Kua rahi ake ō awaawa waikura? Me kōrero tātou mō te Mate Caroli!

Kei te ahua kōwhai tō tinana i ētahi wā, kei te ahua mamae rānei te taha matau o tō puku? Tērā pea ka pāngia ētahi tāngata e te kirika me te ruaki me ēnei tohu. Ko tētahi take pea mō ēnei ko te mate ka kōrerohia e tātou i tēnei rā e kiia nei ko te 'mate a Caroli.' Me titiro tātou he aha te mate, he aha te take e puta ai, me pēhea te tautuhi i tōna mate, me te aha te rongoā.

He aha te mate Caroli?

Whakaarohia, kei roto i tō tātou ate he ngongo iti e kiia nei ko ngā awa au . Ka haria e ēnei ngongo te wai nakunaku e kiia nei ko te au i hangaia i roto i te ate ki te whekau iti. Nā, ko tēnei mate Caroli he āhuatanga e nui ake ai ngā awa au i te mea noa, arā, ka whānui haere . He mate ira tēnei, arā, nā te huringa o ō tātou ira i puta mai ai. Ina nui ake ēnei ngongo pēnei, ka tīmata te kohikohi o te au, kaua e rere tika. Ina kohikohia tēnei, ka taea te hanga kōhatu awa au , ka puta he mate, ā, i ētahi wā ka puta he pūwero.

He whakarāpopototanga noa iho, me ngāwari te rere o te au o te ate, me te kore e mau. Heoi, ina nui rawa ngā awa o te au o te ate, ka ngāwari te kohikohi o te au o te ate. I roto i te wā, ka taea e tēnei au o te au o te au te hanga i ngā kōhatu ririki.

Mena kei a koe te mate Caroli, ka pāngia pea koe e ngā tohu maha o tēnei mate puta noa i tō oranga. Ka tīmata ngā tohu i te 30 tau te pakeke , engari ka pā pea i ngā tau katoa. Ka kite pea koe i tō kiri me te mā o ō kanohi e huri kōwhai ana (e kiia ana tēnei he jaundice ), ā, ka tīmata pea koe ki te pāngia e ngā tohu pēnei i te makariri me te rewharewha. Ahakoa ka pā tēnei mate ki tō hauora me tō kounga o te oranga, kei reira ngā maimoatanga hei āwhina i te whakahaere i ō tohu.

He momo mate a Caroli kei reira?

Ae, e rua ngā momo matua o tēnei:

• Te mate Caroli: Ka pā noa tēnei momo ki ō awaawa aukume. Kāore e puta he raruraru kē atu ki tō ate.
• Te mate Caroli: Koinei te momo kino rawa atu. I roto i tēnei, ka pakaru te ate, ka kiia e tātou ko te fibrosis .

He pēhea te noa o tēnei mate?

He tino onge te mate a Caroli. E kiia ana kotahi anake i roto i te miriona tāngata ka pāngia e tēnei mate . Nō reira, ehara i te mate e pāngia ana e te tini tāngata.

Nā, he aha ngā tohu o te mate a Caroli?

I te nuinga o te wā, kāore he tohu nui e puta mai i ngā kōhatu iti e puta mai ana i roto i ngā awaawa o te ate. Heoi, ki te āraihia e te kōhatu te awaawa o te ate, ka mumura pea. Ka kiia tēnei ko te cholangitis . Koinei te wā e puta ai ngā tohu o te mate a Caroli:

• Te mamae i te taha matau o tō tinana , he maha ngā wā i te puku o runga.
• Te ruaki .
• Te kirika me te makariri .
• Te kōwhai o te kiri me te mā o ngā kanohi (jaundice) .
• Te mākūkū .

He tino onge te kohikohinga o te au ka arahi ki te whanaketanga o tētahi momo pukupuku matepukupuku e kiia nei ko te cholangiocarcinoma i roto i ngā awa au. Koia te mea nui kia tūpato ki tēnei.

He aha te take e puta ai te mate a Caroli? He aha ngā take?

He mate ira te mate Caroli. Ko te tikanga, nā te huringa o ngā ira o roto i tō tātou tinana i puta ai. I te nuinga o te wā, ka puta noa i te wā, arā, kāore e tukuna iho i tētahi whakatipuranga ki tētahi whakatipuranga. Heoi, i roto i ētahi whānau, kua pūrongohia he autosomal dominant , arā, mēnā kei tētahi o ngā mātua tēnei mate, ka taea hoki e ngā tamariki te pāngia.

