He raruraru kei a koe mō ō awa waikura? Me ako tātou mō te Primary Sclerosing Cholangitis (PSC)!

I ētahi wā ka ngenge tātou, kāore he take, ka mamae te taha matau o runga o tō tātou puku, ka rite rānei ki te mea kei te waru noa tātou i a tātou anō. Ahakoa kāore tātou e aro nui ana ki ēnei mea i roto i ō tātou oranga o ia rā, i ētahi wā he tohu ēnei mō tētahi mate kino ake i roto i tō tātou tinana. I tēnei rā ka kōrero tātou mō tētahi mate he uaua, engari he mea tino nui kia mōhio te katoa. Ko te Primary Sclerosing Cholangitis tēnā, ko te PSC rānei te whakarapopototanga.

He aha te mate kiriuhi sclerosing tuatahi (PSC)?

He kupu ngāwari noa iho, ko te PSC he mate e puta ana i roto i ngā awaawa o te ate . E mahara ana pea koe, ka puta te awaawa i roto i te ate. Ka āwhina tēnei awaawa i te nakunaku. Nō reira, he pūnaha ngongo iti kei reira e kawe ana i tēnei awaawa mai i te ate ki ngā whekau. Ka kiia aua ngongo ko ngā awaawa.

I roto i te PSC, he mumura mau tonu , he pupuhi rānei, i roto i ēnei awaawa au. Ka karangatia hoki tēnei e ngā tākuta ko te cholangitis . Ina roa te mumura, ka tīmata te hanga i ngā kōpura i roto i ngā awaawa au. Nā tēnei kōpura ka mārō, ka whaiti hoki ngā awaawa au. Ka kiia tēnei ko te sclerosis .

Whakaarohia he paipa wai kua purua ki te paru, ā, ka whaiti, ka uaua te rere o te wai, tika? Waihoki, ina whaiti ngā awaawa o te au, ka herea te rere o te au. Ka kiia tēnei he stricture biliary . Nō reira, ina kore e taea e te au te rere tika, ka tīmata te hoki ki roto i te ate. Ka kino haere te ate i tēnei kohikohinga o te au i roto i te ate (cholestasis).

He kino rawa atu tēnei āhuatanga o te PSC?

Ae, he mate tino kino te PSC. Tērā pea kāore he tohu i ngā wāhanga tōmua. Engari he mate e ahu whakamua ana. Ina āraia te au i roto i ngā awa au (cholestasis), ka taea e ētahi o ngā paitini i roto i te au te hanga ki roto i te toto. Kātahi ka tīmata koe ki te kore e pai. Waihoki, ka puta he kino tonu ki te ate.

I te nuinga o te wā, ka taea e tēnei kino te arahi ki te ngoikore katoa o te ate i muri i te 10 ki te 15 tau. E mōhio ana koe, kāore e taea e tātou te ora me te kore he ate. Ahakoa ka taea e ngā maimoatanga o nāianei te whakahaere i ngā tohu, kāore anō kia kitea he rongoā mō te PSC. Ko te otinga anake ko te whakawhitinga ate .

He aha te rerekētanga i waenga i te cholangitis sclerosing tuatahi me te tuarua?

Ko te tikanga o te kupu "tuatahi" i konei ko te mate te tino take o te mumura me te whara o ngā awaawa au. Arā, ko tēnei mate anake te take o te mate, kāore he take kē atu.

Engari,I roto i te āhuatanga e kiia nei ko te secondary sclerosing cholangitis, ka puta te mumura (cholangitis) me te whara (sclerosis) o ngā awa waikura hei hua o tētahi atu take. Arā, i puta te āhuatanga i tētahi atu mate, i tētahi atu huihuinga rānei. Anei ētahi tauira:

• Te whara o ngā awaawa o te ate (hei tauira, i te wā e pokanga ana).
• Ngā mate urutā i roto i ngā awaawa o te ate.
• Te rongoā ārai matepukupuku (Chemotherapy).
• Ngā kōhatu o te pūhā e ārai ana i ngā awaawa o te pūhā.
• Te pancreatitis e hokihoki ana.

