He aha te MEN-1? Ngā Tohutohu, Ngā Take, me te Maimoatanga

Ko te Multiple Endocrine Neoplasia Type 1 (MEN-1) tētahi mate tuku iho onge e whakaeke ana i ngā pūngāwerewere ki te tupu i roto i ngā whekau maha e whakaputa ana i ngā homoni puta noa i tō tinana. Ko ēnei pūngāwerewere te nuinga he pai, ā, e kore e pēnei i te mate pukupuku, engari ka taea tonu e rātou te pā ki te huarahi e whakaputa ai tō tinana me te whakamahi i ngā homoni.

Whakaarohia tō pūnaha endocrine hei whatunga karere a tō tinana, me ngā whekau e tuku ana i ngā homoni hei whakahaere i ngā mea katoa mai i te huka toto ki te kaha o ngā wheua. Ki te whai koe i te MEN-1, ka taea e tēnei whatunga te whakawhanake i ngā tipu e whakaroa ana i te whakaputanga homoni noa, ka arahi ki ngā tohu maha kaore pea e rite ki te tīmatanga.

He aha te MEN-1?

Ko te MEN-1 tētahi mate tuku iho e pā ana ki ngā wāhanga matua e toru o tō tinana: ngā whekau parathyroid i roto i tō kaki, te pancreas, me te whekau pituitary i roto i tō roro. Ko te ingoa o te mate nā te mea e pā ana ki ngā whekau endocrine maha e whakawhanake ana i te neoplasia, koinei te kupu hauora mō te tipu o ngā kiko koretake.

Ko tēnei mate e pā ana ki te 1 i roto i ngā tāngata 30,000, no reira he onge rawa. Ko te mea e wero ana i te MEN-1 ko te mea ka taea e ia te whakaeke i ngā tohu rerekē ki ngā tāngata rerekē, ahakoa i roto i te whānau kotahi.

Ko ngā pūngāwerewere e hono ana ki te MEN-1 ka tupu haere i te wa. Ahakoa ko te nuinga he pai, ka taea tonu e rātou te whakaeke i ngā raruraru hauora nui mā te whakaputa i te nui o ētahi homoni, mā te tupu nui rānei ki te pēhi i ngā whekau e tata ana.

He aha ngā tohu o te MEN-1?

Ka rereke haere ngā tohu o te MEN-1, nā te mea ka whakawhirinaki ki tehea whekau e pā ana, me te nui o te homoni e whakaputa ana. He tokomaha kaore e mohio kei a rātou te MEN-1 tae noa ki te pakeke, i te mea ka tupu haere ngā tohu i roto i ngā tau.

Nā te mea e pā ana te MEN-1 ki ō whekau parathyroid te nuinga, ka kite pea koe i ngā tohu e pā ana ki ngā taumata teitei o te kākā i roto i tō toto:

• Ngā kōhatu whatukuhu e hoki mai ana
• Te mamae o ngā wheua, te whati ngāwari
• Te ngenge e kore e pai ake
• Te pouri, ngā huringa ngākau
• Te ngoikore o ngā uaua
• Te matewai nui, te mimi nui
• Te ruaki, te mamae o te puku
• Ngā raruraru mahara, te whakapohehe

Ki te pā te MEN-1 ki tō pancreas, ka kite pea koe i ngā tohu e pā ana ki ngā koretake homoni i reira. Ki te whakawhanakehia ngā pūngāwerewere e whakaputa ana i te insulin, ka taea e koe te whai i ngā wāhanga o te huka toto iti rawa me ngā tohu penei i te werawera, te wiri, te tere o te ngakau, te ngāwari rānei ki te ngaro.

Ko ētahi tāngata ka whakawhanake i ngā pūngāwerewere e whakaputa ana i te gastrin, tētahi homoni e whakapiki ana i te waikawa puku. Ka taea e tēnei te arahi ki ngā mate puku nui, te ngakau tahanga tonu, te korere, te mamae puku rānei e kore e pai te whakautu ki ngā maimoatanga noa.