Ko te mate Caroli i puta mai i te whakarerekētanga ira i roto i te ira e kiia nei ko PKHD1 . Ko tēnei ira te kawenga mō te whakaputa i te pūmua e āwhina ana i ngā awaawa ate me ngā whatukuhu ki te whanake i te wā o te kukune, arā, i te wā kei roto te pēpi i te kōpū. Nā te mea he hononga te mate Caroli me te mate Caroli, e whakapono ana ngā kairangahau kei roto hoki te ira PKHD1 i te mate Caroli. Heoi, me nui ake ngā rangahau hei whakatau i tēnei.

Me pēhea te tautuhi a ngā tākuta i te mate a Caroli?

Ina kite koe i te tākuta, ka tirotirohia e rātou tō tinana , ā, ka pātai atu mō ō tohumate. Kātahi ka tonoa ētahi whakamātautau toto, pērā i te Comprehensive Metabolic Panel (CMP), kei roto ko ngā whākōkī ate . Mena he āhua pikinga o ēnei whākōkī, he tohu pea tēnei kua āraia te awa waikura.

Haunga ngā whakamātautau toto, tera pea ka tonoa e tō tākuta ētahi whakamātautau whakaahua/ira-irirangi , arā, ngā whakamātautau e titiro ana ki roto i tō tinana. Kei roto i ēnei ko:

• Te tirotiro i te puku
• He matawai CT (matawai tomography rorohiko)
• He matawai MRI (matawai whakaahua aukume)

Hei tāpiritanga, ka tono pea tō tākuta kia whakamātauria koe ki te ERCP (endoscopic retrograde cholangiopancreatography) . Ko te tikanga o tēnei ko te whakauru i tētahi ngongo iti, ngāwari (e kiia nei ko te endoscope ) mā roto i tō waha, ka heke iho i tō puku ki roto i ō awaawa au. Mā tēnei ERCP, ka taea e tō tākuta te tirotiro i ō awaawa au me te ate kia kite ai mēnā he pukupuku, he koha rānei.

Ko tētahi atu mea, ka tono pea tō tākuta kia mahia e koe tētahi momo MRI motuhake e kiia nei ko te MRCP (magnetic resonance cholangiopancreatography) . Ka taea e tēnei te ine i te whānui o ō awa au. Ka taea hoki te tirotiro mō ngā kōhatu au.

He aha ngā rongoā mō tēnei? Ka taea te whakaora?

Ki te pono, ko te mate a CaroliKāore anō kia kitea he rongoā. Heoi anō, kei reira ngā maimoatanga hei āwhina i te whakahaere i ngā tohu me te ārai i ngā raruraru. Mā tēnei ka hiahia koe ki te mahi tahi me tētahi rōpū tākuta he tohunga ki ngā momo mara. Kei roto i ēnei ko:

• Tohunga ira
• Tohunga kai
• Tohunga ate/Tohunga mate pukupuku
• He tākuta mimi ( nā te mea ka pāngia hoki ngā whatukuhu)
• He tākuta e tohunga ana ki ngā mate whatukuhu (Ngā Tohunga Mate Pūkahukahu)
• Kaiwhakaora irahiko ( mō ngā mea pēnei i te matawai)
• Taote pokanga whakato (mēnā e tika ana)

Ko te maimoatanga mō te mate a Caroli e whakawhirinaki ana ki te wāhi o te ate e whānui ana te awaawa aukume.

• I ētahi wā ka pokanga: Mena kei te taha maui, kei te taha matau rānei o te ate te awaawa nui o te ate, ka tangohia pea e tō tākuta taua wāhanga anake o te ate. Ka kiia tēnei he hemi-hepatectomy . Ko te hunga kua tangohia tētahi wāhanga o te ate i tēnei ara, kāore e kitea anō ngā tohu.
• Rongoā: Heoi, mēnā kua horapa ngā awaawa aukume kua whānui puta noa i te ate, ka hoatu pea e tō tākuta he rongoā paturopi hei ārai i te mate e kiia nei ko te cholangitis . Waihoki, ka taea e te rongoā e kiia nei ko te waikawa ursodeoxycholic te āwhina i ētahi o ngā raruraru i puta mai i te mate a Caroli.
• Ētahi atu pokanga: I ētahi wā, ka tūtohu pea tō tākuta i tētahi pokanga ā-roto hei āwhina i te tango i te au mai i te ate.
• Te whakawhitinga ate: I ngā wā tino kino, ko te whakawhitinga ate te kōwhiringa maimoatanga pai rawa atu.

Kia pēhea te roa ka taea e koe te ora me te mate Caroli?

