Me kōrero tāua mō te pukupuku onge e kiia nei ko te Pheochromocytoma i tēnei rā?

He toto toto tiketike ohorere tōu i ētahi wā? He werawera noa iho rānei? He ānini tōu me te pakū o te ngākau? Kaua e hohoro te whakaaro he mate kino ētahi o ēnei mea. Heoi, ki te mau tonu ēnei tohu, inā koa he toto toto tiketike e uaua ana ki te whakahaere, he mea pai kia āwangawanga. I tēnei rā ka kōrero tātou mō tētahi mate he onge noa iho, engari ki te tika te tātaritanga, ka taea te rongoā pai. Ko te pheochromocytoma te ingoa.

He aha te Pheochromocytoma? Kia mārama tātou.

I te whakarapopototanga, he pukupuku te pheochromocytoma e tupu ana i te wāhanga waenga o ngā repe adrenal, arā, te adrenal medulla. Ka hangaia ēnei pukupuku e tētahi momo pūtau motuhake e kiia nei ko ngā pūtau chromaffin. Ka whakaputa, ka tuku hoki ēnei pūtau i ngā homoni ki roto i te toto e tika ana mō te urupare "whawhai, rere rānei" a tō tātou tinana. Whakaarohia, ina mataku ohorere koe, ina pāngia rānei koe e te ahotea nui, ko ngā huringa ka puta i roto i tō tinana - ka piki ake te tere o tō ngakau, ka werawera koe, ka wiri tō tinana - ko ēnei homoni te take o tēnei.

I te nuinga o te wā ka puta te pheochromocytoma i roto i te repe adrenal kotahi anake. Heoi, i ētahi wā ka puta i roto i ngā repe e rua. Tērā pea he nui atu i te kotahi te pukupuku i roto i te repe kotahi.

He mea nui, he pai te nuinga o ngā pheochromocytoma (ehara i te mea kino) . Ko te tikanga kāore e horapa ki ētahi atu wāhanga o te tinana. Heoi, i waenga i te 10% me te 15% ka taea te mate pukupuku. Mena he mate pukupuku tēnei, ka whakaahuahia i roto i ētahi wāhanga matua, i runga i te horapa:

• Pheochromocytoma ā-rohe: Kei roto i te kotahi, i ngā repe adrenal e rua rānei te pukupuku.
• Pheochromocytoma rohe: Kua horapa te mate pukupuku ki ngā kōpū lymph e tata ana ki ngā repe adrenal, ki ētahi atu kiko rānei.
• Pheochromocytoma metastatic: Kua horapa te mate pukupuku ki ngā whekau tawhiti pērā i te ate, ngā pukahukahu, ngā wheua rānei.
• Pheochromocytoma auau: Kua hoki mai te mate pukupuku i muri i te maimoatanga. Tērā pea kei te wāhi kotahi i mua, kei tētahi atu wāhi rānei.

He aha ēnei repe adrenal i roto i tō tātou tinana? He aha tā rātou mahi?

E rua ā tātou repe adrenal. Kei muri o tō tātou puku ēnei, kei runga ake i ō tātou whatukuhu, he rite ki te taupoki. He wāhanga ēnei o tō tātou pūnaha endocrine. E rua ngā wāhanga matua o ia repe adrenal. Ko te paparanga o waho e kiia ana ko te adrenal cortex, ā, ko te wāhanga o waenganui e kiia ana ko te adrenal medulla.

