Kāne 2

Te ngā mate pūtau maha-pūngāwerewere, momo 2, e kiia ana hoki ko MEN 2, he āhuatanga onge. Ka pā te mate ki ngā pūngāwerewere i roto i ngā whekau tairoirī me ngā whekau pararoid, ngā whekau adrenal, ngutu, waha, kanohi me te pūnaha kai. Ka taea e te whakamātautau ira te kimi i te ira i hurihia e pā ana ki te MEN 2. Ka taea e ngā kaiwhakarato hauora te whakaora i ngā raruraru hauora e pā ana ki taua ira.

He mate whakatūturu te MEN 2. Ko te tikanga tenei, ko te hunga kei a rātou te ira i hurihia ka taea te tuku atu ki ō rātou tamariki. He 50% te tūponotanga o ia tamaiti ki te whiwhi i te mate.

E rua ngā momo o te MEN 2:

• MEN 2A. E mohiotia ana hoki tenei ko te MEN 2A tuku iho, ko te mate Sipple rānei. Ka pā te mate ki te mate pūngāwerewere tairoirī me ngā pūngāwerewere kore-mate o ngā whekau pararoid me ngā whekau adrenal.
• MEN 2B. He onge tenei momo MEN 2. Ka pā te mate ki te mate pūngāwerewere tairoirī, ngā pūngāwerewere kore-mate o te whekau adrenal, me ngā pūngāwerewere kore-mate i runga i ngā ngutu, i runga i te arero, me te pūnaha kai. Kāore te MEN 2B e pā ki ngā raruraru ki ngā whekau pararoid.

Ko ngā tohu o te MEN 2 e whakahaeretia ana e te momo pūngāwerewere. Ko te hunga he MEN 2B kei a rātou te ahua motuhake. Ka taea e rātou te pupuhi i te arero, ngutu me ngā kanohi. Ka nui ake te teitei me te angiangi me ngā ringa me ngā waewae roa. Ko ngā tohu e whai ake nei ka hono pea ki ia momo pūngāwerewere. Te mate pukupuku thyroid medullary: Nga pupuhi i te korokoro, i te kaki Raru te manawa, te horomia Te reo whakarere Te ruaki Te hyperplasia parathyroid, e mohiotia ana ko te hyperparathyroidism matua: Te mamae o te uaua me te hononga Te ruaki Te ngenge Nga raruraru mahara Nga kohatu whatukuhu Nga pūngāwerewere adrenal, e mohiotia ana ko te pheochromocytoma: Te toto teitei Te tere o te ngakau Te awangawanga Nga mamae o te mahunga Ka puta mai pea nga tohu na te pūngāwerewere thyroid e pēhi ana i ngā kiko i te taha, i te tuku nui rawa o ngā homoni ki te tinana. Kaore pea etahi o te hunga he mate pukupuku thyroid medullary e whai tohu. Ki te whai koe i tetahi o enei tohu, whakapiri atu ki tō kaiwhakarato hauora.

Mēnā kei te whai koe i ētahi o ēnei tohu, tēnā whakapā atu ki tō kaiwhakarato hauora.

Ko te MEN 2 he āhuatanga kua oti te whakawhiwhi. Ko te tikanga tenei, ka taea e te tangata he huringa kirihou ka taea te whakaohooho i te MEN 2 te tuku i taua kirihou ki ōna tamariki.

He tokomaha hoki ka tū hei te tangata tuatahi i roto i ō rātou whānau ki te whiwhi i tēnei mate. Ka tirotirohia ngā tāngata kua tohua he mate kōrearea tiroida medullary mō te MEN 2.

Kaore he uauatanga

Ka taea e te MEN 2 te whakaatu i ngā kōpū parathyroid ki te tuku i te nui o te kōkalcium ki roto i te toto. E mohiotia ana tenei ko te hyperparathyroidism matua. Kei te kaki ngā kōpū parathyroid. Ka taea e te kōkalcium atu i roto i te toto te whakaatu i ngā raruraru maha, tae atu ki ngā wheua ngoikore, e kiia nei ko te osteoporosis, ngā kōhatu whatukuhu me te maha o te mimi. Ka puta te mate pukupuku thyroid medullary hei pupū i runga i te thyroid, i te kaki rānei. He uaua pea te horomia ina nui te mate pukupuku, atu rānei ngā tohu mēnā ka horapa te mate pukupuku ki waho o te kaki. Ka taea hoki e ngā tāngata e whai ana i te MEN 2 te whai i te āhuatanga e kiia nei ko te pheochromocytoma. Ka whakaatu tenei āhuatanga i ngā mate pukupuku kore-mate pukupuku i runga i te kōpū adrenal. Kei runga i ngā whatukuhu ngā kōpū adrenal. Ka taea e ēnei mate pukupuku te tuku i ngā homoni e whakaatu ana i te toto teitei, te werawera me ētahi atu tohu.

