Ke pilikia nei ʻoe me kou mau ʻauwai bile? E aʻo kākou e pili ana i ka Primary Sclerosing Cholangitis (PSC)!

I kekahi manawa luhi mākou me ke kumu ʻole, loaʻa iā mākou kahi ʻeha iki ma ka ʻaoʻao ʻākau o luna o ko mākou ʻōpū, a i ʻole manaʻo paha mākou e kahakaha ana iā mākou iho. ʻOiai ʻaʻole mākou e nānā nui i kēia mau mea i ko mākou ola o kēlā me kēia lā, i kekahi manawa hiki i kēia ke lilo i mau hōʻailona o kahi maʻi koʻikoʻi i loko o ko mākou kino. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi paʻakikī iki, akā he mea nui loa ia no nā mea a pau e makaʻala. ʻO ia ka Primary Sclerosing Cholangitis , a i ʻole PSC no ka pōkole.

He aha ka cholangitis sclerosing mua (PSC)?

I ka ʻōlelo maʻalahi, ʻo ka PSC kahi maʻi e kū mai ana i loko o nā ʻauwai bile i loko o ko mākou kino. E like me kāu e hoʻomanaʻo nei, hana ʻia ka bile i loko o ke akepaʻa. Kōkua kēia bile i ka ʻai ʻana. No laila, aia kahi ʻōnaehana o nā ʻauwai liʻiliʻi e lawe ana i kēia bile mai ke akepaʻa a i nā ʻōpū. Kāhea mākou i kēlā mau ʻauwai he mau ʻauwai bile.

I loko o ka PSC, aia ka mumū mau loa , a i ʻole ka pehu ʻana, i loko o kēia mau ʻauwai bile. Ua kapa pū nā kauka i kēia he cholangitis . Ke hoʻomau kēia mumū no ka manawa lōʻihi, hoʻomaka ka ʻeha e hana i loko o nā ʻauwai bile. ʻO kēia ʻeha ke kumu o ka paʻakikī a me ka haiki o nā ʻauwai bile. Ua kapa ʻia kēia he sclerosis .

E noʻonoʻo i kahi paipu wai i paʻa i ka lepo a haiki, e paʻakikī ai ke kahe ʻana o ka wai, ʻeā? Pēlā nō, ke haiki nā ʻauwai bile, ua kaupalena ʻia ke kahe ʻana o ka bile. Ua kapa ʻia kēia he stricture biliary . No laila, ke hiki ʻole i ka bile ke kahe pono, hoʻomaka ia e hoʻi i luna i loko o ke ake. ʻO kēia hōʻiliʻili ʻana o ka bile i loko o ke ake (cholestasis) e hōʻino mālie i ke ake.

He koʻikoʻi anei kēia kūlana PSC?

ʻAe, he maʻi koʻikoʻi maoli ka PSC. ʻAʻole paha ʻoe e loaʻa i kekahi mau hōʻailona i nā pae mua. Akā he maʻi holomua ia. Ke ālai ʻia ka bile i loko o nā ducts bile (cholestasis), hiki i kekahi o nā mea ʻawahia i loko o ka bile ke hōʻiliʻili i loko o ke koko. A laila hoʻomaka ʻoe e ʻoluʻolu ʻole. Eia kekahi, ke kumu o kēia i ka hōʻino mau ʻana i ke akepaʻa.

ʻO ka maʻamau, hiki i kēia hōʻino ke alakaʻi i ka hāʻule piha ʻana o ke akepaʻa ma hope o kahi o 10 a 15 mau makahiki. E like me kou ʻike, ʻaʻole hiki iā mākou ke ola me ka ʻole o ke akepaʻa. ʻOiai hiki i nā lāʻau lapaʻau o kēia manawa ke kāohi i nā hōʻailona, ​​ʻaʻohe lāʻau lapaʻau no ka PSC. ʻO ka hopena wale nō ka hoʻoili ʻana i ke akepaʻa .

He aha ka ʻokoʻa ma waena o ke cholangitis sclerosing mua a me ke lua?