Ki te uru te whekau pituitary, ka kite pea koe i ngā tohu penei i ngā mamae o te upoko tonu, ngā huringa tirohanga, ngā raruraru rānei ki te tipu me te whanaketanga ira tangata. Ko ētahi tāngata ka pā ki ngā wa o te marama koretake, te iti o te hiahia moepuku, te whakaputa miraka whakaongaonga rānei mai i ngā u.

I roto i ngā take onge, ka taea e te MEN-1 te pā ki ētahi atu wāhanga o tō tinana, te whakaeke i ngā tipu kiri, ngā pūngāwerewere pūkahukahu, ngā pūngāwerewere rānei i roto i ō whekau adrenal. Ko ēnei raruraru he iti te noho, engari ka puta pea i te whakawhānui o te mate.

He aha ngā take o te MEN-1?

Ko te MEN-1 te take o ngā rerekētanga i roto i te kiripuaki MEN1, e awhina ana i te aukati i ngā pūngāwerewere mai i te hanganga i roto i ō whekau endocrine. Ki te kore e mahi tika tēnei kiripuaki, ka taea e ngā pūtau i roto i ēnei whekau te tupu atu i te mana, te hanganga i ngā pūngāwerewere e tohu ana i tēnei mate.

Ko tēnei huringa ira tuku iho i te āhua e kiia ana e ngā tākuta he autosomal dominant. Ko te tikanga, kotahi noa te kape o te kiripuaki rerekē mai i tētahi mātua hei whakawhanake i te MEN-1. Ki te whai tētahi o ō mātua i te MEN-1, 50% te tūponotanga o te tuku iho o te mate.

I te 10% o ngā take, ka puta te MEN-1 hei rerekētanga hou, ko te tikanga kaore tētahi o ngā mātua e whai ana i te mate. Ko ēnei take e kiia ana he rerekētanga de novo, ā, ka puta ngāwari i te hanganga o ngā pūtau whakaputa uri, te whanaketanga wawe rānei.

Ko te kiripuaki MEN1 e whakaputa ana i te pūtau e kiia ana ko te menin, he rite ki te kaitiaki i roto i ō pūtau. Ka awhina te menin ki te whakahaere i te wehenga pūtau, te aukati i ngā pūtau mai i te tupu tere rawa. Ki te pakaru te kiripuaki, ka kore te mahi tiaki, ka tukuna te whakawhanaketanga pūngāwerewere i roto i ngā kiko e whakaputa ana i ngā homoni.

Nōhea te haere ki te taakuta mō te MEN-1?

Me whakapiri atu koe ki tō taakuta ki te pā ki a koe ngā kōhatu whatukuhu e hoki mai ana, ina koa ki te taitamariki koe, ki te hoki mai tonu rānei i te maimoatanga. Ko ngā kōhatu whatukuhu maha me ngā raruraru wheua, te ngenge tonu rānei ka tohu pea i te take me ō whekau parathyroid.

Rapua te tiaki hauora ki te whai koe i ngā wāhanga o te werawera, te wiri, te tere o te ngakau, te whakapohehe rānei e tohu ana pea i ngā hekenga kino o te huka toto. Ko ēnei tohu, ina koa ki te puta mai i te kore kai, me whakamātautau tonu.

Whakaritea he hui ki te whakawhanake koe i ngā mate puku e kore e ora ki te maimoatanga noa, ki te whai koe rānei i te ngakau tahanga tonu me te korere me te mamae o te puku. Ko tēnei huinga o ngā tohu ka tohu pea i ngā pūngāwerewere e whakaputa ana i te homoni i roto i tō pancreas.

Whakapiri atu ki tō kaiwhakarato hauora ki te pā ki a koe ngā mamae o te upoko tonu me ngā huringa tirohanga, i te mea ka tohu pea tēnei i te pūngāwerewere pituitary. Pēnei anō, ki te kite koe i ngā huringa whakaongaonga i ō wa o te marama, te hiahia moepuku, te whakaputa miraka u rānei, ko ēnei pea ngā tohu o te uru o te pituitary.

Ki te whai koe i te hītori whānau o te MEN-1, ngā āhuatanga e hono ana rānei, he mea nui kia kōrero ki tō taakuta mō te whakamātautau ira me te tirotiro, ahakoa kaore ano koe e whai tohu. Ka taea e te kite wawe te āwhina i te aukati i ngā raruraru me te arahi i te tirotiro tika.

