Kua pāngia tetahi o tō whānau e te mate pukupuku koroni, inā koa i te wā taitamariki? Kua kōrerotia rānei e te tākuta he maha ngā polyp kei roto i tō koroni? Tērā pea he mate ira kei roto i tō whānau. I tēnei rā ka kōrero tātou mō tētahi mate onge engari he tino nui. Ko te ingoa ko te FAP, ko te Familial Adenomatous Polyposis rānei. Ahakoa he uaua te ahua o te ingoa, kia māmā noa iho.
He aha te FAP?
He mate ira te FAP ka taea te tuku iho i tētahi whakatipuranga ki tētahi whakatipuranga. Ka whanakehia e te hunga whai mate nei he maha ngā pukupuku e kiia nei ko ngā adenomas , ka taea te whanake hei mate pukupuku, i roto i ō rātou koroni me te rectum.
Inaianei kei te whakaaro pea koe, "He mea noa te whai i tētahi pūwero i tō koroni?" Ae, he mea noa ki ētahi tāngata te whakawhanake i tētahi, i ētahi rānei o ēnei pūwero i a rātou e pakeke haere ana. Engari ko tētahi tangata whai FAP ka whakawhanake i ngā rau, tae atu ki ngā mano, o ēnei pūwero. Ā, ka tīmata i te wā taitamariki tonu, tae atu ki te 15, 16 rānei tau te pakeke.
Kāore ēnei polyp i te mate pukupuku i te tīmatanga. Engari e kiia ana he 'precancerous'. Ko te tikanga, ki te kore e rongoatia, he tino nui te tūponotanga ka whanake tētahi, neke atu rānei o ēnei polyp hei mate pukupuku colorectal .
Whakaarohia te uaua o te tango i ngā rau o ēnei pukupuku takitahi. Kāore e taea. Koia te take, hei whakahaere i tēnei mōrearea nui, ka tūtohu ngā tākuta kia tangohia te whekau nui katoa mā te pokanga, arā, he tango katoa i te puku . I ētahi wā, ka tangohia hoki te pūkahukahu (proctocolectomy).
Ki te kore tēnei pokanga, ko te nuinga o te hunga whai FAP ka pāngia e te mate pukupuku i te pakeketanga. Waihoki, ko te hunga whai FAP kei te mōrearea ki te whakawhanake i ngā pukupuku ehara i te mea i roto i te koroni anake engari i roto hoki i ētahi atu wāhanga o te tinana (hei tauira, te puku, te whekau iti, te kiri, me ngā wheua). Nō reira, ahakoa kua tangohia te koroni, me tirotirohia tonutia rātou e te hauora puta noa i ō rātou oranga.
He aha te mōrearea o te mate pukupuku me te FAP?
Ki te kore e rongoatia, tata ki te 100% te tūponotanga ka pāngia te FAP e te mate pukupuku koroni. He mate tino kino tēnei. Ka pāngia te hunga whai FAP e te mate pukupuku i te wā tamarikitanga, ā, ka tere ake i te tangata noa. Nō reira, ki te mōhio koe he hītori whānau tō te FAP, me whai colonoscopy tāu tamaiti i ia tau, mai i te 10 tau te pakeke.
Haunga te mate pukupuku koroni, ka tūpono hoki te hunga whai FAP ki te whakawhanake i ētahi atu momo mate pukupuku.
| Momo mate pukupuku | Te tūponotanga o te puta (mā te tūponotanga ) |
|---|---|
| Matepukupuku duodenal | ~8% |
| Matepukupuku tairoi papillary | ~2% |
| Matepukupuku repeure | ~2% |
| Matepukupuku ate (Hepatoblastoma) - inā koa i roto i ngā tamariki | ~1.5% |
| Matepukupuku puku | ~1% |
| Ngā pukupuku roro me te tuaiwi | I raro iho i te 1% |
He rerekē ngā momo FAP?
Ae, he momo matua o te FAP me ētahi momo iti he rerekē paku, he iti ake te kino. Kia tirohia tātou he aha rātou.
| Momo FAP | Whakaahuatanga |
|---|---|
| FAP Matarohia (FAP Matarohia) | Koinei te momo tino noa. Neke atu i te 100, pea mano, o ngā polyp e puta ana i roto i te koroni. |
| FAP Whakaitihia (AFAP) | He iti ake te kino o tēnei. Ko te maha o ngā whēkau e tupu ana i roto i te whekau kei waenga i te 20 me te 100. |
| Te mate Gardner | He rite tēnei ki te FAP Matarohia, arā, neke atu i te 100 ngā pukupuku e puta mai ana. Hei tāpiri, ka puta mai anō hoki ētahi atu momo pukupuku i ētahi atu wāhanga o te tinana, hei tauira, i roto i te kiri, i ngā kiko ngohengohe, me ngā wheua. |
| Te mate Turcot | Ka puta he maha ngā pukupuku i roto i ngā whekau, me te pukupuku matepukupuku hoki i roto i te roro. |
He aha te rerekētanga i waenga i te FAP me te Lynch Syndrome?
