He aha te MEN-2? Ngā Tohutohu, Ngā Take, me te Maimoatanga

He āhuatanga tuku iho onge te MEN-2 e puta ai ngā pūngāwerewere ki ngā whekau e hanga ana i ngā homoni puta noa i tō tinana. Ka taea e ēnei pūngāwerewere te pai (kāore i te mate pukupuku) te kino rānei (mate pukupuku), ā, ko te nuinga o te wā ka pā ki tō tāiroiti, ngā whekau adrenal, me ngā whekau parathyroid.

He mate tuku iho tenei e rere ana i roto i ngā whānau, ā, ko te take ko ngā huringa i roto i te kotahi o ngā ira e kiia nei ko RET. Ahakoa ka rongo te ingoa he wehi, ko te mārama ki te MEN-2 ka taea te āwhina i a koe me tō rōpū hauora ki te hanga i te mahere whakamātau me te maimoatanga.

He aha te MEN-2?

Ko te Multiple Endocrine Neoplasia Type 2 (MEN-2) he mate pukupuku tuku iho e pā ana ki tō pūnaha endocrine. Kei roto i tō pūnaha endocrine ngā whekau e hanga ana i ngā homoni hei whakahaere i ngā mahi maha o te tinana penei i te whakaheke, te toto toto, me ngā taumata kāratiuma.

Ka whiwhi te āhuatanga i tōna ingoa nā te mea ka puta mai ngā pūngāwerewere maha (neoplasia) i roto i ngā whekau endocrine maha i te wa kotahi. Whakaarohia he rite ki ngā whare whakamahi homoni o tō tinana e whanake ana ngā tipu ka taea te whakaroa i te hanga homoni noa.

E rua ngā momo matua o te MEN-2. Ko te MEN-2A te momo noa, ko te MEN-2B te onge ake engari he kaha ake te whakaeke. Ko ngā momo e rua e puta mai ana i ngā rerekētanga i roto i te ira kotahi engari ka rerekē te pānga ki te tangata.

He aha ngā momo o te MEN-2?

Ko te MEN-2A te momo noa rawa, e 95% pea o ngā keehi MEN-2 katoa. Ko te nuinga o te hunga e pā ana ki te MEN-2A ka whanake i te mate pukupuku tāiroiti medullary, ā, he tokomaha hoki ka whiwhi i ngā pūngāwerewere i roto i ō rātou whekau adrenal e kiia nei ko ngā pheochromocytomas.

Ko ētahi o te hunga e pā ana ki te MEN-2A ka whanake hoki i ngā pūngāwerewere parathyroid, ka taea te whakaeke i ngā taumata kāratiuma i roto i tō toto. He iti te wāhanga ka taea te whiwhi i te āhuatanga e kiia nei ko te mate Hirschsprung, e pā ana ki te kopu nui.

Ko te MEN-2B he iti te noa engari he kaha ake te whakaeke. Ko te hunga e pā ana ki tenei momo ka whanake i te mate pukupuku tāiroiti medullary i te tau iti ake, me ngā pheochromocytomas. Ka taea hoki e rātou te whai i ngā āhuatanga tinana motuhake penei i ngā pūngāwerewere i runga i ō rātou arero me ō rātou ngutu, me te ahua roa, angiangi.

He aha ngā tohutohu o te MEN-2?

Ka rereke haere ngā tohutohu o te MEN-2 i runga i te whekau e pā ana me te momo pūngāwerewere e whanake ana. Kāore te tokomaha o te iwi e kite i ngā tohutohu i ngā wā tīmatanga, koia te take i nui ai te whakamātau ira me te whakamātau noa mō ngā whānau e pā ana ki tenei āhuatanga.

Ko ngā tohutohu noa rawa e pā ana ki ngā momo pūngāwerewere ka taea te whanake:

• He pūngāwerewere, he pupuhi rānei i tō kaki mai i ngā pūngāwerewere tāiroiti
• Te uaua ki te horomia, te reo ngūngū rānei
• Ngā wāhanga toto toto teitei e haere mai ana, e haere atu ana rānei
• Te tere o te ngākau, te pupuhi rānei
• Ngā mamae o te upoko nui
• Te wera nui
• Te manukanuka, te wehi rānei
• Ngā kōhatu whatukuhu, te mamae iwi rānei mai i ngā raruraru kāratiuma
• Te ruaki, te ngaro kai rānei
• Te ngoikore o ngā uaua, te ngenge rānei

I roto i te MEN-2B motuhake, ka taea e koe te kite i ngā pūngāwerewere i runga i tō arero, ngutu, i roto rānei i tō waha. Ko ētahi o te iwi he ahua roa, angiangi hoki me ngā waewae roa.