ʻO ke ʻano o ka huaʻōlelo "kumu mua" ma ʻaneʻi, ʻo ia hoʻi, ʻo ka maʻi ke kumu nui o ka mumū a me ka ʻeha o nā ʻauwai bile. ʻO ia hoʻi, ua hoʻokumu ʻia ke kūlana e kēia maʻi wale nō, me ke kumu ʻole.

Akā,I loko o ke kūlana i kapa ʻia ʻo secondary sclerosing cholangitis, ʻo ka mumū (cholangitis) a me ka ʻeha (sclerosis) o nā ʻauwai bile e kū mai ana ma muli o kekahi kumu ʻē aʻe. ʻO ia hoʻi, ua hoʻokumu ʻia ke kūlana e kekahi maʻi a hanana paha. Eia kekahi mau laʻana:

• Ka hōʻeha ʻana i nā ʻāpana bile (no ka laʻana, i ka wā o ke ʻoki ʻana).
• Nā maʻi i loko o nā ʻauwai bile.
• Lāʻau lapaʻau anti-cancer (Chemotherapy).
• Nā pōhaku gall e ālai ana i nā ʻauwai bile.
• Ka hoʻi hou ʻana o ka pancreatitis.

He aha ka ʻokoʻa ma waena o ka cirrhosis biliary mua (PBC) a me ka PSC?

ʻO ka maʻi, i kapa mua ʻia ʻo Primary Biliary Cirrhosis, i kēia manawa ua kapa ʻia ʻo Primary Biliary Cholangitis (PBC) .

ʻO PBC a me PSC nā maʻi holomua e hoʻopilikia ana i ke akepaʻa, e hoʻopilikia ana i nā ʻauwai bile. Ua like loa nā hōʻailona a me nā hopena o nā mea ʻelua. Hoʻonāwaliwali mālie nā maʻi ʻelua i nā ʻauwai bile. Ke hōʻino ʻia, ʻeha, a haiki nā ʻauwai bile, ua ālai ʻia ke kahe o ka bile. A laila hoʻi hou ka bile i loko o ke akepaʻa, e hōʻino hou ana i ke akepaʻa. Hiki i kēia ke alakaʻi i ka ʻeha o ke akepaʻa a ma hope o ka cirrhosis o ke akepaʻa .

Akā aia kekahi mau ʻokoʻa nui ma waena o kēia mau maʻi ʻelua:

• Hoʻopilikia wale ka PBC i nā ʻauwai bile i loko o ke akepaʻa (intrahepatic). Hiki i ka PSC ke hoʻopilikia i nā ʻauwai bile i loko o ke akepaʻa a me nā ʻauwai bile ma waho o ke akepaʻa (extrahepatic).
• ʻOi aku ka maʻamau o ka PBC i nā wahine (lakio 10: 1), ʻoiai ʻoi aku ka maʻamau o ka PSC i nā kāne (lakio 2: 1).
• Ma kahi o 80% o nā maʻi PSC i loaʻa i kahi maʻi ʻōpū mumū (IBD) mua, ʻoi aku hoʻi ka ulcerative colitis . Eia nō naʻe, ʻaʻole kēia he pilina koʻikoʻi me PBC.
• Aia kahi pilikia nui o ka maʻi ʻaʻai bile duct me PSC. ʻAʻohe pilikia kiʻekiʻe e like me PBC.
• Hoʻohana ʻia ka Ursodeoxycholic acid (UDCA) e mālama i ka PBC, akā ʻaʻohe lāʻau lapaʻau kūikawā no ka PSC i kēia manawa.

Pehea ka maʻamau o kēia ʻano i kapa ʻia ʻo PSC? ʻO wai ka mea maʻamau e loaʻa ai?

He maʻi laha ʻole ka PSC. Ma ka honua holoʻokoʻa, ua manaʻo ʻia he hoʻokahi wale nō i loko o ka ʻumi tausani kānaka i loaʻa i kēia maʻi.

