō naʻe, inā ʻike mua ʻia a nānā mau ʻia, hiki ke mālama maikaʻi ʻia kēia maʻi a ola pū me ia.

Pehea wau e mālama ai iaʻu iho?

Inā he ʻōpū nui kou ma muli o ka maʻi Tangier, pono ʻoe e pale i nā haʻuki pili, no ka mea, hiki i kēlā ʻano pili ke kumu o ka haki ʻana o ka ʻōpū, he mea weliweli ia.

Eia kekahi, pono e kaohi ʻia nā kumu pilikia ʻē aʻe no ka maʻi puʻuwai:

• Ke kāohi nei i ke koko kiʻekiʻe .
• Inā loaʻa iā ʻoe ka maʻi diabetes , e kaohi pono iā ia.
• Ke pale nei i ka hoʻohana ʻana i nā huahana paka.

Āhea wau e ʻike ai i ke kauka?

Inā loaʻa iā ʻoe ka maʻi Tangier, pono ʻoe e ʻike pinepine i kāu mea mālama ola . Ma kēlā ala, inā e ʻoi aku ka ʻino o kāu mau hōʻailona, ​​​​hiki iā ʻoe ke hana koke.

E nānā pinepine ke kauka i kēia mau mea:

• ʻŌnaehana aʻalolo.
• Puʻuwai.
• Nā maka.

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

Ke ʻike ʻoe i kāu kauka, hiki iā ʻoe ke nīnau i nā nīnau e like me kēia:

• Pono anei au e lawe i nā lāʻau lapaʻau e hoʻomaikaʻi i koʻu pae cholesterol?
• Aia kekahi hui kākoʻo e hiki i ka poʻe me ka maʻi Tangier ke hui pū?
• ʻEhia pinepine e pono ai iaʻu ke hele e ʻike iā ʻoe?

ʻOiai hiki i ka maʻi Tangier ke hoʻopilikia i nā ʻāpana like ʻole o ke kino, ʻoi aku ka maikaʻi o ka nānā mau ʻana no kēlā me kēia ʻāpana. Pono paha ʻoe e ʻike i kekahi mau loea like ʻole. E lawe i ka lāʻau lapaʻau e like me ke kauoha a kāu kauka, a e hana i kāu mea maikaʻi loa e mālama i kahi papaʻai olakino a me ka hoʻoikaika kino.

Nā mea nui a mākou e hoʻomanaʻo ai (Leka Lawe-Home)

ʻAe, e ʻae mai iaʻu e hōʻuluʻulu i kekahi o nā mea e pono ai ʻoe e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai.

• He maʻi laha ʻole ka maʻi Tangier, he maʻi hoʻoilina e hoʻemi nui ai i ka cholesterol maikaʻi (HDL).
• Hiki i kēia ke hana i ka cholesterol maikaʻi ʻole (LDL) a me ka momona e hōʻiliʻili i loko o ke kino a hoʻopilikia i nā ʻōpū e like me ke ake, ka spleen, nā tonsils, a me ka puʻuwai.
• ʻOkoʻa nā hōʻailona, ​​​​a loaʻa paha i kekahi poʻe nā pilikia neurological, nā pilikia ʻili, a me nā pilikia ʻike.
• Hana ʻia kēia e kahi kīnā i loko o ka gene ABCA1 .
• Hoʻomaopopo ʻia ka maʻi ma o nā hoʻāʻo koko, nā hoʻāʻo genetic, a i kekahi manawa nā hoʻāʻo ʻiʻo (biopsies) .
• ʻOiai ʻaʻohe lāʻau lapaʻau kikoʻī, hiki ke kaohi ʻia nā hōʻailona me ka ʻai, nā loli o ke ʻano o ka nohona, nā lāʻau lapaʻau, a me kekahi mau ʻoki kino.
• He mea nui ka ʻike mua ʻana a me ka nānā pinepine ʻana i ke kauka .

No laila, ke lana nei koʻu manaʻo he kōkua kēia ʻike iā ʻoe. Inā he mau nīnau hou aku kāu e pili ana i kēia, mai kānalua e kamaʻilio me ke kauka. E noho olakino!

ʻO ka maʻi Tangier, ka cholesterol HDL, ka cholesterol LDL, nā maʻi genetic, ka mālama ʻana i ka cholesterol, ka pilikia o ka maʻi puʻuwai, ApoA1