Ka whakaputahia e tō tātou roro adrenal tētahi rōpū homoni e kiia nei ko ngā catecholamines. Ka whakahaeretia e ēnei homoni ētahi tukanga tino nui i roto i tō tātou tinana:

• Te tere o te manawa
• Te toto totoka
• Te taumata huka toto `(Te huka toto)`
• Te urupare a te tinana ki te ahotea (te urupare "whawhai, rere rānei")

Ko ngā momo matua o ngā catecholamines ko:

• Dopamine
• Epinephrine (Adrenaline) `(Epinephrine / Adrenaline)`
• Norepinephrine (Noradrenaline) `(Norepinephrine / Noradrenaline)`

Ina kitea he pheochromocytoma, ka taea e ia te tuku i te nui atu o ēnei homoni, te adrenaline me te noradrenaline, ki roto i te toto i te mea e tika ana. Ko te wā tēnā ka tīmata te puta mai o ngā tohu rerekē.

He aha te rerekētanga i waenga i te Pheochromocytoma me te Paraganglioma?

He momo pukupuku tino ōrite, he onge ēnei. Ka puta mai ngā pūtau chromaffin kotahi i kōrerotia i mua ake nei. Heoi, ka puta te pheochromocytoma i waenganui o te repe adrenal (te adrenal medulla), ko te paraganglioma ia ka puta mai i ētahi atu wāhi i waho o te repe adrenal .

Ko wai te mea tino tūpono ka pāngia e tēnei mate? He pēhea te noa o tōna pānga?

Ka taea te whakawhanake i te pheochromocytoma i ngā tau katoa, engari ko te nuinga o te wā ka pā ki te hunga i waenga i te 30 me te 50 tau te pakeke. Tata ki te 10% o ngā keehi ka puta i te tamarikitanga.

He pukupuku tino onge tēnei. He uaua ki te kī tika e hia ngā tāngata e pāngia ana e tēnei mate. Nā te mea kāore ētahi tāngata e whakaatu i ngā tohu, tērā pea kāore e kitea te mate. E kiia ana he iti iho i te 1% o te hunga he toto toto tiketike tō rātou he pheochromocytoma.

He aha ngā tohu e whakaatuhia ana e te tangata whai pheochromocytoma?

Ka puta ngā tohu o te pheochromocytoma ina tukuna e te pukupuku he nui rawa o ngā homoni adrenaline (epinephrine) me te noradrenaline (norepinephrine) ki roto i te toto. Heoi, kāore ētahi pukupuku e whakaputa i te nui rawa o ēnei homoni, ā, kāore pea he tohu o te mate.

Ko ngā tohu e kitea whānuitia ana ko:

• Te toto toto tiketike (hypertension): Koinei te tohu matua. I ētahi wā he uaua ki te whakahaere.
• Te ānini o te upoko: Ka pā mai pea he ānini nui, ohorere hoki.
• Te werawera nui, kāore he take.
• Ko ngā palpitations he kare ā-roto o te tere o te ngakau, te koretake, te patupatu rānei.
• Te āhua e wiri ana tō tinana.

Ngā tohu kāore i te tino kitea:

• Mamae o te uma me/ranei te puku.
• Ka huri whakarere te kiri hei kiri mā ake i te āhua noa.
• Whakapairuaki me/ranei te ruaki.
• Te korere.
• Te mate kōroke.
• Ko te ngoikore o te toto (orthostatic hypotension) he hekenga ohorere o te toto toto ina tū koe. Ko te tikanga tēnei ka pōuri koe ina tū ohorere koe mai i te tūnga noho.
• Te ngaronga taumaha mō te kore take.

Tērā pea ka puta mai ēnei tohu, ka kino atu rānei i muri i ētahi huihuinga. Hei tauira:

• Te korikori tinana kaha.
• Te whara tinana, te ahotea hinengaro nui rānei.
• Te whānautanga.
• Te whiwhi rongoā whakamoe.
• Te mahi pokanga.
• Te kai i ngā kai he nui te tyramine (hei tauira, te waina whero, te tiakarete, te tiihi).

Kei te noho tonu ngā tohu? Kāore rānei, ka haere mai, ka haere atu?

Tērā pea ka roa te toto toto tiketike o te tangata e pangia ana e te pheochromocytoma, ka haere mai, ka memeha atu rānei .

