Ke manaʻo nei ʻoe i kekahi manawa e melemele ana kou kino, a i ʻole ke manaʻo nei ʻoe e loaʻa ana ka ʻeha ma ka ʻaoʻao ʻākau o kou ʻōpū? Hiki i kekahi poʻe ke loaʻa i ke kuni a me ka luaʻi pū me kēia mau hōʻailona. ʻO kekahi kumu paha ma hope o kēia, ʻo ia ke kūlana a mākou e kamaʻilio ai i kēia lā i kapa ʻia ʻo 'Caroli's disease.' E nānā kākou i ke ʻano o ia mea, no ke aha e hana ʻia ai, pehea e ʻike ʻia ai, a me ke ʻano o ka lāʻau.
I ka ʻōlelo maʻalahi, he aha ka maʻi Caroli?
E noʻonoʻo, i loko o ko kākou akepaʻa aia nā ʻōmole liʻiliʻi i kapa ʻia ʻo nā ʻōmole bile . Lawe kēia mau ʻōmole i ka wai ʻai i kapa ʻia ʻo ka bile i hana ʻia i loko o ke akepaʻa i ka ʻōpū liʻiliʻi. I kēia manawa, ʻo kēia maʻi Caroli kahi kūlana kahi e lilo ai nā ʻōmole bile i mea nui aʻe ma mua o ka maʻamau, ʻo ia hoʻi, ua hoʻonui ʻia lākou . He maʻi genetic kēia, ʻo ia hoʻi, ua hoʻokumu ʻia e kahi loli i loko o kā mākou mau genes. Ke lilo kēia mau ʻōmole i mea nui aʻe e like me kēia, hoʻomaka ka bile e hōʻiliʻili ma kahi o ke kahe pono ʻana. Ke hōʻiliʻili kēia, hiki ke hana ʻia nā pōhaku ʻōmole bile , hiki ke loaʻa nā maʻi, a i kekahi manawa hiki ke hana ʻia nā cysts.
I ka ʻōlelo maʻalahi, pono e hiki i ka bile ke kahe maʻalahi me ka ʻole o ka paʻa. Eia nō naʻe, i ka wā e nui loa ai nā ʻauwai bile, hiki i ka bile ke hōʻiliʻili maʻalahi. I ka hala ʻana o ka manawa, hiki i kēia bile i hōʻiliʻili ʻia ke hana i nā pōhaku liʻiliʻi.
Inā loaʻa iā ʻoe ka maʻi Caroli, hiki iā ʻoe ke ʻike i nā hōʻailona he nui o kēia ʻano i kou ola a pau. Hoʻomaka pinepine nā hōʻailona ma kahi o 30 mau makahiki , akā hiki ke hana ʻia i kēlā me kēia makahiki. E ʻike paha ʻoe i kou ʻili a me nā keʻokeʻo o kou mau maka e melemele ana (kapa mākou i kēia jaundice ), a hiki paha iā ʻoe ke hoʻomaka e loaʻa nā hōʻailona e like me ke anu a me ka maʻi palū pinepine. ʻOiai hiki i kēia ʻano ke hoʻopilikia i kou olakino a me ke ʻano o ke ola, aia nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i kāu mau hōʻailona.
Aia kekahi ʻano o ka maʻi Caroli?
ʻAe, ʻelua mau ʻano nui o kēia:
- Maʻi Caroli: Hoʻopilikia wale kēia ʻano i kāu mau ʻauwai bile. ʻAʻole ia e hoʻoulu i nā pilikia ʻē aʻe me kou ake.
- ʻO Caroli syndrome: ʻO kēia ke ʻano ʻoi aku ka ʻino. I loko o kēia, lilo ke akepaʻa i ʻeha, a mākou e kapa nei ʻo fibrosis .
Pehea ka maʻamau o kēia maʻi?
He maʻi laha ʻole ka maʻi Caroli. Ua manaʻo ʻia hoʻokahi wale nō i loko o hoʻokahi miliona mau kānaka e loaʻa ana iā ia . No laila, ʻaʻole ia he maʻi e hoʻopilikia i nā poʻe he nui.
No laila, he aha nā hōʻailona o ka maʻi ʻo Caroli?