He aha ngā take tūponotanga mō te MEN-1?

Ko te take tūponotanga matua mō te MEN-1 ko te whai i tētahi mātua e whai ana i te mate. Nā te mea e whai ana te MEN-1 i te tauira tuku iho autosomal dominant, 50% te tūponotanga o ngā tamariki o ngā mātua e pā ana ki te tuku iho o te rerekētanga ira.

Ko te whai i te hītori whānau o ngā pūngāwerewere e pā ana ki te homoni, ahakoa kaore i whakatauhia hei MEN-1, ka piki pea tō tūponotanga. I etahi wā kaore e kitea te mate i roto i ngā whānau mō ngā whakatupuranga, ina koa ki te ngawari ngā tohu, ki te tohu rānei ki ētahi atu take.

Kaore i rite ki ngā āhuatanga maha atu, kaore te MEN-1 e whai take tūponotanga e pā ana ki te noho. Kāore ō kai, ngā tikanga whakakori tinana, ngā whakaaturanga taiao, ngā whiringa whaiaro rānei e pā ana ki te mea ka whakawhanake koe i tēnei mate tuku iho.

Ka taea e te tau te pā ki te wa e puta mai ai ngā tohu, engari kāore e whakarereke tō tūponotanga ki te whai i te mate. Ko te nuinga o ngā tāngata e whai ana i te MEN-1 ka whakawhanake i ngā tohu i waenga i ngā tau 20 me te 40, ahakoa kaore pea ētahi e whakaatu i ngā tohu tae noa ki muri atu i te ora, ki te tamarikitanga rānei, he onge.

Kāore te ira tangata e pā nui ana ki tō tūponotanga ki te tuku iho o te MEN-1, ahakoa ka rereke pea te whakaatu o ētahi tohu i roto i ngā tāne me ngā wāhine nā ngā rerekētanga homoni.

He aha ngā raruraru pea o te MEN-1?

Ka taea e ngā raruraru o te MEN-1 te kino, ā, e hono ana ki ngā pānga roa o ngā koretake homoni, te noho tinana rānei o ngā pūngāwerewere e tupu ana. Ko te mārama ki ēnei raruraru pea ka āwhina i a koe me tō rōpū hauora ki te tiaki mō ngā raruraru.

Ko ngā taumata teitei o te kākā mai i ngā whekau parathyroid e mahi ana ka arahi pea ki ētahi raruraru awangawanga:

• Te osteoporosis nui me te piki o te tūponotanga whati
• Te mate whatukuhu roa mai i ngā kōhatu whatukuhu maha
• Ngā raruraru o te ngakau mai i ngā koretake kākā
• Te pouri nui, te ngoikore hinengaro
• Ngā mate puku ka taea te pakaru, te toto rānei

Ka taea e ngā pūngāwerewere Pancreatic te whakaeke i ngā hekenga o te huka toto e mate ai, ka arahi pea ki ngā hopu, te coma, te kino rānei o te roro ki te kore e maimoatia wawe. Ko ētahi pūngāwerewere pancreatic e whakaputa ana i te nui o te gastrin, te arahi ki ngā mate nui ka taea te pakaru i te puku, te whekau rānei.

Ka taea e ngā pūngāwerewere Pituitary te tupu nui ki te pēhi i ngā hanganga e tata ana, te whakaeke pea i te ngaro tirohanga tonu, ngā koretake homoni nui rānei. Ka taea hoki e ngā pūngāwerewere pituitary nui te whakaeke i te pēhanga nui i roto i tō anga, te arahi ki ngā mamae o te upoko tonu me ngā raruraru neurological.

Ahakoa ko te nuinga o ngā pūngāwerewere MEN-1 he pai, he iti te tūponotanga ka puta etahi hei mate pukupuku i te wa. Ko ngā pūngāwerewere Pancreatic te tūponotanga nui o te huringa kino, koia te take he mea nui te tirotiro tonu.

I roto i ngā take onge, ka taea e te MEN-1 te whakaeke i ngā pūngāwerewere i roto i ngā pūkahukahu, te thymus, ngā whekau adrenal rānei. Ka taea e ēnei raruraru onge te kino, ā, me whai ngā huarahi maimoatanga motuhake.

