Kua rongo kē koe i tētahi mate ira onge? I ētahi wā, ka pāngia ō tātou tinana e ngā raruraru kāore tātou e mōhio kei a tātou. I tēnei rā, ka kōrero tātou mō tētahi mate onge he mea nui kia mōhiotia. Ko te mate Turcot te ingoa.

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He aha te āhua o te mate Turcot?

He māmā noa iho, he mate ira onge te Turcot Syndrome. Ko te nuinga o te wā , ko te whanaketanga o ngā tipu iti (e kiia ana ko ngā polyp) i roto i tā tātou pūnaha nakunaku, arā, i roto i ngā whekau, me ngā pukupuku i roto i te roro, i te taurakira rānei. Whakaarohia te whakarihariha o te raru i ngā wāhi e rua i te wā kotahi.

Ina puta ēnei tipu iti (polyps) i roto i ngā whekau, ka pāngia ētahi tāngata e ngā tohu pēnei i te toto i te pūkahukahu, te neke haere o te puku (korere), me te mamae o te puku . Waihoki, i runga i te rahi me te wāhi o te pukupuku i roto i te roro, i te taurakira rānei , ka pāngia pea ētahi atu tohu neurological pēnei i te ānini, te tirohanga pōuri, te ngaronga o te taurite, me te hinganga pinepine .

E whakapono ana ētahi kairangahau hauora ko te Turcot Syndrome he momo kē atu pea o te Familial Adenomatous Polyposis (FAP). Heoi, kāore anō kia whakamātauhia tēnei. He āhuatanga anō hoki te FAP e puta ai he maha ngā polyp iti i roto i te koroni i mua i te whanaketanga o te mate pukupuku. Tērā pea he hononga kei waenganui i ngā mea e rua, nā te mea he whakarerekētanga ira kei roto i te ira APC o ētahi tūroro Turcot Syndrome. Ka taea hoki e ngā whakarerekētanga ira o te ira APC te whakaputa i te FAP.

He tino onge tēnei, ā, he iti noa iho ngā kēhi kua pūrongohia i roto i ngā pūkete hauora o te ao katoa, tata ki te 150. Nō reira, ka taea e koe te whakaaro he onge rawa tēnei.

He mate tuku iho tēnei? Me pēhea te whanaketanga o tēnei mate?

Ae, he mate ira tuku iho te Turcot Syndrome, arā, ka tukuna iho i ngā mātua ki ngā tamariki mā roto i ngā ira . Ka puta mai i ētahi huringa, i ngā whakarerekētanga rānei, i roto i ō tātou ira. E rua ngā huarahi matua e pā ai ki a tātou:

1. Momo 1 Te Mate Turcot: E mōhiotia ana hoki tēnei ko te mate Turcot "tūturu". Ka tukuna iho hei āhuatanga tuku iho ā-ira. Ki te kī ngāwari, kia pāngia ai te tamaiti e tēnei mate, me whiwhi ngā mātua e rua i te whakarerekētanga ira. He rite ki te toa i te rota (engari ehara tēnā i te mea pai!). Ko te nuinga o te wā ka puta tēnei momo i ngā whakarerekētanga ira i roto i ngā ira (MLH1) me (PMS2).

2. Te Mate Turcutt Momo 2:He "āhua rangatira" tēnei i tukuna iho. Arā, ka taea e te tamaiti te pāngia e tēnei mate ahakoa ka riro mai i a rātou te whakarerekētanga ira e tika ana mai i te mātua kotahi anake, arā, te whaea, te pāpā rānei. Nā te panonitanga o te ira "(APC)" tēnei i puta ai. Ko te mahi a tēnei ira "(APC)" he ārai i te hanganga o ngā pukupuku matepukupuku i roto i tō tātou tinana, he aukati rānei i te tipu. Nō reira, ina kore taua ira e mahi tika, ka puta he raruraru.

He aha ngā tohu matua o tēnei mate?

Ko ngā tohu matua o te Turcot Syndrome, e ai ki te kōrero i mua ake nei , ko ngā polyp i roto i ngā whekau me te kotahi, neke atu rānei o ngā pukupuku i roto i te roro, i te taurakira rānei. Ka taea e ētahi tāngata te whai i ngā tini polyp, arā, ka tīmata te whanake i te wā taitamariki tonu.

Ngā tohu o ngā tipu iti (polyps) i roto i ngā whekau

Ka rerekē te maha o ēnei tipu iti (polyps) e tupu ana i roto i te tangata i ia tangata, i ia tangata.