ʻO ka maʻamau, ʻaʻole nā pōhaku liʻiliʻi e hana ʻia i loko o nā ʻauwai bile e hōʻike i nā hōʻailona nui. Eia nō naʻe, inā ālai kahi pōhaku i kahi ʻauwai bile, hiki iā ia ke mumū. Kāhea mākou i kēia cholangitis . ʻO kēia ka manawa e ʻike ʻia ai nā hōʻailona o ka maʻi ʻo Caroli:
- ʻEha ma ka ʻaoʻao ʻākau o ke kino , pinepine ma ka ʻōpū luna.
- Luaʻi .
- Kuni a me ke anu .
- Melemele o ka ʻili a me nā keʻokeʻo o nā maka (jaundice) .
- ʻŌhūhū .
He mea kākaʻikahi loa, hiki i kēia hōʻiliʻili ʻana o ka bile ke alakaʻi i ka ulu ʻana o kahi ʻano puʻupuʻu kanesa i kapa ʻia ʻo cholangiocarcinoma i loko o nā ʻauwai bile. No laila he mea nui e makaʻala i kēia.
No ke aha i loaʻa ai ka maʻi ʻo Caroli? He aha nā kumu?
He maʻi hoʻoilina ka maʻi Caroli. ʻO ia hoʻi, ua hoʻokumu ʻia e ka loli o nā genes i loko o ko kākou kino. I ka hapa nui o ka manawa, hiki ke loaʻa pinepine ʻia, ʻo ia hoʻi, ʻaʻole ia e hoʻoili ʻia mai kēlā hanauna a i kēia hanauna. Eia nō naʻe, i loko o kekahi mau ʻohana, ua hōʻike ʻia he autosomal dominant , ʻo ia hoʻi, inā loaʻa i kekahi makua, hiki i nā keiki ke loaʻa pū kekahi.
Hoʻokumu ʻia ka maʻi Caroli e kahi hoʻololi genetic i loko o kahi gene i kapa ʻia ʻo PKHD1 . ʻO kēia gene ke kuleana no ka hana ʻana i kahi protein e kōkua i nā ʻauwai bile a me nā puʻupaʻa e ulu i ka wā embryonic, ʻo ia hoʻi, i ka wā e noho ana ke pēpē i loko o ka ʻōpū. Ma muli o ka pili ʻana o ka maʻi Caroli a me ka maʻi Caroli, manaʻoʻiʻo nā kānaka noiʻi ua pili pū ka gene PKHD1 i ka maʻi Caroli. Eia nō naʻe, pono ka noiʻi hou aku e hoʻoholo i kēia.
Pehea e ʻike ai nā kauka i ka maʻi o Caroli?
Ke ʻike ʻoe i ke kauka, e hana mua lākou i kahi hoʻokolokolo kino a nīnau e pili ana i kāu mau hōʻailona. A laila, e kauoha lākou i kekahi mau hoʻokolohua koko, e like me ka Comprehensive Metabolic Panel (CMP), nona nā enzymes ate . Inā he ʻano hoʻokiʻekiʻe i loko o kēia mau enzymes, hiki iā ia ke hōʻike ua ālai ʻia kahi ʻauwai bile.
Ma waho aʻe o nā hoʻokolohua koko, e kauoha paha kāu kauka i kekahi mau hoʻokolohua kiʻi/radiology , ʻo ia hoʻi nā hoʻokolohua e nānā i loko o kou kino. ʻO kēia mau mea:
- ʻO ka ultrasound ʻōpū
- ʻO ka scan CT (scan tomography i helu ʻia)
- ʻO kahi scan MRI (scan kiʻi resonance magnetic)
Eia kekahi, hiki i kāu kauka ke noi iā ʻoe e hana i kahi hoʻāʻo ERCP (endoscopic retrograde cholangiopancreatography) . Pili kēia i ke komo ʻana i kahi ʻōmole liʻiliʻi a palupalu (i kapa ʻia he endoscope ) ma kou waha a i lalo i kou ʻōpū i loko o kou mau ʻāpana bile. Me kēia ERCP, hiki i kāu kauka ke nānā i kou mau ʻāpana bile a me ke akepaʻa e ʻike inā he mau puʻupuʻu a i ʻole he ʻeha.
