Chylomicronemia maʻi maʻi

ʻO Chylomicronemia maʻi kahi maʻi kahi e haki ʻole ai ke kino i nā momona (lipids) pololei. ʻO kēia ke kumu o nā momona momona i kapa ʻia nā chylomicrons e kūkulu i loko o ke koko. Ua hala ka maʻi i waena o nā ʻohana.

Hiki i ka Chylomicronemia syndrome ke hana ma muli o kahi maʻi genetika ʻike ʻia kahi protein (enzyme) i kapa ʻia lipoprotein lipase (LpL) i haki a nalowale paha. Hiki iā ia ke kumu i ka loaʻa ʻole o ka helu ʻelua i kapa ʻia ʻo apo C-II, nāna e hoʻoheheʻe iā LpL. Loaʻa ʻo LpL i ka momona a me nā mākala. Kōkua ia e wāwahi i kekahi mau lipids. Ke nalowale a haki paha ʻo LpL, kūkulu nā mea momona i kapa ʻia he chylomicrons i loko o ke koko. Kapa ʻia kēia kūkulu ʻana ʻo chylomicronemia.

ʻO nā hemahema i ka apolipoprotein CII a me ka apolipoprotein AV hiki ke kumu i ka maʻi pū kekahi. Hiki ke hana ʻia i ka wā o ka poʻe i predisposed e loaʻa i nā triglycerides kiʻekiʻe (e like me ka poʻe i loaʻa i ka hyperlipidemia i hui ʻia a ʻohana a i ʻole hypertriglyceridemia ʻohana) e hoʻomohala i ka maʻi diabetes, ka momona a i ʻole e hōʻike ʻia i kekahi mau lāʻau.

E hoʻomaka paha nā ʻōuli ma ka wā kamaliʻi a hoʻopili i:

• ʻO ka ʻeha o ka ʻōpū ma muli o ka pancreatitis (ka wela o ka pancreas).
• Nā ʻōuli o ka hōʻino o ke aʻalolo, e like me ka nalo ʻana o ka manaʻo i nā wāwae a i ʻole nā ​​wāwae, a me ka nalo ʻana o ka hoʻomanaʻo.
• ʻO nā waiho melemele o nā mea momona i ka ʻili i kapa ʻia he xanthomas. Hiki ke ʻike ʻia kēia ulu ʻana ma ke kua, ka ʻāpā, ka wāwae o ka wāwae, a i ʻole nā ​​kuli a me nā kuʻe lima.

E hōʻike paha kahi hōʻike kino a me nā hoʻāʻo.

• Hoʻonui i ke ake a me nā ʻāpala
• Ka hoʻohaunaele o ka pancreas
• Waihona momona ma lalo o ka ʻili
• ʻO nā waihona momona paha i ka retina o ka maka

E ʻike ʻia kahi papa momona i ka wā e kolo ai ke koko i ka mīkini hale hana. Pili kēia papa i nā chylomicrons i ke koko.

Kiʻekiʻe loa ka pae triglyceride.

Koi ʻia kahi papaʻai momona, ʻaihue ʻole. Pono paha ʻoe e hōʻoki i ka lawe ʻana i kekahi lāʻau i hiki ke hōʻeha i nā ʻōuli. Mai hōʻoki i ka lawe ʻana i nā lāʻau me ka ʻole e kamaʻilio mua me kāu kauka olakino. ʻO nā kūlana e like me ka make wai a me ka maʻi kō hiki ke hōʻino i nā ʻōuli. Inā ʻike ʻia, pono e mālama ʻia kēia mau ʻano.

Hiki i kahi papaʻai momona ʻole ke hōʻemi i nā ʻōuli.

Ke mālama ʻole ʻia, hiki i nā chylomicrons keu aku ke alakaʻi i ka pā o pancreatitis. Hiki ke hōʻeha loa kēia ʻano a hoʻoweliweli hoʻi i ke ola.

E ʻimi koke i ka lāʻau lapaʻau inā loaʻa iā ʻoe ka ʻeha o ka ʻōpū a i ʻole nā ​​hōʻailona ʻē aʻe o ka pancreatitis.

Kāhea i kāu mea hāʻawi inā loaʻa iā ʻoe kahi moʻolelo pilikino a ʻohana paha o nā pae triglyceride kiʻekiʻe.

ʻAʻohe ala e pale aku ai i kekahi mai ka hoʻoili ʻana i kēia maʻi maʻi.

Ka nele o ka lipoprotein lipase ʻohana; Familial hyperchylomicronemia syndrome, ʻAno I hyperlipidemia

• Hepatomegaly
• ʻO Xanthoma ma ke kuli

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.

Robinson JG. Nā maʻi o ka lipid metabolism. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 195.