ʻOi aku ka pinepine o kēia maʻi i nā kāne ma mua o nā wahine. ʻIke pinepine ʻia ka maʻi ma kahi o 40 mau makahiki. Ma kahi o 80% o ka poʻe me PSC e loaʻa pū ana iā IBD (Inflammatory Bowel Disease) , ʻoiai ʻo Ulcerative Colitis . Inā loaʻa i kekahi o ka ʻohana kēia maʻi, aia kekahi poʻe i ka pilikia o ka loaʻa ʻana iā ia.

He aha ke kumu o ka PSC?

ʻAʻole ʻike ʻia ke kumu kikoʻī o ka PSC, akā ua manaʻo ʻia ua hana ʻia e ka hui pū ʻana o nā kumu. ʻO kēia mau mea:

• Nā ʻano hoʻoilina
• Nā kumu kaiapuni
• Nā pūnaewele pale

Manaʻo nā kauka he ʻano maʻi autoimmune paha ka PSC. ʻO ke ʻano kēia, ʻo ka ʻōnaehana pale ponoʻī o ko mākou kino, nā koa i manaʻo ʻia e pale iā mākou mai ka maʻi, e hoʻomaka hewa ana e hoʻouka i kā mākou mau hunaola olakino ponoʻī. ʻO ka mumū kekahi o nā mea kaua o kā mākou ʻōnaehana pale. He manawa pōkole wale nō ia, e hana ana i kahi mea hoʻouka kaua. Akā inā lilo ka mumū i mea maʻi mau, he hōʻailona ia o ka maʻi.

Ua ʻike pū nā kauka he ʻoi aku ka nui o ka poʻe me PSC e hoʻomohala i nā maʻi autoimmune ʻē aʻe. Eia kekahi laʻana:

• Nā maʻi ʻōpū mumū
• Maʻi Celiac
• Maʻi thyroid
• ʻAno 1 diabetes
• ʻO ka maʻi hepatitis autoimmune
• ʻO ka pancreatitis autoimmune

Ua manaʻo ʻia e hiki ke hana ʻia kēia ʻano pane autoimmune ke hōʻike ʻia nā kānaka me kekahi mau ʻano genetic i kekahi mau mea pili i ke kaiapuni (e laʻa, ka ʻike ʻia ʻana i nā kemika ʻawahia).

He aha nā hōʻailona o ka PSC?

I ka holomua ʻana o ka PSC, loli nā hōʻailona. Ma kahi o 50% o ka poʻe ʻaʻohe o lākou hōʻailona ke ʻike ʻia ka maʻi. ʻIke pinepine ʻia ka PSC ma ke ʻano ulia pōpilikia, i ka wā o nā hoʻokolohua no kekahi maʻi ʻē aʻe. ʻAʻole maopopo loa nā hōʻailona mua. ʻO lākou:

• Luhi
• ʻEha ʻōpū o ka ʻaoʻao ʻākau o luna
• ʻIli ʻāʻī (pruritus)

ʻO nā hōʻailona e hiki ke kū mai i ka wā e holomua ai ka maʻi:

• ʻŌpū pehu
• Ake nui
• ʻO ka spleen i hoʻonui ʻia
• Jaundice - ʻO ke ʻano kēia o ka melemele ʻana o nā keʻokeʻo o nā maka a me ka ʻili.
• kuni
• Ka pohō kaumaha i manaʻo ʻole ʻia

He aha nā pilikia o ka PSC koʻikoʻi?

He maʻi lohi ka cholangitis sclerosing mua, akā i ka wā e lilo ai kou mau ʻauwai bile a me ke akepaʻa i ʻeha a hōʻino ʻia kā lākou hana, hiki ke loaʻa nā pilikia like ʻole.