Kāore te mate Caroli e whakatau tika i tō roanga o te ora. Ka pāngia te roanga o te ora e ngā raruraru ka pā mai. Ko ēnei:

• Cholangitis: Ko tēnei cholangitis whakapeka, ka puta mai i ngā kohatu keri i roto i ngā awa waikura, me whiwhi maimoatanga hauora urupare, ā, ka mate pea i roto i ngā hāora ki ngā rā.
• Matepukupuku o te awa au (cholangiocarcinoma): Ka puta pea tēnei matepukupuku nā te mumura roa me te aukati i ngā awa au. He mea nui te kitenga wawe me te maimoatanga mō ngā putanga pai.
• Te ngoikore o te ate:Ki te haere tonu te mumura o ngā awaawa au, ka taea e ia te arahi ki ngā mate kino pērā i te ngoikore o te ate.

Ko te mea nui ko te whakahaere. Mēnā ka tika te rongoā mō te cholangitis, ā, ka tirohia auautia ngā raruraru e te tākuta, ka taea e te hunga whai mate Caroli te ora tonu.

He ara hei ārai i tēnei mate Caroli?

He mate ira te mate Caroli, nō reira kāore e taea te ārai nā te mea he mea nō roto mai i ō tātou ira.

Āhea ahau me toro atu ki te tākuta?

Ki te puta mai ngā tohu o te mate a Caroli – pērā i te mamae i te taha matau o tō puku, te ruaki, te kirika, me te jaundice – me toro atu koe ki te tākuta. Kaua e whakaroa.

He aha ngā pātai me pātai atu e au ki te tākuta?

He mea pai kia pātaihia ēnei pātai ina kite koe i tētahi tākuta:

• Kei a au te mate Caroli, te mate Caroli rānei?
• E ai ki ngā hua o aku matawai, he pēhea te kino o tōku āhua?
• He aha te mahere maimoatanga pai rawa atu mōku?
• He rongoā kei reira hei āwhina i te whakahaere i aku tohumate, hei ārai rānei i ngā raruraru?
• Ka taea e au te pokanga? Mena koinā, he aha te momo pokanga me mahi?
• He aha ngā mōrearea me ngā painga o te pokanga?
• He aha ngā tohu o ngā raruraru me mōhio ahau?

He aha te rerekētanga i waenga i te Mate Ate Polycystic (PCLD) me te mate a Caroli?

Ka pāngia ngā awaawa o te ate e te mate ate polycystic (PCLD) me te mate Caroli. Engari he rerekētanga nui i waenganui i ngā mea e rua. I roto i te PCLD, he maha ngā pūwero wai (ngā pūwero) ka puta puta noa i te ate. He rite ēnei ki ngā pēke rerekē. Ahakoa ka taea hoki te puta ngā pūwero i roto i te mate Caroli, ko te nuinga o te wā ko te whakawhānui i ngā awaawa motuhake i roto i te ate.

Nā, he aha te whanaungatanga i waenga i te mate a Caroli me ngā pukupuku choledochal?

Ka pāngia ngā awaawa aukume o roto i te ate e te mate Caroli, arā, ngā awaawa aukume i roto i te ate . Ko ngā pūwero e puta mai ana i roto i ēnei awaawa aukume o roto i te ate he momo pūwero choledochal . Nō reira, ki te mea he maha āu o ēnei pūwero, ka kiia tēnā he mate Caroli. He māmā noa iho, ko te mate Caroli he momo pūwero choledochal.

Hei whakamutunga, ko ngā mea tino nui me maumahara koe

He ahua whakamataku te rongo mō te mate Caroli. Heoi, nā te whanaketanga o te pūtaiao hauora, kua kitea ngā huarahi whai hua hei whakahaere i tēnei mate. Mā te tirotiro auau, ka taea e koe me tō tākuta te aroturuki i tō mate. He maha ngā tāngata e pāngia ana e te mate Caroli ka kitea he whakaoranga mai i ō rātou tohu mā te rongoā , ā,He iti noa te whakaekenga, arā, kāore he tapahi nui, he maha ngā wā ka taea te whakamahi i ngā tikanga hauora.

Kia maumahara, ko te arotahi i konei ko te pupuri i a koe kia ora, kia kaha hoki mō te roa e taea ai. Nō reira, kaua e mataku, me whai noa i ngā tohutohu a tō tākuta. Kaua e wehi ki te pātai mēnā he pātai āu.

Te Mate Caroli , te Ate, ngā Awa Au, ngā Mate Ira, te Waikura, ngā Kōhatu Puku