He aha te rerekētanga i waenga i te cirrhosis biliary tuatahi (PBC) me te PSC?

Ko te mate i mua i mōhiotia ko te Primary Biliary Cirrhosis, ināianei ka kiia ko te Primary Biliary Cholangitis (PBC) .

He mate ahu whakamua te PBC me te PSC e pā ana ki te ate, e pā ana ki ngā awaawa o te ate. He tino ōrite ngā tohu me ngā pānga o ngā mea e rua. Ka ngoikore haere ngā awaawa o te ate i ēnei mate e rua. Ina pakaru, ina whara, ina whaiti hoki ngā awaawa o te ate, ka āraia te rere o te ate. Kātahi ka hoki te ate ki roto i te ate, ka kino ake te ate. Ka taea e tēnei te arahi ki te whara o te ate, ā, i te mutunga ka mate a te ate ki te cirrhosis .

Engari he maha ngā rerekētanga matua i waenga i ēnei mate e rua:

• Ka pā noa te PBC ki ngā awaawa au i roto i te ate (intrahepatic). Ka pā pea te PSC ki ngā awaawa au i roto i te ate me ngā awaawa au i waho o te ate (extrahepatic).
• He nui ake te kitea o te PBC i roto i ngā wāhine (ōwehenga 10:1), ko te PSC ia he nui ake te kitea i roto i ngā tāne (ōwehenga 2:1).
• Tata ki te 80% o ngā tūroro PSC kua pāngia e te mate puku mumura (IBD) i mua, inā koa te mate puku ulcerative . Heoi, ehara tēnei i te hononga nui ki te PBC.
• He nui ake te mōrearea o te mate pukupuku o te awaawa ate me te PSC. Kāore he mōrearea nui pērā me te PBC.
• E whakamahia ana te waikawa Ursodeoxycholic (UDCA) hei rongoā i te PBC, engari kāore anō kia kitea he rongoā motuhake mō te PSC i tēnei wā.

He pēhea te noa o te ingoa o tēnei mate ko te PSC? Ko wai te hunga e pāngia ana e tēnei mate?

He mate tino onge te PSC. E kiia ana kotahi anake i roto i te tekau mano tāngata kei te pāngia e tēnei mate puta noa i te ao.

E rua ngā wā ka kitea tēnei mate i roto i ngā tāne i ngā wāhine. Ka kitea te mate i te nuinga o te wā i te 40 tau te pakeke. Tata ki te 80% o te hunga whai PSC he IBD (Inflammatory Bowel Disease) anō hoki, inā koa ko te Ulcerative Colitis . Mēnā he mate tēnei kei roto i tētahi o te whānau, ka tūpono ētahi atu ki te pāngia e tēnei mate.

He aha ngā take o te PSC?

Kāore anō kia mōhiotia te tino take o te PSC, engari e whakaarohia ana he maha ngā āhuatanga i puta mai ai. Ko ētahi o ēnei ko:

• Ngā ira
• Ngā āhuatanga taiao
• Ngā pūtau ārai mate

E whakaaro ana ngā tākuta he momo mate pūnaha ārai mate te PSC. Ko te tikanga o tēnei, ka tīmata te pūnaha parepare o tō tātou tinana, arā, ngā hōia e tiakina ana tātou i te mate, ki te whakaeke hē i ō tātou ake pūtau hauora. Ko te mumura tētahi o ngā patu o tō tātou pūnaha ārai mate. He wā poto noa iho, e mahi ana ki tētahi whakaeke. Engari, ki te roa te mumura, he tohu mate noa iho.