Me pēhea te whakatau i te MEN-1?

Ko te whakatau i te MEN-1 e whakauru ana i te whakamātautau hauora, ngā whakamātautau taiwhanga, ngā rangahau whakaahua, me te whakamātautau ira. Ka tīmata tō taakuta mā te arotake i ō tohu me tō hītori whānau ki te whakatau mena he tūponotanga te MEN-1.

He mea nui ngā whakamātautau toto mō te whakatau, ā, ko te nuinga e whakauru ana i te tirotiro i te kākā, te homoni parathyroid, me ngā homoni pancreatic maha. Ko te kākā teitei me ngā taumata homoni parathyroid teitei e whakarato ana i te tohu tuatahi pea kei te noho te MEN-1.

Ka tono pea tō taakuta i ētahi atu whakamātautau homoni, tae atu ki te gastrin, te insulin, te glucagon, me ngā homoni pituitary. Ka āwhina ēnei whakamātautau ki te whakatau i tehea whekau e pā ana, me te nui o te whakaputa homoni.

Ka āwhina ngā rangahau whakaahua ki te kimi i ngā pūngāwerewere me te aromatawai i ō raatau rahi. Ka taea e koe te whai i ngā tātaritanga CT, ngā tātaritanga MRI, ngā tātaritanga rongoā pūtau rānei e taea te kite i ngā pūngāwerewere iti e whakaputa ana i te homoni kaore pea e puta i ngā whakaahua noa.

Ka taea e te whakamātautau ira te whakau i te whakatau mā te kimi i ngā rerekētanga i roto i te kiripuaki MEN1. He mea nui tēnei whakamātautau mō ngā mema whānau e hiahia ana ki te mohio mena kei a rātou te rerekētanga ira, ahakoa kaore ano rātou e whai tohu.

Ko te whakatau e whakautia ana i te nuinga ina whai koe i ngā pūngāwerewere i roto i te iti rawa e rua o ngā whekau whāinga matua e toru (parathyroid, pancreas, pituitary rānei), ki te whakaatu rānei te whakamātautau ira i te rerekētanga kiripuaki MEN1.

He aha te maimoatanga mō te MEN-1?

Ko te maimoatanga mō te MEN-1 e aro ana ki te whakahaere i ngā koretake homoni me te whakautu i ngā pūngāwerewere takitahi i te mea ka tupu. Nā te mea he mate ora tonu tēnei, ka whakawhanake pea tō mahere maimoatanga i te wa e puta mai ana ngā pūngāwerewere hou, te tupu rānei o ngā mea o mua.

Mō ngā whekau parathyroid e mahi ana, ko te pokanga te maimoatanga pai. Ko tō taote pokanga e tango ana i te toru me te hawhe o ō whekau parathyroid e wha, te waiho i te kiko nui hei pupuri i ngā taumata kākā noa i te aukati i te mate mai i te hoki mai wawe.

Ko ngā pūngāwerewere Pancreatic e hiahia ana ki te maimoatanga takitahi e whakawhirinaki ana ki ō rātou rahi, te wāhi, me te whakaputanga homoni. Ko ngā pūngāwerewere iti, kore mahi pea ka tirohia noa, engari ko ngā pūngāwerewere nui ake, te whakaputa homoni rānei e hiahia ana ki te tango pokanga.

Ki te whai koe i ngā pūngāwerewere e whakaputa ana i te insulin e whakaeke ana i te huka toto iti rawa, ka tono pea tō taakuta i ngā rongoā penei i te diazoxide hei āwhina i te pupuri i ngā taumata huka toto pumau. Mō ngā pūngāwerewere e whakaputa ana i te gastrin, ka taea e ngā kaihoroi waikawa proton te āwhina i te whakahaere i te whakaputanga waikawa puku.

Ko ngā pūngāwerewere Pituitary e maimoatia ana i te nuinga me ngā rongoā ka taea te whakaiti i ētahi momo pūngāwerewere, te aukati rānei i ō rātou whakaputanga homoni. Ka hiahiatia pea te pokanga mō ngā pūngāwerewere nui ake, ngā mea rānei e kore e whakautu ki te rongoā.