Ka taea e ēnei tohutohu te whanake haere i roto i ngā marama, ngā tau rānei. Ko ētahi o te iwi e pā ana ki ngā wāhanga ohorere o ngā tohutohu, ko te nuinga o te wā e pā ana ki ngā pheochromocytomas, ka taea te whakaeke i ngā piki nui o te toto toto me te tere o te ngākau.

He aha te take o te MEN-2?

Ko te take o te MEN-2 ko ngā rerekētanga i roto i te ira RET, e āwhina ana ki te whakahaere i te tipu o ngā pūtau me te whanaketanga. Ki te kore tenei ira e mahi tika, ka korero ki ētahi pūtau kia tipu, kia wehewehe rānei i te wā e kore ai, e arahina ana ki te hanganga pūngāwerewere.

Ka tuku iho tenei huringa ira, arā, ka pā atu i ngā mātua ki ngā tamariki. Ki te whai tētahi o ō mātua i te MEN-2, 50% te tupono o te tuku iho o te āhuatanga. Heoi, ko te 5% pea o ngā keehi MEN-2 e puta ana i ngā tāngata kāore he hītori whānau, e whakaatu ana i te rerekētanga i puta mai i te kore whakaaro.

Ko te ira RET te mahi penei i te tīwiri e whakahaere ana i te wā e tipu ai ngā pūtau. I roto i te MEN-2, ka mau tenei tīwiri ki te tūnga "on", e whakaeke ana i ngā pūtau i roto i ō whekau e hanga ana i ngā homoni kia maha rawa te maha, kia hanga ai ngā pūngāwerewere.

Ko ngā momo rerekētanga ira RET e puta ai ngā momo MEN-2. Ko te wāhi motuhake me te momo rerekētanga ka taea te āwhina i ngā tākuta ki te kī he whekau pea ka pā me te kaha o te āhuatanga.

Nōhea te haere ki te tākuta mō te MEN-2?

Me haere koe ki te tākuta ki te whai koe i te hītori whānau o te MEN-2, te mate pukupuku rānei e pā ana, ahakoa kāore koe e whai tohutohu. Ko te kite wawe mā te whakamātau ira ka ora ai te ora, nā te mea ka taea te maimoatanga whakamātau i mua i te whanake o ngā mate pukupuku.

Rapua te awhina hauora i te wa poto ki te pāngia koe e ngā wāhanga ohorere o te toto toto teitei, ngā mamae o te upoko nui, te tere o te ngākau, te wera nui rānei. Ka taea e ēnei te tohu o te pheochromocytoma, ka taea te whakaeke i ngā piki morearea o te toto toto.

Whakapa atu hoki ki tō kaiwhakarato hauora ki te kite koe i te pūngāwerewere i tō kaki, ngā huringa i tō reo, te uaua ki te horomia, te mamae iwi tonu rānei. Ahakoa he maha ngā take o ēnei tohutohu, he tika te aromatawai, ina koa ki te whai koe i ngā morearea mō te MEN-2.

Ki te kīia kē koe he MEN-2, whai i tō mahere whakamātau tata. Ka taea e ngā tirotiro noa te hopu i ngā pūngāwerewere hou i te wā e taea ai te rongoā.

He aha ngā morearea mō te MEN-2?

Ko te morearea matua mō te MEN-2 ko te whai i te hītori whānau o te āhuatanga. Nō te mea ka tuku iho te MEN-2 i roto i te tauira autosomal dominant, kotahi noa te kape o te ira rerekētanga e hiahiatia ana mai i tētahi mātua kia whanake ai te āhuatanga.

Ko ēnei ngā morearea matua hei whakaaro:

• Te whai i te mātua e whai ana i te MEN-2 (50% te tupono o te tuku iho)
• Te hītori whānau o te mate pukupuku tāiroiti medullary
• Te hītori whānau o te pheochromocytoma
• Te hītori whānau o ngā pūngāwerewere parathyroid
• Te whānau mai ki te whānau e mohiotia ana ngā rerekētanga ira RET

Kāore i rite ki ētahi atu āhuatanga, ko te tau, te oranga, me ngā take taiao kāore e pā nui ki tō morearea ki te whanake i te MEN-2. Ko te waahanga ira te mea nui rawa.

Heoi, ki te kawe koe i te rerekētanga ira, ko ētahi take pea ka pā ki te wā e puta mai ai ngā tohutohu, te kaha rānei o te whanake. Ka taea e te ahotea, te hapu, me ētahi atu āhuatanga hauora te whakaoho i ngā tohutohu i ngā tāngata kua whai kē i te āhuatanga ira.

