Ka nele o ka lipoprotein lipase ʻohana

ʻO ka hemahema lipoprotein lipase ʻohana kahi hui o nā ʻano maʻi laha ʻole kahi e nele ai ke kanaka i kahi protein e pono ai e wāwahi i nā mole momona. Hoʻokumu ka maʻi i ka nui o nā momona e kūkulu i loko o ke koko.

Hoʻokumu ʻia ka hemahema lipoprotein lipase ʻohana e kahi ʻāpana hemahema i hoʻoili ʻia i waena o nā ʻohana.

ʻO ka poʻe me kēia ʻano hemahema i kahi enzyme i kapa ʻia lipoprotein lipase. Me ka ʻole o kēia enzyme, ʻaʻole hiki i ke kino ke wāwahi i ka momona mai nā meaʻai i hoʻoulu ʻia. Kūkulu nā mea momona i kapa ʻia nā chylomicrons i loko o ke koko.

ʻO nā kumu pilikia me kahi moʻolelo ʻohana o ka hemahema lipoprotein lipase.

ʻIke pinepine ʻia ke ʻano i ka wā pēpē a kamaliʻi paha.

Pākuʻi ʻia paha nā ʻōuli i kekahi o kēia mau mea:

• Paʻi ʻōpū (hiki ke ʻike ʻia ma ke ʻano he colic i nā pēpē)
• Loaʻa ka ʻai ʻole
• Nualea, luaʻi
• ʻEha ma nā mākala a me nā iwi
• Hoʻonui i ke ake a me nā ʻāpala
• ʻO ka holomua ʻole i ke keiki hānai
• Waihona momona i ka ʻili (xanthomas)
• Nā pae triglyceride kiʻekiʻe i ke koko
• ʻO nā retina pale a me nā kīʻaha koko keʻokeʻo i nā retina
• ʻO ka mumū mau o ka pancreas
• Yellowing o nā maka a me ka ʻili (jaundice)

E hana ka mea mālama ola i kahi hoʻokolokolo kino a nīnau e pili ana i nā hōʻailona.

E hana ʻia nā hoʻokolohua koko e nānā i nā pae kolesterol a me nā triglyceride. I kekahi manawa, hana ʻia kahi hoʻāʻo koko kūikawā ma hope o kou hāʻawi ʻia ʻana i ka lahilahi o ke koko ma o kahi aa koko. Nānā kēia hōʻoia no ka hana lipoprotein lipase i kou koko.

E hana ʻia paha nā hoʻāʻo genetic.

Hoʻolālā ka mālama ʻana e kaohi i nā ʻōuli a me nā pae triglyceride koko me ka papaʻai momona momona loa. E paipai paha kāu mea hāʻawi iā ʻoe e ʻai ma mua o 20 mau momona o ka momona i kēlā me kēia lā e pale i nā hōʻailona mai ka hoʻi ʻana.

Iwakāluakūmālima gram o ka momona e like me kekahi o kēia:

• ʻElua mau aniani 8-auneke (240 milliliters) o ka waiū holoʻokoʻa
• 4 teaspoons (9.5 gram) o margarine
• 4 auneke (113 gram) lawelawe i kaʻiʻo

ʻO ka ʻai maʻamau o ʻAmelika ka momona o ka ʻai a hiki i 45% o ka nui o nā calorie. Paipai ʻia nā huaora A, D, E, a me K a me nā mea hoʻopili mineral i mea momona no nā poʻe e ʻai i ka papa momona momona. E makemake paha ʻoe e kūkākūkā i kāu mau pono papaʻai me kāu mea hoʻolako a me kahi dietitian i hoʻopaʻa inoa ʻia.

ʻO Pancreatitis e pili ana i ka lipoprotein lipase deficit e pane i nā mālama ʻana no kēlā maʻi.

Hiki i kēia mau kumuwaiwai ke hāʻawi i ka ʻike hou aku e pili ana i ka hemahema lipoprotein lipase ʻohana:

• National Organization for Rare Disorder - rarediseases.org/rare-diseases/familial-lipoprotein-lipase-deficiency
• NIH Genetics Home Reference - ghr.nlm.nih.gov/condition/familial-lipoprotein-lipase-deficiency

ʻO nā kānaka me kēia ʻano e ukali nei i ka papaʻai momona momona hiki ke noho i ke kūlana makua.

Hiki ke ulu i ka Pancreatitis a me nā hanana hou o ka ʻeha o ka ʻōpū.

ʻAʻole ʻeha ka Xanthomas i ka maʻamau ke ʻole ʻōpala nui ʻia lākou.

Kāhea i kāu mea lawelawe no ka loiloi inā loaʻa i kekahi o kou ʻohana ka hemahema lipoprotein lipase. Paipai ʻia ka ʻōlelo aʻoaʻo genetic no kēlā me kēia me ka moʻolelo ʻohana o kēia maʻi.

ʻAʻohe mea i ʻike ʻia no ka pale ʻana no kēia maʻi kūpilikiʻi. ʻAe ka hoʻomaopopo ʻana i nā pilikia i ka ʻike mua ʻana. Ma hope o ka papaʻai momona momona momona hiki ke hoʻomaikaʻi i nā hōʻailona o kēia maʻi.

ʻAno I hyperlipoproteinemia; Fameliial chylomicronemia; Ka nele o ka LPL ʻohana

• Maʻi maʻi ʻaʻai Coronary

ʻO Genest J, Libby P. Nā maʻi lipoprotein a me nā maʻi maʻi ʻōpū. I: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. ʻO ka maʻi puʻuwai ʻo Braunwald: He puke aʻo no ka lāʻau Cardiovascular. 11th hua. Piladelapia, PA: Elsevier; 2019: chap 48.

Semenkovich CF, Goldberg AC, Goldberg IJ. Nā maʻi o ka lipid metabolism. I: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. ʻO Williams Textbook of Endocrinology. 13th ed. Piladelapia, PA: Elsevier; 2016: chap 37.