Ka whakamahia te whakamātautau ira ki te kimi mena kei te tangata te huringa ira e pā ana ki te MEN 2. Ko ngā tamariki o te tangata kei a rātou tēnei huringa ira ka taea te whiwhi mai i te ira huringa, ka whanake i te MEN 2. Ka taea hoki e ngā mātua me ngā teina te whiwhi i te ira huringa ahakoa kahore rātou e whai tohu. Ki te kitea tetahi o tō whānau he MEN 2, ka tūtohu pea tō kaiwhakarato hauora kia whakamātautau ira koe me ō whānau. Koinei i te mea ka taea te whakaora, te whakahaere rānei i te MEN 2 mā te tango i te kohanga tairoirō i te timatanga o te ora. Ko te tirotiro hoki mō ngā pūngāwerewere parathyroid, adrenal rānei ka āwhina. Ki te kore e kitea he huringa ira i roto i ngā mema o te whānau, kaore e hiahiatia ngā whakamātautau tirotiro atu. Heoi, kaore te whakamātautau ira e kite i ngā huringa ira MEN 2 katoa. Ki te kore e kitea te MEN 2 i roto i te hunga pea kei a rātou, ka whakamātautau toto me ngā whakaahua rātou me ō rātou whānau i te wa roa ki te tirotiro i ngā tohu o te mate.

Hei whakatau i te maha o ngā mate pūngao, momo 2, e kiia ana hoki ko MEN 2, ka mahi to kaiwhakarato hauora i tetahi whakamātau tinana. Ka titiro rātou ki tō hītori hauora me tō hītori whānau. Ka mahi hoki rātou i te whakamātau ira ki te kite mena kei a koe tetahi huringa ira e pā ana ki te MEN 2. Ka taea te mahi i ngā whakamātau toto me te mimi me ngā whakamātau whakaahua. Ka uru pea ēnei ki:

• Ngā taumata karitonini i roto i te toto
• Te konupūmā toto
• Ngā taumata homoni parathyroid
• Te whakamātau mimi, rānei, plasma mō ngā catecholamines me ngā metanephrines
• Te whakaahua pūmanawa (MRI)
• Te karapa tomokōwhiwhi rorohiko (CT)
• Te karapa ultrasound

I roto i te MEN 2, ka taea e ngā pūngāwerewere te tipu ki runga i te tairoirī, te paratairoiti, me ngā whatukuhu adrenal. Ka taea e ēnei pūngāwerewere te arahi ki ngā āhuatanga rerekē, ka taea katoa te whakaora. Ka uru pea ēnei āhuatanga me ngā rongoā ki:

• Te mate pukupuku tairoirī medullary. Ka whakaorangia te mate pukupuku tairoirī, tae atu ki te mate pukupuku kua horapa ki ētahi atu wāhanga o te tinana, mā te tango i te tairoirī me ngā ngota lymph i te taha o reira mā te pokanga. Ka taea hoki te whakamahi i ngā rongoā mena kua horapa te mate, ā, kāore e taea te tango mā te pokanga.
• Te whānui o te paratairoiti. Ka taea te whānui o ngā whatukuhu paratairoiti, ā, ka nui rawa te homoni paratairoiti e hangaia ana. Ko te rongoā noa ko te pokanga hei tango i te katoa, i tetahi wāhanga rānei o ngā whatukuhu paratairoiti kua whānui, me te waiho i ngā whatukuhu kāore i pā.
• Ngā pūngāwerewere adrenal. Mō ēnei momo pūngāwerewere, ka tūtohu pea tō kaiwhakarato hauora ki te tango i tetahi, i ngā rua rānei o ngā whatukuhu adrenal, kei te whakaritea ki te mea kei te whakaatuhia i runga i te CT, i te MRI rānei.

Kaore he uauatanga