Nā maʻi metabolic

Inā paʻa loa kou mau ala bile, ʻaʻole hiki iā lākou ke hoʻopuka pono i ka bile i loko o kou ʻōpū liʻiliʻi. He mea nui ka bile no ka ʻeli ʻana i ka meaʻai, ʻoiai ka momona, a no ka omo ʻana i nā huaora momona-soluble (A, D, E, a me K). Me ka ʻole o ka bile, ʻaʻole hiki i kāu ʻōnaehana ʻai ke hana pono i ka momona, a ʻaʻole hiki iā ia ke omo i kēia mau huaora koʻikoʻi mai ka meaʻai. Hiki i kēia ke alakaʻi i nā pilikia hou aʻe:

• Nā ʻōpala momona a me ka maʻi pākī
• ʻO ka malabsorption a me ka ʻai ʻole
• ʻO ka maʻalahi o ka ʻeha a me ke kahe ʻana o ke koko (ma muli o ka nele o ka huaora K)
• Ka nāwaliwali o nā iwi - Osteomalacia a me ka osteoporosis (ma muli o ka nele o ka wikamina D)
• ʻIke ʻole i ka pō (ma muli o ka nele o ka huaora A)

Ke koko kiʻekiʻe o ka puka

I ka holomua ʻana o ka cirrhosis, emi iho ke kahe o ke koko ma o ke akepaʻa. Hoʻonui kēia i ke kaomi i loko o ke aʻa portal, kahi e holo ana ma kāu ʻōnaehana hoʻoheheʻe. Hiki i kēia kaomi ke hoʻonui a pohā i nā aʻa ʻē aʻe i loko o kou esophagus a me ka ʻōpū. Hiki i kēia ke alakaʻi i ke kahe koko i loko .

Nā maʻi

Hiki i nā ʻauwai bile i paʻa ke lilo i ka maʻi, e hana ai i nā maʻi e like me ke kuni, ka ʻeha o ka ʻōpū, a me ka lāʻau make koko (sepsis).

Ka maʻi ʻaʻai

I ka lilo ʻana o ka PSC i mea koʻikoʻi, piʻi ka pilikia o ka hoʻomohala ʻana i kekahi mau ʻano maʻi kanesa:

• ʻO ke kanesa o ka ʻauwai bile (cholangiocarcinoma) - Ka pilikia ma waena o 5% a me 20%.
• Ka maʻi ʻaʻai ʻōpū
• Ka maʻi ʻaʻai o ke akepaʻa (hepatoma)
• ʻO ke kanesa colorectal - inā loaʻa iā ʻoe ʻo IBD.

Pehea e ʻike ʻia ai ka PSC?

ʻO ka hapa pinepine, ʻike ʻia ka maʻi ma ka manawa kūpono, i ka wā e hoʻāʻo ai no kekahi mea ʻē aʻe. Ma kahi o 50% o ka poʻe ʻaʻole paha e loaʻa nā hōʻailona ke ʻike ʻia ka maʻi. Hiki ke ʻike ʻia nā hōʻailona mua o ka maʻi me kahi hoʻāʻo koko a i ʻole kahi hoʻāʻo kiʻi. Hōʻike nā scan o nā ʻauwai bile me PSC i nā hiʻohiʻona kūikawā. Inā hōʻike kahi hoʻāʻo koko i nā pae kiʻekiʻe o kahi enzyme i kapa ʻia ʻo alkaline phosphatase , a inā he mau antibodies e hōʻike ana i kahi pane pale i nā ʻauwai bile, hiki ke lilo i hōʻailona o PSC. ʻO ka hoʻonui ʻia o nā hunaola koko keʻokeʻo he hōʻailona ia o kahi maʻi ate.

No ka hōʻoia ʻana i ka maʻi, hiki i kāu kauka ke kuhikuhi i kekahi mau hoʻokolohua kikoʻī hou aʻe:

• Hoʻāʻo hana ate (LFT)Nānā kēia mau hoʻāʻo koko i nā pae kiʻekiʻe o nā enzymes kikoʻī i loko o ke ake. Hiki i nā pae kiʻekiʻe o ka alkaline phosphatase ke hōʻike i ka PSC.
• ʻO ka Magnetic resonance cholangiopancreatography (MRCP) : He scan MRI (Magnetic Resonance Imaging) kēia. Hiki iā ia ke kiʻi i nā kiʻi kikoʻī o kāu ʻōnaehana biliary (ake, gallbladder, a me nā ʻāpana bile). ʻO kēia ka scan mua e ʻike ai i ka PSC. ʻO kēia no ka mea ʻaʻole ia he invasive a ʻaʻole pili i ka radiation. Eia nō naʻe, i kekahi manawa, inā he mua ka maʻi a ʻaʻole paha i koʻikoʻi loa, ʻaʻole paha e hiki ke ʻike pololei. A laila pono paha kekahi scan ʻē aʻe.