Ko te mate Lynch tētahi atu mate ira ka taea te tuku iho i roto i ngā whakatipuranga, ā, ka piki ake te mōrearea o te mate pukupuku koroni. Engari he rerekētanga nui kei waenganui i ngā mea e rua. Kāore te hunga whai mate Lynch e whakawhanake i ngā rau polyp, pērā i te FAP. I ētahi wā, ka whanake tētahi, e rua rānei ngā polyp, ā, ka tere tonu te noho hei mate pukupuku. Koia te take i ētahi wā ka kiia ai te mate Lynch he mate "nonpolyposis". Ko ngā mate e rua ka puta mai i ngā hapa o ngā ira rerekē.
He aha ngā tohu o te FAP?
I roto i te FAP, ka tīmata te whanaketanga o ngā pukupuku koroni i mua atu i te taupori whānui, he maha ngā wā i te wā taitamariki. Kāore ēnei pukupuku e puta he tohu tae noa ki te wā e tino nui ana. Koia te hiranga o te tirotiro.
Heoi, nā te nui o ngā pūwero, ā, ka tere te tipu, ka puta pea ngā tohu i ētahi wā. Ko ēnei tohu ko:
- Te toto o te rectum
- Te mate korere tonu
- Mamae puku mau tonu
Haunga tēnei, tera pea ka pāngia te hunga whai FAP e ngā tohu o waho ka taea e te tākuta te kite ngāwari.
- Dermatofibromas: He puku mārō, rite ki te koha e hanga ana i raro i te kiri.
- Ngā pūwero kiri: He pūwero porowhita kī tonu i te keratin e hanga ana i raro i te kiri.
- Ngā wheua wheua:He pukupuku kore-matepukupuku e puta mai ana i roto i ngā wheua. He maha ngā wā ka kitea ēnei i roto i te angaanga, i te hope rānei.
- Niho tāpiri, niho pakaru rānei: Ka taea te kite i ēnei mā te hihi-X niho.
- Ngā ira whero o te retina (CHRPE): Ka kitea ēnei i te wā e tirotirohia ana te kanohi. Heoi, kāore e pā ki te tirohanga.
He aha te tino take o te FAP?
E ai ki tā mātou i kōrero ai i mua ake nei, ko te FAP i puta mai i tētahi whakarerekētanga ira. Ko te ira e pā ana ki tēnei ko te ira APC (Adenomatous Polyposis Coli) .
Ko ngā mea katoa o roto i tō tātou tinana e whakahaeretia ana e ngā ira, pērā i te hōtaka rorohiko. Ko tēnei ira APC he 'ira pehi pukupuku'. Ara, ko te mahi matua o tēnei ira he aukati i ngā pūtau kia kore e taea te wehewehe me te hanga pukupuku. Pērā i ngā parai o te motuka.
He ira APC kua whakarerekētia te tangata whai FAP, arā, he hapa tōna. Kātahi, pērā i te motuka kua pakaru ngā paraikete, ka wehea ngā pūtau me te kore e taea te whakahaere, ā, ka hanga rau ngā pukupuku.
He mea tuku iho tēnei hapa ira. Mēnā kei tētahi o ngā mātua tēnei whakarerekētanga ira APC, e 50% te tūponotanga ka riro i te tamaiti . Ko te tikanga tēnei, tera pea ka pāngia ia tamaiti, tera pea kāore rānei. Heoi, tata ki te 30% o te hunga kua tāpaetia he FAP kāore he hītori whānau. Ko te tikanga tēnei kua puta ake tēnei hapa ira i roto i ō rātou tinana i ēnei wā tata nei (whakarerekētanga taketake).
He aha ngā raruraru ka pā mai i te FAP?
Ko te raruraru nui rawa atu, me te mea tino morearea hoki hei whakahaere mā te FAP ko te matepukupuku koroni . Ka tino whakaitihia tēnei mōrearea ina tangohia te koroni mā te pokanga. Heoi anō, he uauatanga anō kei te noho me te kore he koroni. Ka rerekē te tukanga nekenekehanga o te puku. Mō tēnei, ka taea te whakamahi i te putea ileostomy , i te putea ileal rānei.
Nā te mea kei roto i ngā pūtau katoa o te tinana te hapa o te ira APC, ka taea hoki e ngā pukupuku te puta i waho o te whekau. Ko ētahi o ēnei he tūponotanga ka noho hei mate pukupuku.
- Ngā polyp o te duodenum: Ka puta ēnei i roto i te nuinga o te hunga whai FAP. Ka taea e te iti o ēnei te whanake hei mate pukupuku o te duodenum, te awa au, te awa pancreatic rānei.
- Ngā polyps o te puku: Ahakoa e puta ana i roto i te 90% o te tangata, he iti noa iho te ōrau, tata ki te 1%, ka huri hei mate pukupuku.