Tērā pea ka pāngia ētahi tāngata e ngā "whakaekenga paroxysmal," arā, ngā whakaekenga ohorere o ngā tohu. Ko te tikanga o tēnei he pikinga ohorere o te toto toto, me ngā tohu pēnei i te mamae nui o te upoko, te tere o te manawa, me te werawera nui. Ka taea e ēnei "whakaekenga" te puta i ētahi wā i te rā, i te kotahi, i te rua rānei i te marama.

He mea nui: Mena kei a koe ētahi o ēnei tohu, inā koa te toto toto tiketike ohorere, te ānini, te werawera, me te tere o te pā o te manawa, he mea tino nui kia rapu tohutohu hauora.

He aha te take e puta ai te pheochromocytoma? He aha ngā take?

I te nuinga o te wā, kāore e kitea he tino take mō te pheochromocytoma. Ka puta noa mai.

Heoi, i roto i te 25% ki te 35% o ngā take, e pā ana tēnei āhuatanga ki ngā āhuatanga tuku iho. Ko ētahi o ngā mea matua ko:

• Te mate pukupuku endocrine maha 2, momo A me te B (MEN2A me te MEN2B)
• Te mate a Von Hippel-Lindau (VHL)
• Neurofibromatosis momo 1 (NF1)
• Te mate paraganglioma tuku iho
• Te takirua o Carney-Stratakis [paraganglioma me te pukupuku stromal gastrointestinal (GIST)]
• Te toru o Carney (paraganglioma, GIST me te chondroma pūkahukahu)

Haunga ēnei āhuatanga ira, ka taea hoki te whakawhanake i te pheochromocytoma nā ngā whakarerekētanga ira i roto i te iti rawa 10 ngā ira rerekē.

Me pēhea te tautuhi i tēnei mate? (Tautuhinga)

He uaua te tautuhi i te pheochromocytoma nā te mea he pukupuku onge, ā, i ētahi wā kāore he tohu e puta. I ētahi wā, ka kitea te pukupuku mā te whakamātautau mō tētahi atu take.

He maha ngā take e whakapae ai te tākuta kei a koe tēnei mate:

• NāuHe hītori hauora taipitopito, inā koa mēnā kua pāngia tētahi o te whānau e te pheochromocytoma i mua.
• He whakamātautau tinana me te hauora katoa.
• Ko ētahi tohu motuhake , hei tauira ko ngā "whakaekenga paroxysmal" i kōrerohia e tātou me te toto toto tiketike kāore e taea te whakahaere e ngā maimoatanga noa.

He aha ngā momo whakamātautau e mahia ana?

Ka tūtohu pea tō tākuta i ngā whakamātautau e whai ake nei hei whakaū mēnā kei a koe te pheochromocytoma:

• Whakamātautau mimi 24-hāora: Ko te kohikohi i tō mimi i te roanga o te rā, me te ine i te nui o ngā catecholamine kei roto. Ka ine hoki i ngā matū ka puta ina pakaru ēnei homoni. Mena he teitei ake ēnei taumata i te tikanga, he tohu pea tēnei nō te pheochromocytoma.
• Ngā whakamātautau catecholamine toto: Ka ine ēnei i ngā taumata o ngā catecholamine i roto i te toto. Pērā i ngā whakamātautau mimi, ka rapua hoki ngā matū i hangaia e te pakaru o ngā homoni.
• Matawai CT (Matawai Tomography Rorohiko): Ka hiahiatia he raupapa hihi-X hei hanga pikitia taipitopito o roto o tō tinana. Tērā pea ka tūtohu tō tākuta i tēnei hei tirotiro i ō repe adrenal.
• Matawai MRI (MRI - Magnetic Resonance Imaging): Ka whakamahia he aukume, he ngaru reo irirangi, me te rorohiko hei hanga pikitia taipitopito o roto o te tinana. Ka whakamahia hoki hei tirotiro i ngā repe adrenal.

