ʻO kahi pilikia i loaʻa i nā kānaka he nui i kēia mau lā, ʻo ia ka piʻi ʻana o nā momona e like me ka cholesterol a me nā triglycerides i loko o ke koko. Akā i kekahi manawa, ʻo kēia mau pae momona, ʻoi aku ka nui o nā pae triglyceride, he mau tausani manawa kiʻekiʻe ma mua o ka maʻamau. ʻO ke kumu o kēia paha he ʻano genetic kakaikahi loa i hoʻoili ʻia mai nā mākua mai. I kēia lā, ke kamaʻilio nei mākou e pili ana i kekahi ʻano maʻi, ʻo Familial Hyperchylomicronemia Syndrome, a i ʻole FCS no ka pōkole. ʻOiai he kumuhana paʻakikī kēia, e hoʻomaopopo maʻalahi kākou.
I ka ʻōlelo maʻalahi, he aha ka FCS?
He maʻi laha ʻole ka FCS i hoʻoilina ʻia mai kou mau mākua mai. ʻO ke kanaka me kēia maʻi, ʻaʻole hiki iā ia ke wāwahi, a hoʻoheheʻe paha, i nā momona, ʻoiai hoʻi nā triglycerides .
E noʻonoʻo he hale hana liʻiliʻi ko ko kākou kino e wāwahi i ka momona. Ma nā ʻōlelo lapaʻau, he enzyme kēia i kapa ʻia ʻo lipoprotein lipase (LpL) . I kekahi me FCS, ʻaʻole hana pono kēia 'hale hana', a ʻaʻole paha e hana iki.
ʻO ka hopena, ʻaʻole hiki ke omo ʻia ka momona i loko o ka meaʻai a mākou e ʻai ai, i kapa ʻia ʻo triglycerides, e ke kino a hōʻiliʻili ia i loko o ke koko ma ke ʻano he mau kulu momona i kapa ʻia ʻo chylomicrons . ʻO kēia hōʻiliʻili ʻana o nā chylomicrons ka mea a mākou e kapa nei he chylomicronemia syndrome. ʻO kēia ke kumu o nā pae triglyceride kiʻekiʻe loa i loko o ke koko.
He mea kākaʻikahi loa ia ʻo hoʻokahi a ʻelua paha kānaka i hoʻokahi miliona ma ka honua holoʻokoʻa e hoʻopilikia ʻia. ʻIke pinepine ʻia ma mua o ka piha ʻana o 10 mau makahiki, a ua ʻike ʻia kekahi mau keiki me ke kūlana i loko o ka makahiki mua o ke ola.
No ke aha lā kēia e hana nei?
ʻO ke kumu nui o kēia, he loli hoʻoilina ia. ʻO ke kumu o kēia, he kīnā i loko o nā genes e pili ana i ka hana ʻana i ka enzyme lipoprotein lipase (LpL) a mākou i kamaʻilio ai.
ʻO ka mea nui , no ka loaʻa ʻana o kēia maʻi, pono ʻoe e hoʻoilina i kēia gene kīnā mai kou makuahine a me kou makuakāne. Inā hoʻoilina ʻoe mai hoʻokahi makua wale nō, ʻaʻole ʻoe e loaʻa i ka maʻi, akā e lilo ʻoe i 'mea lawe' o kēlā gene. ʻO ia hoʻi, loaʻa iā ʻoe ka hiki ke hoʻoili i kēia gene i kāu mau keiki.
Hoʻopuka ʻia ka enzyme LpL e ko kākou pancreas. ʻO kāna hana nui ka wāwahi ʻana i nā chylomicrons i loko o ke koko a kōkua i ke kino e hoʻohana i ka momona i loko o lākou no ka ikehu. No laila, ke hana pono ʻole ʻo LpL, ʻaʻole hiki ke wāwahi ʻia ka momona a piʻi nui nā pae triglyceride i loko o ke koko.
He aha nā hōʻailona o kēia?