Kua kitea hoki e ngā tākuta he nui ake te tūponotanga o te hunga whai PSC ki te whakawhanake i ētahi atu mate pūnaha ārai mate. Hei tauira:

• Ngā mate puku mumura
• Mate celiac
• Mate tairoi
• Mate huka momo 1
• He mate hepatitis ā-aunoa
• Pancreitis ā-aunoa

E whakaarohia ana ka puta pea tēnei momo urupare ārai mate ina pāngia te hunga whai āhuatanga ira e ētahi āhuatanga taiao (hei tauira, te pānga ki ngā matū paitini).

He aha ngā tohu o te PSC?

I te haere whakamua o te PSC, ka rerekē ngā tohu. Tata ki te 50% o te tangata kāore pea he tohu ina kitea te mate. He maha ngā wā ka kitea te PSC i te wā o ngā whakamātautau mō tētahi atu mate. Ko ngā tohu tōmua kāore i te tino kitea. Kei roto i ēnei:

• Ngenge
• Te mamae o te puku o te taha matau o runga
• Kiri mākūkū (pruritus)

Ko ngā tohu ka puta i te whanaketanga o te mate ko:

• Puku pupuhi
• Ate nui haere
• Te whēkau nui
• Te kiri māwhero - Ko te tikanga tēnei he kōwhai ngā mā o ngā kanohi me te kiri.
• Kirika
• Te whakahekenga taumaha kāore i te whakaarohia

He aha ngā raruraru o te PSC kino?

He mate āta haere te primary sclerosing cholangitis, engari i te mea ka pakaru ngā awaawa o tō ate me tō ate, ka heke hoki te mahi a aua awaawa, ka puta pea ngā raruraru maha.

Ngā mate pāwera

Mena he nui te aukati o ō awa au, kāore e taea e rātou te pupuhi tika i te au ki roto i tō whekau iti. He mea nui te au mō te nakunaku i te kai, inā koa te ngako, me te mimiti i ngā huaora wairewa-ngako (A, D, E, me te K). Ki te kore he au, kāore e taea e tō pūnaha nakunaku te tukatuka tika i te ngako, ā, kāore e taea te mimiti i ēnei huaora nui mai i te kai. Ka arahi pea tēnei ki ētahi atu raruraru:

• Ngā tūtae momona me te mate korere
• Te ngoikore o te mimiti me te kore kai pai
• Ngāwari te maru me te toto (nā te korenga o te huaora K)
• Te ngoikore o ngā wheua - Osteomalacia me te osteoporosis (nā te korenga o te huaora D)
• Te tirohanga pōuri (nā te korenga o te huaora A)

Te toto toto tiketike o te tomokanga

I te haere whakamua o te mate ate cirrhosis, ka heke te rere o te toto i roto i te ate. Ka piki ake te pēhanga i roto i te uaua portal, e rere ana i roto i tō pūnaha nakunaku. Ka taea e tēnei pēhanga te whakanui me te pakaru o ētahi atu uaua i roto i tō korokoro me tō puku. Ka taea e tēnei te arahi ki te toto o roto .

Ngā mate urutā

He maha ngā wā ka pangia ngā awaawa au kua purua, ka puta ai ngā āhuatanga pēnei i te kirika, te mamae o te puku, me te paihana toto (sepsis).

Matepukupuku

I te kaha haere o te PSC, ka piki ake te mōrearea o te whakawhanake i ētahi momo mate pukupuku:

• Matepukupuku o te awa au (cholangiocarcinoma) - Te tūponotanga i waenga i te 5% me te 20%.
• Matepukupuku o te pūkahukahu
• Matepukupuku ate (hepatoma)
• Matepukupuku repeure - mēnā kei a koe anō te IBD.

Me pēhea te tātaritanga o te PSC?