Kia whakaūtia te mate, ka mahia pea ētahi atu whakamātautau hei whakatau mēnā he mate pukupuku (kino) he pai rānei (pai), ā, mēnā kua horapa ki ētahi atu wāhanga o te tinana.

He aha te hiranga o te whakamātautau ira?

Mena ka tāpaetia koe he pheochromocytoma, ka tūtohu pea tō tākuta i tētahi tohutohu ira me te whakamātautau hei whakatau mena he mate tuku iho tōu e tūpono ai koe ki te whakawhanake i ētahi atu mate pukupuku.

Tērā pea ka tūtohuhia he whakamātautau ira i roto i ēnei take:

• Mena kei a koe, kei tētahi rānei o tō whānau ngā tohu e pā ana ki te pheochromocytoma ira tuku iho, te paraganglioma rānei.
• Mena he pukupuku kei roto i ō repe adrenal e rua.
• Mena he nui atu i te kotahi te pukupuku i roto i te repe adrenal kotahi.
• Mena he tohu kei te piki ake ngā taumata o ngā catecholamines i roto i te toto.
• Mena ka kitea te pheochromocytoma i mua i te 40 tau te pakeke.

Mena ka kitea he huringa ira i roto i te whakamātautau ira, ka tūtohu pea te kaitohutohu ira kia whakahaerehia hoki tēnei whakamātautau e ētahi atu mema o tō whānau (te hunga kāore he tohu engari kei te mōrearea).

He aha ngā maimoatanga mō te Pheochromocytoma?

Ko te maimoatanga pai rawa atu mō tēnei ko te tango i te pukupuku mā te pokanga, ki te taea .

Ko te whiriwhiri i te maimoatanga e whakawhirinaki ana ki etahi mea:

• Te rahi o te pukupuku.
• Ahakoa he mate pukupuku (kino) te pukupuku, he pai rānei (pai).
• Mēnā he tohu kei te puta mai i te pikinga o ngā homoni catecholamine.
• Kei te kotahi anake te wāhi o te pukupuku, kua horapa rānei ki ētahi atu wāhanga o te tinana? (Kua horapa ki te toto)
• Ahakoa i kitea tuatahitia te mate, kua hoki mai rānei i muri i te maimoatanga o mua.

Mena kei a koe ngā tohumate nā te pikinga o ngā homoni adrenal, ka tohua pea e tō tākuta he rongoā hei whakahaere i aua tohumate. Hei tauira:

• Ngā rongoā hei pupuri i te toto toto ki te taumata noa, pērā i ngā aukati-āra.
• Ngā rongoā hei pupuri i te tere o te manawa ki te taumata noa, pērā i ngā beta-blockers.
• Ngā rongoā e aukati ana i ngā pānga o ngā homoni e tukuna ana e te repe adrenal.

Ko ngā huarahi matua ki te rongoā i te pheochromocytoma ko:

• Pokanga
• Te rongoā irahiko
• Te rongoā matū
• Te rongoā whakaweti
• Te rongoā whakapūkara
• Te rongoā arotahi

Māu me tō tīma hauora e whakatau te mahere maimoatanga e tika ana māu.

Ngā tikanga maimoatanga matua

• Pokanga:

Koinei te maimoatanga matua mō te pheochromocytoma. Tata ki te 90% o ngā pukupuku ka taea te tango angitu mā te pokanga .

Tērā pea ka tūtohu tō tākuta i tētahi pokanga hei tango i tētahi, i ngā repe adrenal rānei e rua (adrenalectomy). I te wā o te pokanga, ka tirotirohia e te taote pokanga ngā kiko e karapoti ana me ngā kōpū lymph kia kite ai mēnā kua horapa te pukupuku. Mēnā kua horapa, ka tangohia taua kiko, ki te taea.