Ko ēnei polyp e whanake ana i roto i te hunga whai mate Turcotte Momo 1 ka nui ake te tūponotanga ka noho hei mate pukupuku.
Ko te hunga whai mate Turcotte Momo 2 he nui ake te tūponotanga ka pāngia rātou e te mate kua whakahuatia i mua ake nei "Familial Adenomatous Polyposis (FAP)".

Ka taea e ēnei tipu iti (ngā polyp o te puku) e puta mai ana i roto i ngā whekau te whakaputa i ngā tohu pēnei i:

Mamae puku
Te kōroke
Korere
Te toto o te rectum
Te whakaheke taumaha, arā, te whakaheke taumaha

Ngā Tohu o ngā Puku o te Roro/Uira Tuaiwi

Ka pāngia te pūnaha io matua (CNS) e ngā pukupuku e puta mai ana i roto i te roro, i te taurakira rānei. Ko tō tātou pūnaha io matua te pūnaha e whakahaere ana i ngā mahi maha o te tinana, tae atu ki te roro me te taurakira. Ka puta pea ngā tohu pēnei i:

Te ngaronga o te taurite, te hinganga pinepine (ngā raruraru taurite)
Ngā mamae nui o te upoko
Te ngaronga o te kare ā-roto - te ngoikore o ngā ringa, ngā waewae, ngā wāhanga rānei o te tinana
Whakapairuaki, ruaki rānei
Ngā mate hopuhopu
Ngā raruraru tirohanga, tae atu ki te tirohanga rua, te tirohanga pōuri rānei
Te ahua nei ka ngoikore tetahi taha o tō tinana (hei tauira, tetahi ringa, tetahi waewae, tetahi taha rānei o tō tinana)

Ētahi atu āhuatanga me ngā momo mate pukupuku me te mōrearea nui ake

I ētahi wā, ka puta he pukupuku ngako kore-matepukupuku (lipomas) he ira parauri iti rānei (ngā ira café-au-lait) ki te kiri o te hunga whai mate Turcot Syndrome.

Hei tāpiritanga, ka whakanui ake tēnei āhuatanga i te mōrearea o te whakawhanake i ētahi momo mate pukupuku me ngā pukupuku. Ko ngā mea matua ko:

Matepukupuku koroni
Astrocytoma (he momo pukupuku roro)
Ependymoma (he momo pukupuku e tīmata ana i roto i ngā pūtau e whakarārangi ana i ngā wāhi wai o te roro me te taurakira)
Glioma (ngā pukupuku e puta mai ana i ngā pūtau tautoko o te roro)
Ko te Glioblastoma (he momo mate pukupuku roro tino kaha)
Ko te Medulloblastoma (he momo mate pukupuku e puta ana i roto i te cerebellum, arā, i te wāhanga o raro o te roro e tata ana ki te angaanga)
Te mate pukupuku pūtau taketake o te kiri

Me pēhea te tautuhi i tēnei mate? (Tautuhinga)

Hei whakatau mēnā kei a koe te mate Turcot Syndrome, ka whakahaerehia e tō tākuta ētahi whakamātautau, ko te nuinga ka tirohia tō roro me ngā wāhi huri noa i ō whekau. Mō tēnei:

Tērā pea ka mahia ngā whakamātautau pēnei i te hihi-X, te MRI, me ngā karapa CT hei rapu i ngā pukupuku roro, i ngā polyp rānei o te puku. I ētahi wā motuhake, ka tūtohutia pea he karapa PET.
Colonoscopy: Ko te whakaurunga o tētahi ngongo iti, he rama kei roto i te nono hei tirotiro i te roto o te whekau nui me te rectum mō ngā āhuatanga rerekē.
Whakamātautau koiora: Mena ka kitea he pukupuku i roto i te koroni, i te roro rānei, ka tangohia he wahi iti o te kiko ka tirotirohia i raro i te karu hiko.

Ko te mea nui, mēnā he mate Turcot Syndrome tō tētahi o ō mātua, he nui ake te tūponotanga ka tirotirohia koe mō te mate.

I roto i tēnei āhuatanga, ka tūtohu pea te tākuta i ngā mea penei:

Whakamātautau DNA: Ka taea e tēnei whakamātautau ira te kite i te aroaro o ngā ira kua whakarerekētia e hua ai te mate Turcotte.
Sigmoidoscopy: Mā tēnei ka tirohia te wāhanga o raro o te whekau nui (te koroni sigmoid). Ka taea tonu e ngā taiohi kua riro mai i a rātou te ira mō te mate Turcotte te tirotiro i te koroni tae noa ki te 35 tau te pakeke. Mā tēnei ka taea te kite wawe me te rongoā i ngā tipu iti (polyps) i roto i te koroni.