I te nuinga o te wā, ka kitea te mate mā te tūponotanga, ina whakamātauria mō tētahi atu mea. Tata ki te 50% o te tangata kāore pea he tohu ina tautuhia te mate. Ka kitea ngā tohu tōmua o te mate mā te whakamātautau toto, mā te whakamātautau whakaahua rānei. Ka whakaatuhia e ngā matawai o ngā awaawa au me te PSC ngā āhuatanga motuhake. Mena ka whakaatuhia e te whakamātautau toto he taumata teitei o te whākōkī e kiia nei ko te alkaline phosphatase , he tohu rānei kei reira ētahi paturopi e tohu ana i te urupare ārai mate ki ngā awaawa au, he tohu pea tēnei nō te PSC. Ko te pikinga o ngā toto toto mā he tohu noa iho nō te mate ate.

Hei whakaū i te mate, ka tūtohu pea tō tākuta i ētahi atu whakamātautau motuhake:

• Whakamātautau mahi ate (LFT)Mā ēnei whakamātautau toto ka kitea he taumata teitei o ngā whākōkī motuhake i roto i te ate. Tērā pea he tohu PSC te taumata teitei o te phosphatase kawakore.
• Ko te cholangiopancreatography aukume (MRCP) : He matawai MRI (Magnetic Resonance Imaging) tēnei. Ka taea e ia te tango pikitia taipitopito o tō pūnaha biliary (ate, pūkahukahu, me ngā awa aukume). Koinei te matawai tuatahi hei tautuhi i te PSC. Nā te mea kāore e uru ki te whakaekenga, ā, kāore e uru ki te pānga o te irahiko. Heoi, i ētahi wā, ki te mea he wawe te mate, kāore rānei i te tino kino, kāore pea e taea te kite tika. Kātahi ka hiahiatia he matawai anō.

Me pēhea te whakahaere i te PSC?

I tēnei wā, kāore he maimoatanga hei aukati, hei whakakore rānei i te haere whakamua o te mate korokoro sclerosing tuatahi. Heoi, ka taea te rongoā tika i ngā tohu me ngā raruraru. Hei tauira, ka tohua pea e tō tākuta ngā mea pēnei i:

• Ngā rongoā hei rongoā i te kiri mākūkū (pruritus)
• Ngā tāpiringa hei rongoā i ngā ngoikoretanga o te huaora
• Ngā paturopi hei rongoā i ngā mate pāwera

Ka tirotirohia e tō tākuta tō ate me ō awa wai. I te haere tonu o te mate, ka huakina pea e rātou tētahi awa wai kua āraia. Ka taea tēnei mā te whakamātautau e kīia nei ko te ERCP (endoscopic retrograde cholangiopancreatography) . He tikanga tēnei e āhei ai koe ki te tiro ki roto i ō awa wai me te kore pokanga.

He momo endoscopy te ERCP. Ko te tirotiro i ō whekau mā te whakamahi i te endoscope, he ngongo roa, ngāwari me te kāmera iti me te mārama kei runga. I muri i te whakamoenga, ka whakaurua e tō tākuta tēnei ngongo ki raro i tō korokoro, ki roto hoki i tō puku. Ka taea te tuku i ngā taputapu iti mā roto i tēnei ngongo hei rongoā i ō awa wai. Ka taea e te tohunga hangarau te whakamahi i te poihau hei whakawhānui i tō awa wai, te whakauru rānei i te stent hei pupuri i te tuwhera. Mena kāore e taea e te ERCP te tae atu ki te aukati, ka whakamahia i ētahi wā he tikanga e kiia nei ko te percutaneous trans-hepatic cholangiography (PTHC).

Engari he otinga rangitahi noa iho tēnei. I roto i te 10 ki te 20 tau, ka kino haere te mate ate, ka kino haere te mate ate, ā, ka mutu ka pā te ngoikore o te ate. Ka aroturukihia e tō tākuta te kino o tō ate, ā, ka whakatau i te wā e whakaarohia ai he whakawhitinga ate . Me tutuki hoki e koe ētahi paearu hei whakauru atu ki te rārangi tatari mō te whakawhitinga ate.