He aha ngā raruraru pea o te MEN-2?

Ko te raruraru nui rawa atu o te MEN-2 ko te whanake o ngā mate pukupuku kaha, ko te mate pukupuku tāiroiti medullary. Ki te kore he whakamātau me te maimoatanga tika, ka taea e ēnei mate pukupuku te horapa ki ngā whekau lymph me ētahi atu wāhanga o tō tinana.

He maha ngā raruraru ka puta mai i ngā momo pūngāwerewere:

• Te horapa o te mate pukupuku tāiroiti medullary ki ngā whekau lymph, ki ngā whekau tawhiti rānei
• Ngā piki morearea o te toto toto mai i ngā pheochromocytomas
• Ngā raruraru ngākau mai i ngā wāhanga toto toto teitei kaore i te rongoā
• Te pēhanga, te mate ngākau rānei i ngā wā morearea o te toto toto
• Ngā kōhatu whatukuhu me ngā raruraru iwi mai i ngā pūngāwerewere parathyroid
• Te osteoporosis nui mai i ngā koretake kāratiuma
• Te aukati o te kopu i ngā tāngata e whai ana i te mate Hirschsprung

Ka taea e ngā Pheochromocytomas te whakaeke i ngā raruraru morearea i te wā o te pokanga, te hapu, te wā rānei o te ahotea tinana. Ka taea e ēnei pūngāwerewere te whakaoho i ngā piki toto toto morearea ki te kore e whakahaere tika.

Ko te rongo pai, mā te whakamātau me te maimoatanga tika, ka taea te aukati, te whakahaere rānei i te nuinga o ēnei raruraru. Ko te kite wawe me te pokanga whakamātau ka taea te whakakore i te morearea o te mate pukupuku katoa i roto i ngā keehi maha.

Me pēhea te whakatau o te MEN-2?

Ko te whakatau o te MEN-2 ka tīmata mā te whakamātau ira, ina koa ki te whai koe i te hītori whānau o te āhuatanga. Ka taea e te whakamātau toto ngāwari te kite i ngā rerekētanga i roto i te ira RET me te whakau i te kawe koe i te huringa ira e puta ai te MEN-2.

Ka whakamahi hoki tō tākuta i ētahi atu whakamātau hei tirotiro i ngā pūngāwerewere me te whakamātau i tō āhuatanga. Ka taea e ngā whakamātau toto te ine i ngā homoni motuhake me ngā tohu pūngāwerewere e whakaatu ana he pūngāwerewere kei reira me te kaha o te mahi.

Ko ngā rangahau whakaahua e āwhina ana ki te kimi me te aromatawai i ngā pūngāwerewere puta noa i tō tinana. Ka taea e ēnei te whakauru i te ultrasound o tō kaki, ngā rangahau CT, MRI rānei o tō kopu, me ngā rangahau motuhake ka taea te kite i ngā pūngāwerewere e hanga ana i ngā homoni.

Ki te whai kē koe i ngā tohutohu, ka taea e tō tākuta te mahi i ngā whakamātau atu penei i te ine i tō toto toto i ngā wāhanga, te tirotiro i ngā taumata kāratiuma, te tango rānei i ngā tauira kiko mai i ngā pūngāwerewere whakapae.

He aha te maimoatanga mō te MEN-2?

Ko te maimoatanga mō te MEN-2 e aro ana ki te aukati i ngā mate pukupuku kia whanake, ki te whakahaere rānei i ngā pūngāwerewere e puta ana. Ko te huarahi e whakawhirinaki ana ki tō rerekētanga ira motuhake, ngā pūngāwerewere kei reira, me tō hauora whānui.

Ko te pokanga te maimoatanga matua. Mō te hunga e whai ana i ngā rerekētanga ira morearea teitei, ka taea e ngā tākuta te tūtohutia te tango tāiroiti whakamātau i mua i te whanake o te mate pukupuku. Ka taea e tenei pokanga, e kiia nei ko te thyroidectomy prophylactic, te aukati katoa i te mate pukupuku tāiroiti medullary.

Ko ngā huarahi maimoatanga e whakauru ana i ētahi atu whiringa:

• Te thyroidectomy prophylactic hei aukati i te mate pukupuku tāiroiti
• Te tango i ngā pheochromocytomas ina whanake ana
• Te pokanga parathyroid mō ngā raruraru e pā ana ki te kāratiuma
• Te rongoā whakakapi homoni i muri i te tango whekau
• Ngā rongoā toto toto mō te whakahaere pheochromocytoma
• Ngā rongoā whakaeke mō ngā mate pukupuku whanake
• Te whakamātau noa me ngā whakamātau toto me ngā rangahau whakaahua

Ko te wā o te pokanga he mea nui, e whakawhirinaki ana ki tō tau, te momo rerekētanga ira, me ngā āhuatanga pūngāwerewere. Ka mahi tahi tō rōpū hauora ki a koe hei whakatau i te wā pai mō ngā tukanga whakamātau.