He aha ngā maimoatanga mō te mate Turcot?

He rerekē te maimoatanga mō te mate Turcot i runga i ngā tohu.

Me tango pea he polypectomy hei tango i ngā tipu iti (polyps) i roto i tō koroni. Tērā pea ka tūtohu tō tākuta kia maha ngā pokanga hei aukati i te puta anō o ēnei tipu. Hei tauira:

Ileoproctostomy: Ka tangohia te whekau nui, ā, ka honoa te pūkahukahu me te whekau iti.
Ileostomy:Ka tangohia te pūkahukahu, ka honoa te whekau iti ki waho o te puku (hei whakarato i tētahi ara motuhake mō te putanga o te paru).
Ileoanal anastomosis: Ka hono i te whekau iti me te nono.
Colectomy: Te tango i tētahi wāhanga, i te katoa rānei o te koroni.
Proctocolectomy: Ka tangohia te koroni me te pūkahukahu.

Ka rerekē pea te maimoatanga mō ngā pukupuku i roto i te roro, i te taurakira rānei. I te nuinga o te wā ka ngana ngā tākuta ki te tango i te pukupuku. I te wā e rongoatia ana, ka ngana rātou ki te whakaiti i te kino ki ngā kiko hauora e karapoti ana. Hei mahi i tēnei:

Te rongoā matepukupuku
Te rongoā irahiko
Pokanga

Ngā tikanga maimoatanga pēnei i:

Kei te tūpono anō hoki ahau ki te pāngia e tēnei mate?

Mena kei tētahi o ō mātua te mate Turcot Syndrome, he nui ake te tūponotanga ka pāngia koe e te mate. Tērā pea he kaikawe ira koe. Ko te tikanga he kaikawe ira koe, kāore he tohu, engari kei a koe te ira e puta ai te mate, kia taea ai e āu tamariki te tuku atu.

Ki te whakapae koe kei a koe te mate Turcot Syndrome, ka tūtohu pea tō tākuta kia whakamātauria te DNA. Mā tēnei ka taea te tirotiro i te aroaro o te whakarerekētanga ira e tika ana.

He aha ngā mea ka puta ina noho tahi koe me tēnei mate? Kāore e taea te rongoā?

Engari, kāore he rongoā mō te mate Turcot Syndrome. Mena kei a koe tēnei mate, ko te mea nui ko te mahi tahi me tō tākuta ki te tirotiro i ngā pūtau roro me te mate pukupuku koroni. Ko te wawe o te kitenga i tētahi mea pēnei i te mate pukupuku, ko te nui ake o te tūponotanga ka angitu. Nō reira, he mea nui kia kaua e mataku, engari kia whai i ngā tohutohu a tō tākuta.

Ngā pātai nui hei pātai atu ki tō tākuta

Ka taea e koe te pātai atu ki tō tākuta i ētahi pātai pēnei i ēnei:

He aha te tino take o aku tohumate?
He aha ngā whakamātautau me mahi e au kia tino mōhio ai ahau mēnā kei a au te mate Turcot?
He kaikawe ahau i te ira mō tēnei mate?
He aha ngā kōwhiringa maimoatanga mō te mate Turcot?
He aha ngā mea ka pā mai mēnā kāore au e whiwhi maimoatanga?
He aha te tūponotanga ka whānau mai he tamaiti māku me te mate Turcot?

Kia maumahara, ko ngā pukupuku roro pēnei i te glioblastoma ehara i te mea tuku iho. Heoi, ko te hunga whai mate tuku iho pēnei i te Turcot Syndrome ka pāngia pea e ēnei pukupuku i ētahi wā.

Hei whakamutunga, ētahi mea hei maumahara

He mate ira onge te Turcot Syndrome e puta ai ngā tipu iti i roto i ngā whekau (polyps) me ngā pukupuku i roto i te roro, i te taurakira rānei. Ka rerekē te maimoatanga i runga i ngā tohu. Tērā pea me pokanga koe hei tango i tētahi wāhanga o ō whekau, i tētahi pukupuku rānei i roto i tō roro, i tō taurakira rānei. Ahakoa kāore he rongoā mō tēnei mate, mā te aroturuki i ō tohu, te whakamātautau i ia wā , me te whiwhi maimoatanga wawe ka āwhina i a koe ki te whakahaere i tō mate. Nō reira, he mea nui kia tiakina tō hauora.

Te Mate Turcot , ngā mate ira, ngā polyp o te puku, ngā pukupuku roro, te mōrearea o te mate pukupuku, te whakamātautau ira