Pehea e mālama ʻia ai ka PSC?

I kēia manawa, ʻaʻohe lāʻau lapaʻau e hiki ke hoʻōki a hoʻohuli paha i ka holomua o ka cholangitis sclerosing mua. Eia nō naʻe, hiki ke mālama pololei ʻia nā hōʻailona a me nā pilikia. No ka laʻana, hiki i kāu kauka ke kuhikuhi i nā mea e like me:

• Nā lāʻau lapaʻau e mālama ai i ka ʻili ʻāʻī (pruritus)
• Nā mea hoʻohui no ka mālama ʻana i nā hemahema o ka wikamina
• Nā lāʻau antibiotic e mālama ai i nā maʻi

E nānā pono ana kāu kauka i kou akepaʻa a me nā ʻauwai bile. I ka holomua ʻana o ka maʻi, hiki iā lākou ke wehe i kahi ʻauwai bile i paʻa i kekahi manawa. Hiki ke hana ʻia kēia ma o kahi hoʻāʻo i kapa ʻia ʻo ERCP (endoscopic retrograde cholangiopancreatography) . He hana kēia e hiki ai iā ʻoe ke ʻike i loko o kou mau ʻauwai bile me ka ʻole o ke ʻoki ʻana.

He ʻano endoscopy ka ERCP. Pili ia i ka nānā ʻana i kou mau kino me ka hoʻohana ʻana i kahi endoscope, kahi ʻōmole lōʻihi a palupalu me kahi kāmela liʻiliʻi a me ke kukui ma luna. Ma hope o kou hoʻomaʻamaʻa ʻia ʻana, e hoʻokomo kāu kauka i kēia ʻōmole i lalo o kou puʻu a i loko o kou ʻōpū. Hiki ke hoʻokomo ʻia nā mea hana liʻiliʻi ma o kēia ʻōmole e mālama i kāu mau ʻāpana bile. Hiki i ke loea ke hoʻohana i kahi balloon e hoʻonui i kāu ʻāpana bile, a i ʻole e hoʻokomo i kahi stent e mālama iā ia. Inā ʻaʻole hiki i ka ERCP ke hiki i ka mea i paʻa ai, hoʻohana ʻia kekahi kaʻina hana i kapa ʻia ʻo 'percutaneous trans-hepatic cholangiography (PTHC)'.

Akā, he hopena manawa pōkole wale nō kēia. Ma loko o kahi manawa o 10 a 20 mau makahiki, e ʻoi aku ka ʻino o ka cholangitis sclerosing mua, e ʻoi aku ka ʻino o ka maʻi akepaʻa, a ma hope e hiki mai ka hāʻule ʻana o ke akepaʻa. E nānā kāu kauka i ka hōʻino ʻia o kou akepaʻa a hoʻoholo i ka wā e noʻonoʻo ai i kahi hoʻoili akepaʻa . Pono nō hoʻi ʻoe e hoʻokō i kekahi mau kūlana e noho ma ka papa inoa kali no kahi hoʻoili akepaʻa.

ʻEhia ka lōʻihi e hiki ai iā ʻoe ke noho me PSC?

Ke ʻike ʻia, ʻokoʻa ka awelika o ke ola ma waena o 10 a me 20 mau makahiki. Hiki i kahi hoʻoili akepaʻa ke hāʻawi i kahi ola hou. Eia nō naʻe, ma 15% a 20% o nā hihia, hiki i ka PSC ke hoʻi hou mai ma hope o ka hoʻoili akepaʻa. Inā hana ʻia kēlā, hiki i ke akepaʻa hou ke hāʻule. I ia mau hihia, ʻo ka awelika o ke ola ma kahi o ʻeiwa mahina.