I muri i te pokanga, ka hiahia koe ki te rongoā whakakapi homoni hei whakakapi i ngā homoni e hangaia ana e ō whekau i tangohia. Ko te nuinga o te wā ka whakauruhia te homoni tāiroiti, me ētahi atu homoni i runga i te whekau i tangohia.

Me pēhea te whakahaere i te MEN-2 i te kāinga?

Ko te whakahaere i te MEN-2 i te kāinga e whakauru ana i te whai i tō mahere maimoatanga ata, te mataara ki ngā huringa i ō tohutohu. Ko te tango i ō rongoā whakakapi homoni kia rite ki te tohutohu he mea nui mō te pupuri i tō hauora me ō taumata kaha.

Pupurihia he puka tohutohu hei whai i ngā huringa, ngā tohutohu hou rānei e whanake ana. Ka āwhina ēnei korero i tō rōpū hauora ki te whakarite i tō maimoatanga me te hopu i ngā raruraru hou i te wā wawe.

Ko ēnei ngā rautaki whakahaere kāinga nui:

• Tangohia ngā rongoā katoa kia rite ki te tohutohu, ko te nuinga o ngā whakakapi homoni
• Whakamātautia tō toto toto noa ki te whai koe i ngā pheochromocytomas
• Pupurihia ngā korero whakapā āwhina i te wā ngāwari
• Pupurihia te kai hauora me te mahi whakakori noa
• Haere ki ngā whakaritenga whai muri i te whakarite
• Whakawhitiwhiti tika ki ngā mema whānau mō ngā morearea ira
• Whakahaerehia te ahotea mā ngā tikanga whakahaere, te tohutohu rānei

Ki te pāngia koe e ngā tohutohu ohorere penei i ngā mamae o te upoko nui, te tere o te ngākau, te huringa nui rānei o te toto toto, whakapa atu ki tō kaiwhakarato hauora i te wa poto, ki te rapu rānei i te awhina āwhina.

Whakaarohia te whakauru ki ngā rōpū tautoko mō te hunga e whai ana i te MEN-2, ngā āhuatanga rite rānei. Ko te hono atu ki ētahi atu e mārama ana ki tō wheako ka taea te whakarato i te tautoko ngākau nui me ngā tohutohu mahi mō te whakahaere o ia rā.

Me pēhea te whakarite mō tō whakaritenga tākuta?

Ko te whakarite mō tō whakaritenga e āwhina ana ki te whakarite ka whiwhi koe i te nuinga o tō wā me tō rōpū hauora. Kawe mai he rārangi katoa o ō tohutohu, tae atu ki te wā i tīmata ai rātou me te aha e pai ake ai, te kino ake rānei.

Kohikohia tō hītori hauora whānau, ko te nuinga o ngā korero mō ngā whanaunga e whai ana i te mate pukupuku tāiroiti, ngā pheochromocytomas, ngā pūngāwerewere e pā ana ki ngā homoni rānei. He mea nui ēnei korero mō te aromatawai i tō morearea me te whakarite i tō tiaki.

Hangaia he rārangi whakarite whānui:

• Rārangihia ngā rongoā me ngā taapiri katoa o nāianei
• Tuhia ō tohutohu me ngā rā me te kaha
• Kohikohia ngā korero hītori hauora whānau
• Whakaritehia ngā pātai mō te whakamātau ira, ngā whiringa maimoatanga rānei
• Kawe mai ngā hua whakamātau o mua me ngā rēkōta hauora
• Whakaarohia te kawe mai i te mema whānau mō te tautoko
• Tuhia ō raruraru matua me ō whāinga mō te maimoatanga

Kaua e whakaroa ki te ui pātai mō tetahi mea kaore koe e mārama. E hiahia ana tō rōpū hauora ki te āwhina i a koe ki te whakatau whakamārama mō tō tiaki.

Ki te whakaaro koe ki te whakamātau ira, te pokanga whakamātau rānei, whakaritehia ngā pātai mō ngā painga, ngā morearea, me ngā whiringa atu. Ko te mārama ki ō whiringa katoa e āwhina ana i a koe ki te whakatau i ngā whakatau pai mō tō āhuatanga.

He aha te mea nui mō te MEN-2?