ʻO kekahi mea ʻē aʻe, hiki i kāu kauka ke noi iā ʻoe e hana i kahi ʻano MRI kūikawā i kapa ʻia ʻo MRCP (magnetic resonance cholangiopancreatography) . Hiki i kēia ke ana i ka laulā o kāu mau ʻauwai bile. Hiki iā ia ke nānā i nā pōhaku gall.
He aha nā lāʻau lapaʻau no kēia? Hiki ke ho'ōla ʻia?
ʻO kaʻoiaʻiʻo, ʻo ka maʻi o CaroliʻAʻohe lāʻau lapaʻau i kēia manawa. Eia nō naʻe, aia nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona a pale i nā pilikia. Pono kēia iā ʻoe e hana pū me kahi hui o nā kauka loea i nā ʻano hana like ʻole. ʻO kēia mau mea:
- He kanaka loea i ka hoʻoilina
- Kauka ʻōpū
- Kauka lapaʻau akepaʻa/Hepatologist
- ʻO kahi urologist ( ʻoiai ua pili pū nā puʻupaʻa)
- He kauka loea i nā maʻi puʻupaʻa (Nephrologist)
- Radiologist ( no nā mea e like me nā scans)
- Kauka lapaʻau hoʻololi (inā pono)
ʻO ka lāʻau no ka maʻi ʻo Caroli e pili ana i kahi o ke akepaʻa o ka ʻauwai bile i hoʻonui ʻia.
- I kekahi manawa hana ʻoki kino: Inā aia ka ʻāpana bile i hoʻonui ʻia ma ka ʻaoʻao hema a ʻākau paha o ke akepaʻa, hiki i kāu kauka ke wehe wale i kēlā ʻāpana o ke akepaʻa. Ua kapa ʻia kēia he hemi-hepatectomy . ʻO ka poʻe i wehe ʻia kekahi ʻāpana o ke akepaʻa ma kēia ʻano, ʻaʻole lākou e loaʻa hou i nā hōʻailona.
- Lāʻau lapaʻau: Eia nō naʻe, inā ua pālahalaha nā ʻauwai bile i hoʻonui ʻia ma loko o ke akepaʻa, hiki i kāu kauka ke hāʻawi iā ʻoe i nā lāʻau antibiotic e pale aku i kahi maʻi i kapa ʻia ʻo cholangitis . Eia kekahi, hiki i kahi lāʻau lapaʻau i kapa ʻia ʻo ursodeoxycholic acid ke kōkua i kekahi o nā pilikia i hoʻokumu ʻia e ka maʻi ʻo Caroli.
- Nā hana ʻoki ʻē aʻe: I kekahi manawa, hiki i kāu kauka ke ʻōlelo aku i kahi bypass biliary kūloko e kōkua i ka hoʻokahe ʻana i ka bile mai ke ake.
- Hoʻoili akepaʻa: I nā hihia koʻikoʻi loa, ʻo ka hoʻoili akepaʻa ke koho lapaʻau maikaʻi loa.
ʻEhia ka lōʻihi e hiki ai iā ʻoe ke ola me ka maʻi Caroli?
ʻAʻole ka maʻi Caroli ponoʻī e hoʻoholo pololei i kou ola. Hoʻopilikia ʻia ka manaʻolana ola e nā pilikia e hiki ke hana. Eia kēia mau mea:
- Cholangitis: ʻO kēia cholangitis nui, hiki ke hana ʻia e nā gallstones i loko o nā ʻauwai bile, pono ke mālama ʻia i ka lāʻau lapaʻau pilikia a hiki ke make i loko o nā hola a me nā lā.
- ʻO ke kanesa o ke aʻa bile (cholangiocarcinoma): Hiki ke ulu kēia maʻi kanesa ma muli o ka mumū lōʻihi a me ka paʻa ʻana o nā aʻa bile. He mea nui ka ʻike mua ʻana a me ka mālama ʻana no nā hopena maikaʻi.
- Ka hemahema o ke akepaʻa:Inā hoʻomau ka mumū o nā ʻauwai bile, hiki iā ia ke alakaʻi i nā maʻi koʻikoʻi e like me ka hāʻule ʻana o ke ake.
ʻO ka mea nui loa, ʻo ia ka hoʻokele ʻana. Inā mālama pono ʻia ka cholangitis a nānā mau ʻia nā pilikia e ke kauka, hiki i ka poʻe me ka maʻi Caroli ke ola piha.