ʻO kekahi kumu e hiki ke hoʻopilikia i ka manaʻolana o ke ola, ʻo ia ka maʻi kanesa. Inā loaʻa iā ʻoe ka maʻi kanesa ma ke ʻano he pilikia o ka PSC, ʻaʻole paha ʻoe he moho maikaʻi no ka hoʻoili ʻana i ke akepaʻa. I nā hihia i koho pono ʻia, hiki i nā kauka ke mālama mua i ke kanesa me ka radiation a i ʻole chemotherapy, a laila hoʻāʻo i ka hoʻoili ʻana i ke akepaʻa.

Pehea lā wau e mālama ai iaʻu iho inā loaʻa iaʻu kēia maʻi?

ʻO ka hoʻololi ʻana i ke ʻano o ka nohona e hiki ke kōkua i ka hoʻokele ʻana i ka luhi i maʻamau me ka PSC a pale i ka hōʻino hou ʻana o ke akepaʻa. Eia kekahi laʻana:

• E hōʻalo loa i ka waiʻona.
• E ʻai i nā meaʻai i hana ʻia me ka liʻiliʻi a e ʻai i nā meaʻai holoʻokoʻa.
• E kāohi i ke kaumaha.
• Hiamoe maikaʻi.
• E hana i kekahi mau hoʻoikaika kino i kēlā me kēia lā.

He maʻi laha ʻole ka cholangitis sclerosing mua, hiki ke wānana ʻole ʻia, a hiki ke pale ʻia. Holo mālie ia, a pinepine ʻaʻohe hōʻailona i nā pae mua. Akā inā ʻike pinepine ʻoe i kāu kauka a loaʻa nā nānā maʻamau, hiki ke ʻike ʻia ka maʻi ma mua o ka hoʻomaka ʻana e hoʻopilikia i kou ola. ʻO ia ka manawa e hiki ai iā ʻoe ke hana i nā hoʻololi nohona e hiki ke kōkua i ka mālama ʻana i kou akepaʻa no ka lōʻihi. E kuhikuhi ʻia nō hoʻi ʻoe no ka mālama olakino lōʻihi, ʻo ia hoʻi nā nānā mau a me nā hana komo inā pono.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

ʻAe, no laila ke lana nei koʻu manaʻo ua kōkua nā mea a kākou i kamaʻilio ai e pili ana iā PSC iā ʻoe e hoʻomanaʻo i kekahi o nā mea nui loa. He kūlana paʻakikī iki ia, akā he mea nui loa ka ʻike ʻana i ia mea.

• He maʻi ka PSC e hoʻopilikia ana i nā ʻauwai bile a e ʻoi aku ka ʻino i ka hala ʻana o ka manawa. Hiki iā ia ke hōʻino i ke akepaʻa.
• ʻAʻole paha e ʻike ʻia nā hōʻailona i nā wā mua, no laila he mea nui e hana i nā hoʻokolohua lapaʻau maʻamau.
• ʻAʻohe lāʻau lapaʻau no kēia maʻi i kēia manawa, akā aia nā lāʻau lapaʻau e kaohi i nā hōʻailona a hoʻomaikaʻi i ka maikaʻi o ke ola.
• ʻO ka hoʻoili ʻana i ke akepaʻa ke ala nui i nā hihia koʻikoʻi.
• ʻO ka hahai ʻana i kahi nohona olakino e kōkua i ka mālama ʻana i ke akepaʻa.

Inā he mau nīnau hou kāu e pili ana i ka PSC, a inā manaʻo ʻoe he mau hōʻailona kāu, e ʻike pono i ke kauka no ka ʻōlelo aʻo . Mai hopohopo, aia kekahi mau mea hiki iā ʻoe ke hana e hoʻokele ai inā loaʻa koke.

ʻO ka Cholangitis Sclerosing Mua, PSC, ka maʻi ʻauwai Bile, ka maʻi ʻaʻai, ka jaundice, ka ʻeha ʻōpū, ka Cholangitis Sclerosing Mua