ʻO nā hōʻailona o FCS ke kumu o nā pae kiʻekiʻe o nā triglycerides i loko o ke koko. ʻOi aku ka liʻiliʻi o nā pae triglyceride o ke kanaka olakino ma mua o 150 mg/dL. Eia nō naʻe, i loko o kekahi me FCS, hiki i kēia pae ke ʻoi aku ma mua o 1,000 mg/dL. He mea nui loa ka ʻike ʻana i kēia mau hōʻailona.
| Hōʻailona | He wehewehe maʻalahi |
|---|---|
| ʻEha ʻōpū | ʻO kēia ka hōʻailona maʻamau. Hiki i kēia ʻeha manawa ʻole ke hoʻomaka ma ka ʻōpū o luna a pālahalaha i ke kua. I kekahi manawa hiki ke koʻikoʻi loa. |
| ʻO ka maʻi pancreatitis | ʻO kēia ke kumu pinepine o ka ʻeha o ka ʻōpū. He ʻōkana koʻikoʻi ka pancreas i loko o ko kākou ʻōpū. ʻO ia ka manawa e pehu ai. Hiki ke hele pū mai nā hōʻailona e like me ka nausea, ka hou ʻana, ka nāwaliwali, a me ka melemele o nā maka a me ka ʻili. |
| Nā loli ʻili | Loaʻa i kekahi poʻe kahi maʻi i kapa ʻia ʻo cutaneous xanthomas . He mau puʻupuʻu liʻiliʻi ʻulaʻula-melemele kēia, ʻaʻohe ʻeha e ʻike ʻia ma nā wahi e like me ka puʻupuʻu, nā kuli, a me nā kuʻekuʻe lima. Hana ʻia kēia mau mea i ka momona. Inā ʻike ʻoe i kēia mau mea, he hōʻailona paha ia o ka momona koko kiʻekiʻe. |
| Nā loli i nā maka | ʻO Lipaemia retinalis kahi ʻano i ʻike ʻia ai nā kīʻaha koko i loko o ka maka me he waiū lā. Hoʻokumu ʻia kēia e ka hōʻiliʻili ʻana o ka momona. Eia naʻe, ʻaʻole ia e hōʻeha i ka ʻike. |
| Ka hoʻonui ʻia ʻana o ke akepaʻa a me ka spleen | ʻOi loa aku i nā kamaliʻi ʻōpio, hoʻāʻo kekahi ʻano o ke koko keʻokeʻo (macrophages) e omo i ka momona keu i loko o ke kino. Omo kēia mau pūnaewele i ka momona a waiho i loko o ke akepaʻa a me ka spleen, e hoʻonui ana i kēlā mau ʻōkana. |
| Nā hōʻailona noʻonoʻo a me nā neurological | Hiki i kekahi mau maʻi ke loaʻa i nā kūlana e like me ke kaumaha, ka nalowale o ka hoʻomanaʻo, a me ka dementia. |
Pehea e ʻike ʻia ai kēia a mālama ʻia?
ʻIkepili
No ka mea he maʻi kakaikahi ia, hiki i kekahi manawa ke lōʻihi ka loaʻa ʻana o kahi hōʻailona pololei. Inā loaʻa iā ʻoe ka ʻeha ʻōpū mau, nā hoʻouka pinepine ʻana o ka pancreatitis, a me nā pae triglyceride kiʻekiʻe i loko o kou koko, kānalua paha kāu kauka iā FCS.
Hana ʻia nā hoʻokolohua aʻe e hōʻoia i ka maʻi:
- Hoʻāʻo koko triglyceride hoʻokē ʻai: Hana ʻia kēia hoʻāʻo ma hope o ka hoʻokē ʻai ʻana no kekahi mau hola. Inā ʻoi aku ke kiʻekiʻe o ka pae triglyceride ma mua o 750 mg/dL, he FCS paha ia. I kekahi manawa e like paha ke ʻano o ka hāpana koko me ka waiū.
- Ka hoʻāʻo ʻana i ka pae Lipase: Hāʻawi ʻia kahi inikini kūikawā (heparin) e ana i ka nui o ka enzyme LpL i hana ʻia e ke kino.
- Nā hoʻāʻo hoʻoilina: ʻO kēia wale nō ke ala e maopopo ai he 100%. Hiki iā ia ke nānā inā ua hoʻoilina ʻia ka gene kīnā mai nā mākua ʻelua.
- CT scan: Kōkua kēia e ʻike inā he hōʻino i nā ʻōpū e like me ka pancreas a me ke ake.
Nā ʻano lapaʻau
ʻO ka ʻai ka mea nui o ka mālama ʻana no FCS, a aia kekahi lāʻau hou i ʻāpono ʻia nei.