- Ngā pukupuku Desmoid: Ehara ēnei i te matepukupuku. Engari ka tino kaha te horapa ki ngā whekau me ngā uaua toto e tata ana, ka pakaru. He uaua ki te tango, ā, ka hoki mai anō.
- Matepukupuku tairoi: Ka pā pea tēnei ki te 2% o te hunga whai FAP. He maha ngā wā ka taea te rongoā katoa i ēnei.
- Matepukupuku ate: hepatoblastoma, inā koa i roto i ngā tamariki whai FAPHe iti noa te mōrearea o te whakawhanake i tētahi momo mate pukupuku ate onge e kiia nei ko te .
Me pēhea te rongoā me te whakahaere?
Ka taea te wehewehe i te mahere maimoatanga mō te FAP kia rua ngā wāhanga: te pokanga me te whakamātautau mō te wā katoa.
1. Pokanga
Ko te nuinga o te hunga whai FAP matarohia me tango katoa te kōpū, arā, ko te tango i te kōpū katoa, i te 20 tau, i te tīmatanga rānei o te 30 tau. Mā tō tākuta e whakatau te wā me te pēhea e mahi ai i tēnei pokanga, i runga i tō āhua.
2. Te tirotiro auau
Me whai whakamātautau koe i mua atu, i muri mai hoki i te pokanga, ā, puta noa i tō oranga. Koinei te huarahi pai hei tiaki i tō oranga.
| Momo whakamātautau | Kaupapa | Te wā e taunakitia whānuitia ana |
|---|---|---|
| Te tirotiro koroni | Hei tirotiro mō ngā pukupuku i roto i te koroni me te tōku. | Mō te hunga e pāngia ana e te mōrearea, i ia tau mai i te 10 tau te pakeke tae noa ki te pokanga. |
| Sigmoidoscope | Hei tirotiro i te toenga o te pūtu, o te pēke ileum rānei i muri i te pokanga. | I muri i te pokanga, i ia 6-12 marama, i ia 1-4 tau rānei. |
| Te tirotiro i te pito o runga | Hei tirotiro mō ngā pukupuku i roto i te puku me te whekau iti (koroni). | I ngā wā ā-wā e ai ki ngā taunakitanga a te tākuta. |
| Matawai Ultrasound | Mō te mate pukupuku o te tairoi, o te ate rānei. | I runga i te tohutohu hauora. |
| Matawai CT, MRI rānei | Hei tirotiro mō ngā pukupuku desmoid. | I runga i te tohutohu hauora. |
Kei te pēhea te oranga me te FAP?
He mea noa te pouri me te ohorere ina rongo koe he mate ira tōu pēnei. Heoi, ko te mōhio wawe ki tēnei mate te huarahi pai ki te whakahaere i tō mōrearea mate pukupuku.
Mā te tiaki hauora tika me ngā tirotiro auau, ka taea e te tangata whai FAP te ora noa, te roa, te hauora . I muri i te tango i te koroni, ko te mōrearea nui rawa atu ka puta mai i ētahi atu mate pukupuku o te ara kai, i ngā pukupuku desmoid rānei. Engari he tino onge ake ēnei i te mate pukupuku koroni.
Ahakoa he huringa nui te pokanga ki te ao, nā te hangarau matatau o ēnei rā, ka taea e te pokanga laparoscopic te āwhina i a koe kia tere ake te ora.
Ko te mea nui ko te mōhio kāore koe i te mokemoke. Kei reira ngā tākuta, te whānau, me ngā hoa hei ārahi me te tautoko i a koe i tēnei haerenga. Nō reira, ki te mea he pātai, he āwangawanga rānei āu, kōrerotia mā te mahorahora ki tō tākuta.
Karere Tangohia ki te Kāinga
- He mate ira kino te FAP e tukuna iho ana i roto i ngā whakatipuranga, ā, ka puta ai ngā rau o ngā pukupuku i roto i te koroni.
- Ki te kore e rongoatia, e tata ana ki te 100% te mōrearea o te mate pukupuku koroni.
- Mena kua pāngia tetahi o tō whānau e te mate pukupuku koroni, e ngā pukupuku maha rānei i te wā taitamariki, kōrero ki tō tākuta mō te whakamātautau ira.
- He mea nui te kitenga wawe me te tirotiro auau hei whakaora i ngā oranga. Me tīmata te tirotiro i ngā tamariki e pāngia ana e te mōrearea i te 10 tau te pakeke.
- Ko te maimoatanga matua hei ārai i te mōrearea o te mate pukupuku ko te tango pokanga i te whekau nui (colectomy).
- Mā te maimoatanga hauora me te tirotiro tika, ka taea e koe te ora i te oranga hauora me te tino pai ahakoa kei a koe te FAP.
💬 Comments (0)
Kaore ano kia tukuna he korero. Tāpirihia to korero ki konei mo te wa tuatahi.
Tāpirihia to korero