I muri i te pokanga, ka whakamātauria ō toto, ō mimi rānei mō ngā taumata catecholamine. Mēnā ka hoki anō ngā taumata ki te āhua noa, ko te tikanga kua tangohia katoatia ngā pūtau pheochromocytoma.

Ki te tangohia ngā repe adrenal e rua, me tango e koe te rongoā homoni mō te toenga o tō oranga hei whakakapi i ngā homoni i hangaia e ngā repe adrenal.

• Te rongoā irahiko:

He maimoatanga tēnei e whakamahia ana hei patu i ngā pūtau matepukupuku, hei aukati rānei i tō rātou tipu. Ka mahia tēnei i roto i tētahi huarahi e kore ai e tino kino ki ngā kiko hauora.

E rua ngā momo rongoā irahiko:

• Te rongoā irahiko o waho: Ka tukuna te irahiko ki te wāhi matepukupuku mai i tētahi mīhini i waho o te tinana.
• Te rongoā irahiko ā-roto: Ka tākaihia he matū irahiko ki roto i ngā ngira, ngā kākano, ngā waea, ngā katitere rānei, ā, ka whakaurua e te tākuta ki roto, ki te taha rānei o te wāhi o te mate pukupuku.

Ko te momo rongoā irahiko e whakawhirinaki ana ki te mea kei te rohe te mate pukupuku, kei te rohe, kei te horapa, kei te hoki anō rānei. Ko te pheochromocytoma kino ka rongoatia i te nuinga o te wā mā te rongoā irahiko hihi o waho me/ranei te rongoā 131I-MIBG. Ko te 131I-MIBG he matū irahiko e piri ana ki ētahi pūtau mate pukupuku ka patu i a rātou.

• Te rongoā matū:

Ka whakamahia ngā rongoā hei patu i ngā pūtau matepukupuku, hei aukati i a rātou kia kaua e wehewehe me te whakanui, hei aukati rānei i te tipu o te matepukupuku. Ko te tikanga ka hoatu ēnei rongoā mā roto i te uaua. Ahakoa he maimoatanga angitu tēnei, ka pā mai pea ngā pānga taha.

• Te rongoā whakaweto:

He maimoatanga iti noa te whakaekenga. Ka whakamahia te wera nui, te makariri nui rānei hei whakangaro i ngā pukupuku.

• Te tango i ngā pūtau matepukupuku me ngā pūtau rerekē: Ka whakamahia e te tango i ngā pūtau matepukupuku me ngā pūtau rerekē ngā ngaru reo irirangi hei whakamahana me te whakangaro i ngā pūtau matepukupuku me ngā pūtau rerekē.
• Cryoablation: Ka whakamahia te hauota wai, te hauhā waro wai rānei hei whakatio me te whakangaro i ngā pūtau matepukupuku me ngā pūtau rerekē.

• Te rongoā whakapūkara:

I roto i tēnei, ka āraia te uaua e tuku toto ana ki te repe adrenal. Ka aukati i te rere o te toto ki te repe, ka mate ngā pūtau matepukupuku e tipu ana i reira.

• Te rongoā arotahi:

Ka whakamahia e tēnei he rongoā, he matū rānei e whakaeke motuhake ana i ngā pūtau matepukupuku anake, engari kāore e whara i ngā pūtau hauora. Ka whakamahia tēnei maimoatanga mō te pheochromocytoma metastatic me te recurrent.

Kei te rangahauhia tētahi aukati tyrosine kinase e kiia nei ko te sunitinib mō te pheochromocytoma metastatic. Ka ārai ēnei rongoā i te tipu o te pukupuku.

Ka taea te ārai i tēnei mate?

Engari, kāore he huarahi hei ārai i te pheochromocytoma. Heoi, ki te mea kei te tūpono koe ki te whakawhanake i tēnei mate nā ngā mate tuku iho me ngā ira, ka taea e te tohutohu ira te āwhina i te tirotiro me te kimi wawe.