ʻO ka mea nui loa: ʻO nā lāʻau lapaʻau e like me nā statins a me nā fibrates, ka mea i hāʻawi pinepine ʻia no ka cholesterol, ʻaʻole lākou e hana no FCS. No ka mea, i mea e hana ai kēlā mau lāʻau lapaʻau, pono e hana pono ka enzyme LpL a mākou i kamaʻilio ai ma mua i loko o ke kino.
ʻAi no FCS
ʻO kēia ka ʻāpana koʻikoʻi loa o ka hoʻokele ʻana. He mea nui e hana pū me kāu kauka a me kahi meaʻai meaʻai e hoʻomohala i kēia papaʻai.
- E kaupalena i ka momona: ʻO ka nui o ka momona i hoʻopau ʻia i kēlā me kēia lā e emi iho ma mua o 20 grams.
- E ho'ōki loa i ka inu ʻona: Hoʻonui hou aku ka waiʻona i nā pae triglyceride.
- E kaupalena i nā kō maʻalahi a me nā kalapona: E hōʻalo i nā mea e like me nā mea inu kō a me nā mea ʻono.
- Nā mea e ʻai ai: Nā mea kanu, nā huaʻai, nā legume, nā palaoa piha, nā keʻokeʻo hua manu, nā huahana waiū momona ʻole, a me nā ʻiʻo wīwī.
- Nā mea hoʻohui huaora: Hiki i kāu kauka ke ʻōlelo e lawe i nā huaora momona e like me nā huaora A, D, E, a me K, kahi mea e pono ai ke kino e hōʻemi i ka momona.
Lāʻau lapaʻau hou
ʻOlezarsen (Tryngolza)He lāʻau lapaʻau hou kēia i ʻāpono ʻia i Dekemaba 2024. He inikini ia i hoʻokomo ʻia ma lalo o ka ʻili i hoʻokahi manawa i ka mahina. I ka ʻōlelo maʻalahi, hana ia ma ka hōʻemi ʻana i ka hana ʻana o ke kino i kahi protein i kapa ʻia ʻo apoC-III, kahi e kōkua ai i ka hōʻiliʻili ʻana o ka momona i loko o ke koko. Hiki iā ʻoe ke aʻo hou aʻe e pili ana i kēia mai kāu kauka.
Nā pilikia o ke ola ʻana me FCS
He kūlana koʻikoʻi ka FCS no ke ola holoʻokoʻa e hiki ke hoʻololi nui i ke olakino kino a me ka noʻonoʻo.
- Nā pilikia ʻai: Hiki ke paʻakikī loa ka ʻai ʻana i waho a me ka hele ʻana i nā pāʻina.
- Nā hopena noʻonoʻo: ʻO ka ʻeha pinepine, ka makaʻu i ka wā e hiki mai ana, ka hopohopo, a me ka "noe lolo" e hiki mai paha.
- Ka hopena i ke ola kaiaulu a me ka hana: ʻO ka hoʻokipa pinepine ʻana i ka haukapila hiki ke paʻakikī i ka mālama ʻana i nā pilina pili kaiaulu a me ka hana.
No laila, he mea nui loa e hana pū me kāu hui lapaʻau a, inā pono, e ʻimi i ke kākoʻo o kahi kākā'ōlelo olakino noʻonoʻo.
Leka Lawe-Home
- He kūlana laha ʻole ka FCS i hiki ʻole ai i ke kino ke wāwahi i nā momona (triglycerides).
- ʻO kēia ke kumu o ke kiʻekiʻe o nā pae triglyceride i loko o ke koko, a ʻo nā hōʻailona nui ka ʻeha nui o ka ʻōpū a me ka mumū o ka pancreas (pancreatitis).
- ʻO ka hapa nui o ka mālama ʻana, ʻo ia ka hahai ʻana i kahi papaʻai momona paʻa loa . Pono e pale loa ʻia ka waiʻona.
- ʻAʻole hana nā lāʻau lapaʻau cholesterol maʻamau no kēia ʻano, akā aia kahi lāʻau hou i hoʻolauna ʻia.
- He pilikia ke ola holoʻokoʻa ka mālama ʻana i kēia maʻi, no laila he mea nui e loaʻa ke kākoʻo o kāu kauka, ka meaʻai meaʻai, a me ka ʻohana.
- Inā loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana kēia mau hōʻailona, e ʻike koke i kāu kauka a e ʻimi i ka ʻōlelo aʻo.
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