Kia pēhea te roa ka taea e koe te noho tahi me te PSC?

Kia kitea te mate, ko te roanga o te oranga toharite kei waenganui i te 10 me te 20 tau. Ka taea e te whakawhitinga ate te hoatu he oranga hou. Heoi, i roto i te 15% ki te 20% o ngā take, ka taea e te PSC te hoki mai ahakoa kua whakawhitinga ate. Mena ka tupu tēnā, ka taea hoki e te ate hou te pakaru. I roto i ēnei take, ko te roanga o te oranga toharite he tata ki te iwa marama.

Ko tētahi atu āhuatanga ka pā ki te roanga o te oranga ko te mate pukupuku. Mena ka pāngia koe e te mate pukupuku hei raruraru o te PSC, kāore pea koe e pai mō te whakawhitinga ate. I roto i ngā take kua whiriwhiria marietia, ka rongoā tuatahi pea e ngā tākuta te mate pukupuku mā te irahiko, te rongoā chemotherapy rānei, kātahi ka whakamātau i te whakawhitinga ate.

Me pēhea taku tiaki i ahau anō mēnā kei a au tēnei mate?

Mā te whakarerekē i te āhua noho ka āwhina i te whakahaere i te ngenge e pā ana ki te PSC, ā, ka ārai i te kino atu o te ate. Hei tauira:

• Āta karohia te waipiro.
• Kaua e kai i ngā kai tukatuka, engari me kai i ngā kai māori.
• Whakahaerehia te ahotea.
• Moe pai.
• Whakakorikori tinana i ia rā, i ia rā.

He mate onge, kāore e taea te matapae, ā, ka taea te ārai te mate kolangitis sclerosing tuatahi. Ka puhoi te whanaketanga, ā, he maha ngā wā kāore he tohu i ngā wāhanga tōmua. Engari ki te toro atu koe ki tō tākuta i ia wā, ā, ka tirotirohia koe i ia wā, ka taea te kite i te mate i mua i te tīmatanga o te pānga ki tō oranga. Koinā te wā ka taea e koe te whakarerekē i te āhua noho hei āwhina i te hauora o tō ate mō te wā roa. Ka tukuna atu hoki koe ki te tiaki hauora mō te wā roa, arā, me tirotiro me te wawao i ia wā mēnā e tika ana.

Hei whakamutunga, ngā mea hei maumahara

Āe, ko tāku tumanako kua āwhina i a koe ngā mea kua kōrerohia e tātou mō te PSC kia maumahara ai koe ki ētahi o ngā mea tino nui. He āhuatanga uaua tēnei, engari he mea tino nui kia mōhio koe ki tēnei.

• He mate te PSC e pā ana ki ngā awaawa o te ate, ā, ka kino haere i te roanga o te wā. Ka taea e ia te kino i te ate.
• Kāore pea he tohu e kitea i ngā wāhanga tōmua, nō reira he mea nui kia tirotirohia te hauora i ia wā.
• Kāore he rongoā mō tēnei mate i tēnei wā, engari he maimoatanga kei reira hei whakahaere i ngā tohu me te whakapai ake i te kounga o te oranga.
• Ko te whakawhitinga ate te otinga matua i ngā take tino kino.
• Mā te whai i tētahi momo noho hauora ka āwhina i te tiaki i te ate.

Mena he pātai anō āu mō te PSC, ki te whakaaro rānei koe kei a koe tētahi o ēnei tohu, me toro atu ki te tākuta mō ētahi tohutohu . Kaua e manukanuka, kei reira ētahi mea ka taea e koe te mahi hei whakahaere i tēnei mate mena ka kitea wawetia.

` Te Mate Pukupuku Tuatahi, PSC, Mate Puku Au, Mate Ate, Waikura, Mamae Puku, Te Mate Pukupuku Tuatahi