Ko tō rōpū maimoatanga pea he maha ngā tohunga, tae atu ki ngā tohunga endocrinology, ngā taote pokanga, ngā tohunga mate pukupuku rānei. Ko te tirotiro tonu me ngā whakamātautau toto me ngā whakaahua ka āwhina i te hopu i ngā raruraru hou i te wa e ngāwari ana te maimoatanga.

Me pēhea te whakahaere i te MEN-1 i te kāinga?

Ko te noho me te MEN-1 e hiahia ana ki te aro tonu ki tō hauora, engari he maha ngā mea ka taea e koe te mahi i te kāinga hei tautoko i tō oranga pai me te mahi tahi pai me tō rōpū hauora.

Pupuri i ngā rekoata taipitopito o ō tohu, tae atu ki te wa e puta mai ai, ō rātou kaha, me ētahi atu whakaohooho pea. Ka āwhina tēnei mōhiohio i ō tākuta ki te whakarite i tō mahere maimoatanga me te hopu i ngā raruraru hou i te wawe.

Ki te whai koe i ngā raruraru e pā ana ki te kākā, kia pai te inu wai mā te inu i te nui o te wai i te rā katoa. Ka āwhina tēnei ki te aukati i ngā kōhatu whatukuhu me te tautoko i tō mahi whatukuhu. Kaua e inu nui rawa i ngā taapiri kākā ki te kore e tūtohutia motuhake e tō taakuta.

Mō te whakahaere huka toto, kai i ngā kai noa, rite, ā, mau i ngā papa huka, ngā kai rānei ki te pā ki a koe ngā wāhanga o te huka toto iti. Ako ki te mohio ki ngā tohu whakatūpato o te hypoglycemia kia taea ai e koe te maimoatanga wawe.

Pupuri i te hauora wheua pai mā te whakakori tinana noa, ā, kia nui te inu hua D. Engari, kōrerohia ngā taapiri katoa me tō rōpū hauora, i te mea ka rereke pea ō hiahia kākā mai i te iwi whānui.

Hangaia tētahi kāri mōhiohio hauora e whakarārangi ana i tō mate, ngā rongoā o nāianei, me ngā hononga āwhina. Pupurihia tēnei i ngā wā katoa, i te mea he mōhiohio nui pea tēnei mō ngā kaiwhakarato hauora ki te hiahia koe ki te tiaki āwhina.

Me pēhea te whakarite mō tō hui taakuta?

Ko te whakarite pai mō ō hui ka āwhina i te whakarite kia whiwhi koe i te nuinga mai i tō wā me tō rōpū hauora, ā, kaua e wareware i ngā pātai nui, ngā awangawanga rānei.

Tuhia ō tohu katoa, tae atu ki te wa i tīmata ai, te maha o te puta, me te mea e pai ake ai, te kino rānei. Whakauruhia ngā tohu kaore pea e rite, i te mea ka taea e te MEN-1 te whakaeke i ngā raruraru maha puta noa i tō tinana.

Mau mai he rārangi katoa o ngā rongoā, ngā taapiri, me ngā huaora katoa e inu ana koe, tae atu ki ngā rāpoi me te maha o te inu. Koinei ngā rongoā kore-tūtohu, i te mea ka taea e ētahi te pā ki ngā maimoatanga MEN-1.

Kohikohia tō hītori hauora whānau, ina koa te aro ki ngā whānau i whai raruraru homoni, ngā pūngāwerewere whakaongaonga, ngā kōhatu whatukuhu, ngā mate puku rānei. Ka taea e tēnei mōhiohio te nui mō te whakatau me te mahere maimoatanga.

Whakaritea he rārangi o ngā pātai mō tō mate, ngā whiringa maimoatanga, me te mea e tatari ana koe ki te haere. Kaua e manukanuka ki te maha o ngā pātai - e hiahia ana tō rōpū hauora kia mārama koe ki tō mate.

Whakaarohia te kawe mai i tētahi mema whānau whakawhirinaki, hoa rānei ki tō hui. Ka taea e rātou te āwhina i a koe ki te mahara ki ngā mōhiohio nui me te whakarato tautoko ngākau i te wā ka taea te whakapae i ngā kōrero mō tō hauora.

