Nā kumu o ka maʻi Alagille a pehea e mālama ai

Anter

• Nā hōʻailona kūpono
• Nā kumu o ka maʻi Alagille
• ʻIke o Alagille Syndrome
• Hoʻomaʻo no ka maʻi Alagille

ʻO ka maʻi ʻo Alagille kahi maʻi maʻi laha ʻole e hoʻopili nui i nā ʻōpala, keu hoʻi i ke akepaʻa a me ka puʻuwai, a hiki ke make. Hoʻohālikelike ʻia kēia maʻi e ka lawa ʻole o nā aʻa a me nā hepatic duct, no laila ke hōʻiliʻili ʻia nei o ka bile i ke akepaʻa, ka mea e ālai iā ia mai ka hana maʻamau e hoʻopau i nā ʻōpala mai ke koko.

Hōʻike mau ʻia nā ʻōuli i ka wā kamaliʻi, a ʻo ia nō paha ke kumu o ka hoʻolōʻihi lōʻihi ʻia i nā keiki hānau hou. I kekahi mau hihia, ʻike ʻole ʻia nā ʻōuli, e hōʻeha ʻole ai i nā hōʻino a i nā hihia ʻoi aku paha ke koʻikoʻi e loli i nā lālā i loli.

Nā hōʻailona kūpono

Ma waho o ka lawa ʻole o ke kahena o ka bile, hana ʻo Alagille Syndrome i nā ʻano hōʻailona a me nā ʻōuli, e like me:

• ʻIli melemele;
• Nā kina o ka maka;
• Iwi iwi kuamoʻo i ke kinona o ka butterfly;
• Pihi ka lae, ka auwae a me ka ihu;
• Nā pilikia puʻuwai;
• Hoʻolālā hoʻomohala;
• ʻIliʻu ākea;
• Waiho Cholesterol ma ka ʻili;
• Perenal pulmonary stenosis;
• Hoʻololi ophthalmic.

Ma waho aʻe o kēia mau ʻōuli, hiki i ke kōpaʻa ʻole ke holo holomua, nā ʻano puʻuwai a me nā puʻupaʻa. Ma ka laulaha, paʻa ka maʻi ma waena o 4 a 10 mau makahiki o ka makahiki, akā i ke alo o ka hake a me ka puʻuwai o ka puʻuwai, ʻoi aku ka nui o ka make o ka make.

Nā kumu o ka maʻi Alagille

ʻO ka maʻi Alagille kahi maʻi autosomal nui, ʻo ia hoʻi inā loaʻa kēia pilikia i kekahi o nā mākua o ke keiki, 50% ka nui o ke keiki i ka maʻi. Eia nō naʻe, hiki ke loli i ke keiki, ʻoiai inā olakino nā mākua ʻelua.

Hoʻokumu ʻia kēia maʻi ma muli o nā loli a i ʻole ka hoʻololi ʻana i ke kaʻina DNA e hoʻopili ai i kahi ʻāpana kikoʻī, aia ma ka chromosome 20, ʻo ia ke kuleana no ka hana maʻamau o ke ake, ka puʻuwai a me nā mea ʻē aʻe, i hana ʻole iā lākou e hana maʻamau.

ʻIke o Alagille Syndrome

Ma muli o ke kumu o nā hōʻailona he nui, hiki ke hana i ke kuhi o kēia maʻi i nā ʻano he nui, ʻo ka mea maʻamau o ia mea he biopsy ate.

Ka nānā ʻana i nā ʻōuli a me nā ʻōuli

Inā he melemele ka ʻili, a inā he ʻano ʻē ka helehelena a me nā ʻeʻepa, nā pilikia o ka puʻuwai a me nā puʻupaʻa, nā loli ophthalmic, a i ʻole ke lohi hoʻomohala ʻana, e ʻeha paha ke keiki i kēia maʻi. Eia nō naʻe, aia kekahi mau ala ʻē aʻe e hōʻike ai i ka maʻi.

Ke ana ʻana i ka hana o nā pancreas

Hiki i nā hoʻokolohua ke hana ʻia e loiloi i ka hana o ka pancreas, e hoʻoholo ana i ka nui o ka momona e lawe ʻia e ka meaʻai e ʻai ʻia e ke keiki, ma o ka loiloi ʻia o nā feces. Eia nō naʻe, pono e hana hou ʻia nā hoʻokolohua hou aku, ʻoiai ʻo kēia hōʻike wale nō he hōʻailona o nā maʻi ʻē aʻe.

ʻO ka loiloi e kahi cardiologist

Hiki i ka cardiologist ke hoʻomaopopo i kahi pilikia o ka naʻau ma o ka echocardiogram, kahi o ka ultrasound o ka puʻuwai e ʻike i ke ʻano a me ka hana ʻana, a i ʻole ma o ka electrocardiogram e ana ai i ka puʻuwai puʻuwai.

ʻO ka loiloi e kahi ophthalmologist

Hiki i ka ophthalmologist ke hana i kahi hoʻokolohua maka ʻike loea i mea e ʻike ai i nā mea kūpono ʻole, i nā haunaele o ka maka a i ʻole nā ​​hoʻololi o ka pigmentation i ka retina.

X-ray loiloi i ka iwi kuamoʻo 

ʻO ka hana ʻana i kahi X-ray i ka iwi kuamoʻo hiki ke kōkua i ka ʻike ʻana i nā iwi o ka iwi kuamoʻo i ke ʻano o ka butterfly, ʻo ia ka kīnā maʻamau e pili pū me kēia maʻi.

Hoʻomaʻo no ka maʻi Alagille

ʻAʻohe mea e ola ai kēia maʻi, akā, no ka hoʻomaikaʻi ʻana i nā ʻōuli a me ke ʻano o ke ola, ʻōlelo ʻia nā lāʻau e hoʻoponopono i ke kahe o ka bile, e like me Ursodiol a me nā multivitamins me ka huaola A, D, E, K, calcium a me ka zinc i mea e hoʻoponopono ai i nā hemahema o ka meaʻai kū ma muli o ka maʻi.

I nā hihia ʻoi loa, pono paha e hele i kahi ʻoki a i ʻole ke transplantation ʻana o nā meaola e like me ke ake a me ka puʻuwai.