Mena kua pāngia tētahi o ō whanaunga tata (tuākana, tuākana, mātua) e te pheochromocytoma, he mate ira rānei tōu pērā i ngā mea i kōrerohia i mua ake nei (Multiple endocrine neoplasia 2 syndrome, Von Hippel-Lindau (VHL) disease, Neurofibromatosis type 1 (NF1), Hereditary paraganglioma syndrome, Carney-Stratakis dyad, Carney triad), he mea tino nui kia kōrero koe ki tō tākuta mō tēnei mea.

He aha te tūponotanga o te ora i muri i te maimoatanga? (Te matapae)

He tino pai te tirohanga mō te pheochromocytoma mēnā ka rongoatia.Kua kōrero kē mātou e tata ana ki te 90% o ngā pukupuku ka taea te tango angitu mā te pokanga.

Heoi, ki te kore e rongoatia, ka taea e tēnei mate te arahi ki ngā raruraru kino, tae atu ki te mate . Hei tauira:

• Ngā mate ngakau
• Te mate ngakau
• Te toto kore e taea te whakahaere i roto i te roro (Te toto o te roro)
• Te kohikohinga wai i roto i ngā pukahukahu (te pupuhi pūkahukahu)

Ko ētahi o ngā tūroro pheochromocytoma kei te mōrearea anō hoki ki te whiu, ki te mate ngakau rānei.

Āhea ahau me toro atu ki te tākuta?

• Mena ka tāpaetia koe he pheochromocytoma, ā, ka puta mai ētahi tohu whakararuraru, me toro atu ki tō tākuta tonu atu.
• Mena kei a koe ngā tohu o te pheochromocytoma, pērā i te toto toto tiketike me te ānini, kōrero ki tō tākuta. Ahakoa he onge te pheochromocytoma, he mea nui kia rongoāhia te toto toto tiketike.
• Mena e mōhio ana koe kei tētahi o ō whanaunga tata (tuākana, tuākana, mātua) he mate ira pēnei i te 'Multiple endocrine neoplasia 2 syndrome' me te 'Von Hippel-Lindau (VHL) disease', tera pea ka nui ake tō tūponotanga ki te whakawhanake i te pheochromocytoma, nō reira me toro atu ki tētahi tākuta ki te kōrero mō ngā whakamātautau ira.

He aha ngā pātai me pātai atu e au ki te tākuta?

Mena kua tāpaetia koe kei te mate koe i te pheochromocytoma, he pai pea te pātai atu ki tō tākuta i ēnei pātai:

• He aha ahau i pāngia ai e te pheochromocytoma?
• Ka taea e aku tamariki, me/ranei aku whanaunga te pāngia e te pheochromocytoma?
• He aha ngā kōwhiringa maimoatanga kei a au?
• He aha ngā pānga taha o ngā maimoatanga rerekē?
• Me pēhea taku whakahaere i aku tohumate?

Hei whakamutunga, ko ngā mea tino nui hei maumahara (Karere hei tango ki te kāinga)

Āe, ahakoa he pukupuku onge te pheochromocytoma, he maha ngā wā he pai, ā, ka taea te rongoā . Waihoki, ka taea te horapa i roto i ngā whānau, nō reira, ki te mea kua tāpaetia koe, tētahi rānei o tō whānau ki tēnei mate, he mea nui kia whakamātauria he whakamātautau ira. Ka āwhina hoki tēnei ki te whakatau mēnā kei te mōrearea koe ki ētahi atu raruraru hauora.

Kia maumahara, ki te mea he pātai āu mō tō tūponotanga ki te whakawhanake i te pheochromocytoma, mō tēnei mate rānei, kaua e wehi ki te kōrero ki tō tākuta. Kei konei rātou hei āwhina i a koe. Kia ora tonu!

Pheochromocytoma , repe adrenal, catecholamines, toto toto tiketike, ānini, werawera, pukupuku, pūnaha endocrine