He āhuatanga tuku iho nui engari ka taea te whakahaere te MEN-2 e pā ana ki ō whekau e hanga ana i ngā homoni. Ahakoa ka rongo te whakatau he nui te morearea, ko ngā whakapainga i roto i te whakamātau ira me te maimoatanga kua pai ake te hua mō te hunga e whai ana i tenei āhuatanga.

Ko te mea nui rawa hei mahara ko te kite wawe me te maimoatanga whakamātau ka taea te aukati i te maha o ngā raruraru nui e pā ana ki te MEN-2. Ko te whakamātau ira e taea ai ngā mahi whakamātau ka taea te whakakore i te morearea o te mate pukupuku katoa i roto i ngā keehi maha.

Ki te whai koe i te hītori whānau o te MEN-2, ngā āhuatanga rite rānei, kaua e tatari kia whanake ngā tohutohu. Ka taea e te tohutohu ira me te whakamātau te whakarato i ngā korero nui e arataki ana i ō whakatau hauora, ka ora pea tō oranga.

Ko te mahi tata ki te rōpū hauora e whai wheako ana ki te rongoā i te MEN-2 e homai ana te tupono pai mō ngā hua roa pai. Mā te tiaki tika, he tokomaha ngā tāngata e whai ana i te MEN-2 e ora ana i ngā oranga noa, hauora.

Ngā pātai e ui pinepinetia ana mō te MEN-2

Ka taea te whakaora i te MEN-2?

Ahakoa kaore e taea te whakaora i te MEN-2, no te mea he āhuatanga tuku iho, ka taea te aukati, te rongoā rānei i ngā mate pukupuku me ngā pūngāwerewere e puta ana. Ka taea e te pokanga whakamātau te whakakore i te morearea o te whanake o ētahi mate pukupuku katoa. Mā te whakamātau me te maimoatanga tika, he tokomaha ngā tāngata e whai ana i te MEN-2 e ora ana i ngā oranga roa kaore he raruraru hauora nui.

Nōhea te whakamātau ira wawe?

Ka taea te mahi i te whakamātau ira mō te MEN-2 i tetahi tau, tae atu ki ngā pēpi hou ki te whai koe i te hītori whānau e mohiotia ana. Heoi, ko te wā o te whakamātau e whakawhirinaki ana ki ngā āhuatanga whānau me te rerekētanga ira motuhake e pā ana. Ko ētahi whānau e whiriwhiri ana ki te whakamātau i ngā tamariki i te wā wawe hei tuku i te whakamātau me te tiaki whakamātau pai, ko ētahi atu e pai ana ki te tatari kia taea e te tamaiti te whai wāhi ki te whakatau.

He aha te mea ka puta mai ki te whakamātau pai koe mō te MEN-2?

Ko te whakamātau pai mō te rerekētanga ira MEN-2 te tikanga ka whanake pea koe i te āhuatanga i tētahi wā, engari ehara tena te tikanga kei a koe te mate pukupuku inaianei. Ka whakawhanake tō rōpū hauora i te mahere whakamātau me te maimoatanga whaiaro, ka taea te whakauru i ngā whakamātau toto noa, ngā rangahau whakaahua, me te pokanga whakamātau pea. Ko te kite wawe e tuku ana i ngā whiringa maimoatanga tino pai.

Ka taea e au te whānau tamariki ki te whai ahau i te MEN-2?

Ae, ka taea e te hunga e whai ana i te MEN-2 te whānau tamariki, engari he whakaaro nui hei kōrero ki tō rōpū hauora. 50% te tupono o ia tamaiti ki te tuku iho o te rerekētanga ira. Ka taea e te tohutohu ira te āwhina i a koe ki te mārama ki ngā morearea me ngā whiringa, tae atu ki te whakamātau i mua i te whānau me te whakamātau ira preimplantation mō te hunga e whakamahi ana i ngā hangarau whakawhānau āwhina.

Nōhea te wā e hiahiatia ana e au ngā whakaritenga whai muri?

Ko te auau o te whai muri e whakawhirinaki ana ki tō āhuatanga motuhake, tae atu ki tō momo rerekētanga ira, tō tau, me te mea kua whai koe i te pokanga whakamātau. Ko te nuinga o te wā, ko te hunga e whai ana i te MEN-2 e hiahia ana ki te whakamātau noa atu i te iwi whānui, ko te nuinga o te wā ka whakauruhia ngā whakamātau toto tau, ngā rangahau whakaahua rānei. Ka hanga e tō rōpū hauora he mahere whakamātau whaiaro e whakawhirinaki ana ki ō morearea motuhake.