He aha te mea nui mō te MEN-1?

Ko te MEN-1 he mate tuku iho uaua engari ka taea te whakahaere e pā ana ki ngā whekau e whakaputa ana i ngā homoni puta noa i tō tinana. Ahakoa e hiahia ana ki te tirotiro me te maimoatanga ora tonu, he tokomaha ngā tāngata e whai ana i te MEN-1 e ora ana i ngā ora, ngā ora mahi me te tiaki hauora tika.

Ko te whakatau wawe me te whai tonu i te whai i te hauora he mea nui mō te aukati i ngā raruraru kino. Ko te mea nui ko te mahi tata atu ki tō rōpū hauora ki te tirotiro mō ngā pūngāwerewere hou me te whakahaere pai i ngā mea o mua.

Ki te whai koe i te MEN-1, ki te whakaaro rānei koe pea, kia mahara ko tēnei mate e pā ana ki te katoa. Kāore pea tō wheako e rite ki te mea e pānui ana koe mō ētahi atu, ā, he mea noa tēnā.

Ko te whai MEN-1 te tikanga ka hiahia koe ki te nui atu o te tiaki hauora i te nuinga o ngā tāngata, engari kāore e whakatau i tō ora. Me te maimoatanga tika me te tirotiro, ka taea e koe te whakahaere i ō tohu me te pupuri i tō kounga ora.

Ngā pātai e ui pinepinetia ana mō te MEN-1

P.1: He tuku iho tonu te MEN-1 mai i ngā mātua?

Ko te 90% o ngā take MEN-1 e tuku iho ana mai i tētahi mātua e whai ana i te mate. Engari, ko te 10% o ngā take ka puta hei rerekētanga hou, ko te tikanga kaore tētahi o ngā mātua e whai ana i te MEN-1. Ki te whai koe i te MEN-1, 50% te tūponotanga o ia tamaiti ki te tuku iho o te rerekētanga ira.

P.2: Ka taea e ngā pūngāwerewere MEN-1 te riro hei mate pukupuku?

Ko te nuinga o ngā pūngāwerewere MEN-1 e noho pai ana i roto i tō oranga, engari he iti te tūponotanga o te huringa kino, ina koa ki ngā pūngāwerewere pancreatic. Koia te take he mea nui te tirotiro tonu me te whai i te tiaki. Ko te kite wawe i ngā huringa ka tukuna te maimoatanga wawe ki te hiahiatia.

P.3: Me pehea te maha o te tirotiro ki te whai ahau i te MEN-1?

Ko te nuinga o ngā tāngata e whai ana i te MEN-1 e hiahia ana ki ngā tirotiro tau, rua tau rānei e whakauru ana i ngā whakamātautau toto me ngā rangahau whakaahua. Ko te auau tika e whakawhirinaki ana ki ō tohu o nāianei, ngā whekau e pā ana, me te pumau o tō mate. Ka hanga e tō rōpū hauora tētahi mahere tirotiro motuhake mōu.

P.4: Ka taea e ngā huringa kai, noho rānei te āwhina i te whakahaere i te MEN-1?

Ahakoa kaore e taea e ngā huringa noho te whakaora i te MEN-1, te aukati rānei i te whakawhanaketanga pūngāwerewere, ka taea e rātou te āwhina i te whakahaere i ngā tohu me te tautoko i tō hauora whānui. Ko te noho makuku ka āwhina i te aukati i ngā kōhatu whatukuhu, ko ngā kai noa ka āwhina i te whakahaere huka toto, ko te whakakori tika ka tautoko i te hauora wheua. Engari, ko te maimoatanga hauora tonu te huarahi matua.

P.5: Me whakamātautauhia aku mema whānau mō te MEN-1?

Ki te whakau koe i te MEN-1, me kōrero ō whānau tuatahi (ngā mātua, ngā teina, me ngā tamariki) ki ō rātou kaiwhakarato hauora mō te whakamātautau ira. Ahakoa kaore rātou e whai tohu, ko te mohio ki ō rātou tūāhua ira ka tukuna te tirotiro tika me te whakauru wawe ki te hiahiatia. Ka taea e te tohutohu ira te āwhina i ngā whānau ki te whakatau whakamārama mō te whakamātautau.