Aia kekahi ala e pale aku ai i kēia maʻi Caroli?
He maʻi hoʻoilina ka maʻi Caroli, no laila ʻaʻole hiki ke pale ʻia no ka mea he mea ia e hele mai me ko kākou mau genes.
Āhea wau e ʻike ai i ke kauka?
Inā loaʻa iā ʻoe nā hōʻailona o ka maʻi ʻo Caroli - e like me ka ʻeha ma ka ʻaoʻao ʻākau o kou ʻōpū, ka luaʻi ʻana, ke kuni, a me ka jaundice - pono ʻoe e ʻike i ke kauka. Mai hoʻokaʻulua.
He aha nā nīnau e pono iaʻu e nīnau aku i ke kauka?
He manaʻo maikaʻi e nīnau i kēia mau nīnau ke ʻike ʻoe i ke kauka:
- Loaʻa iaʻu ka maʻi Caroli a i ʻole ka maʻi Caroli?
- Pehea ke koʻikoʻi o koʻu kūlana ma muli o nā hopena o kaʻu mau scan?
- He aha ka hoʻolālā lapaʻau maikaʻi loa noʻu?
- Aia kekahi mau lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i koʻu mau hōʻailona a pale aku paha i nā pilikia?
- Hiki iaʻu ke hana ʻoki kino? Inā pēlā, he aha ke ʻano o ke ʻoki kino e pono ai iaʻu?
- He aha nā pilikia a me nā pono o ke ʻoki kino?
- He aha nā hōʻailona o nā pilikia e pono iaʻu e makaʻala?
He aha ka ʻokoʻa ma waena o ka maʻi akepaʻa Polycystic (PCLD) a me ka maʻi Caroli?
Hoʻopilikia ka maʻi akepaʻa Polycystic (PCLD) a me ka maʻi Caroli i nā ʻauwai bile i loko o ke akepaʻa. Akā he ʻokoʻa nui ma waena o nā mea ʻelua. I ka PCLD, nui nā cysts i piha i ka wai (cysts) e hana ʻia ma loko o ke akepaʻa. Ua like kēia me nā ʻeke ʻano ʻē. ʻOiai hiki ke hana ʻia nā cysts i ka maʻi Caroli, pili nui ia i ka hoʻonui ʻana o nā ʻauwai bile kūikawā i loko o ke akepaʻa.
No laila he aha ka pilina ma waena o ka maʻi ʻo Caroli a me nā cysts choledochal?
Hoʻopilikia ka maʻi Caroli i nā ʻauwai bile intrahepatic, ʻo ia hoʻi nā ʻauwai bile i loko o ke akepaʻa . ʻO nā cysts e hana ʻia ana i loko o kēia mau ʻauwai bile intrahepatic he ʻano cyst choledochal . No laila, inā he nui kāu o kēia mau cysts, ua kapa ʻia kēlā he maʻi Caroli. I ka ʻōlelo maʻalahi, ʻo ka maʻi Caroli he ʻano cyst choledochal.
ʻO ka mea hope loa, nā mea nui e pono ai ʻoe e hoʻomanaʻo
Hiki ke hopohopo iki ka lohe ʻana i ka maʻi Caroli. Eia nō naʻe, me ka holomua o ka ʻepekema lapaʻau, aia nā ala kūpono e hoʻokele ai i kēia kūlana. Ma ka loaʻa ʻana o nā nānā maʻamau, hiki iā ʻoe a me kāu kauka ke nānā pono i kou kūlana. Nui ka poʻe me ka maʻi Caroli e loaʻa ka hōʻoluʻolu mai ko lākou mau hōʻailona me ka lāʻau lapaʻau , aʻO ka minimally invasive, ʻo ia hoʻi ʻaʻohe ʻoki nui, he koho pinepine nā kaʻina hana lapaʻau.
E hoʻomanaʻo, ʻo ke kumu nui ma ʻaneʻi ka mālama ʻana iā ʻoe iho i ke olakino a me ka hana no ka lōʻihi e hiki ai. No laila, mai hopohopo, e hahai pono i nā ʻōlelo a kāu kauka. Mai makaʻu e nīnau inā he mau nīnau kāu.
ʻO ka maʻi Caroli, ke akepaʻa, nā ʻauwai bile, nā maʻi genetic, jaundice, nā pōhaku Gall
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