Eia nā pane i kāu mau nīnau e pili ana i kēia puʻupuʻu laha ʻole i kapa ʻia ʻo Paraganglioma!

Ke manaʻo koke nei ʻoe i kekahi manawa e hou nui ana ʻoe, ke kuʻi wikiwiki nei kou puʻuwai, ke kuʻi nei kou poʻo? A i ʻole ke manaʻo nei ʻoe e piʻi koke ana kou koko me ke kumu ʻole? ʻOiai paha ʻoe e manaʻo he mau mea wale nō kēia, i kekahi manawa aia paha kekahi kumu ma hope o kēia. ʻO Paraganglioma kekahi ʻano maʻi kakaʻikahi akā koʻikoʻi e makaʻala.

No laila, he aha kēia paraganglioma?

I ka ʻōlelo maʻalahi, he mau puʻupuʻu kakaikahi nā paragangliomas e hana ʻia i loko o kou kino. Hoʻomohala pinepine lākou ma kahi kokoke i ke aʻa carotid, kahi kīʻaha koko nui i kou ʻāʻī, ma nā aʻalolo i kou poʻo a me kou ʻāʻī, a i ʻole ma kahi ʻē aʻe o kou kino. Hana ʻia kēia mau puʻupuʻu me kahi ʻano cell kūikawā i kapa ʻia he mau cell chromaffin . Hoʻopuka kēia mau cell i nā hormones i kapa ʻia he catecholamines a hoʻokuʻu iā lākou i loko o kou kahe koko.

Ua ʻike anei ʻoe ʻo nā kelepa adrenal, aia ma luna o ko kākou mau puʻupaʻa, hana i kekahi mau ʻano hormones? ʻO kēia mau catecholamines he mau hormones e kāohi ana i kekahi mau hana koʻikoʻi i loko o ko kākou kino. Ua ʻike anei ʻoe i ke ʻano o lākou?

• Ka helu o kou puʻuwai.
• Ke koko koko.
• Ka pae kō koko ('glucose koko').
• Pehea e pane ai ko mākou kino i ke kaumaha.

ʻO nā ʻano nui o nā catecholamines i mea nui:

• ʻO ka Dopamine.
• ʻO ka Epinephrine (ʻo kēia ka mea a mākou e kapa nei ʻo adrenaline).
• ʻO Norepinephrine (i kapa ʻia hoʻi ʻo noradrenaline).

ʻOiai ʻaʻole ulu nā paragangliomas i loko o nā kelepa adrenal, ua hoʻokumu ʻia kēia mau puʻupuʻu mai ka ʻiʻo i loko o nā kelepa adrenal. No laila, ma waho aʻe o kēia mau paragangliomas, hiki i nā hormones catecholamine ke hōʻiliʻili i loko o ke koko. ʻO ia ka manawa e hoʻomaka ai nā hōʻailona like ʻole e ʻike ʻia.

He aha ka ʻokoʻa ma waena o Paraganglioma a me Pheochromocytoma?

Hiki i kēia mau inoa ʻelua ke lilo i mea huikau iki, ʻeā? ʻO Paraganglioma a me pheochromocytoma he mau puʻupuʻu kakaikahi e kū mai ana mai nā pūnaewele chromaffin i ʻōlelo ʻia ma luna. ʻO ka ʻokoʻa nui ma waena o nā mea ʻelua kahi i hana ʻia ai lākou i loko o ke kino.

Hoʻomohala ʻia nā Pheochromocytomas ma ka ʻaoʻao waena o kou kelepa adrenal, i kapa ʻia ʻo ka adrenal medulla. Hoʻomohala ʻia nā Paragangliomas ma waho o ka kelepa adrenal. Loaʻa pinepine lākou ma nā aʻa a i ʻole nā ​​​​aʻalolo ma ka ʻāʻī. ʻO ia ke kumu i kapa ʻia ai nā paragangliomas ʻo 'extra-adrenal pheochromocytomas'.

He maʻi ʻaʻai anei ʻo Paraganglioma?

He pilikia kēia i loaʻa i nā poʻe he nui. Hiki i kēia mau puʻupuʻu, i kapa ʻia ʻo paragangliomas, ke lilo i maʻi kanesa (malignant) a i ʻole noncancerous (benign).Ma ke ʻano maʻamau, ma kahi o 20% o nā paragangliomas he maʻi kanesa.

Akā ʻo ka pilikia ma ʻaneʻi, i kekahi manawa he paʻakikī i nā kauka ke ʻike maopopo inā he maʻi kanesa ka puʻupuʻu a ʻaʻole paha. ʻO ia hoʻi, ʻoiai inā ua wehe ʻia ka puʻupuʻu ma ke ʻoki ʻana a ua nānā ʻia kona ʻiʻo ma lalo o kahi microscope, i kekahi manawa ʻaʻole hiki ke ʻōlelo maopopo. No laila, ua manaʻo pinepine ʻia kahi paraganglioma he maʻi kanesa i nā hihia aʻe:

• Inā ua pālahalaha ka ʻōpū i nā ʻiʻo a puni (ua kapa ʻia kēia ʻo 'regional spread of paraganglioma').
• Inā ua pālahalaha aku i nā ʻōpū mamao, e like me kou mau māmā a i ʻole nā ​​iwi (ua kapa ʻia kēia he 'metastasized').
• Inā ua hoʻi mai ma hope o ka mālama mua ʻia ʻana a me ka ho'ōla ʻia ʻana (ua kapa ʻia kēia ʻo 'recurred').

Inā he maʻi ʻaʻai ia, pehea lā e laha ai?

Inā he maʻi ʻaʻai kēia paraganglioma, ʻaʻohe ʻōnaehana hoʻonohonoho maʻamau no ia. Akā, ua wehewehe ʻia penei:

• ʻO ka paraganglioma kūloko: Aia ka puʻupuʻu ma kahi hoʻokahi wale nō.
• ʻO ka paraganglioma ʻāpana: Ua pālahalaha ke kanesa i nā lymph nodes a i ʻole nā ​​​​ʻiʻo ʻē aʻe kokoke i kahi i hoʻomaka mua ai.
• ʻO ka paraganglioma metastatic: Ua pālahalaha ke kanesa i nā ʻāpana ʻē aʻe o kou kino, e like me kou ake, nā māmā, nā iwi, a i ʻole nā ​​​​kikowaena lymph mamao. Ma waena o 35% a me 50% o nā paragangliomas cancerous hiki ke pālahalaha (metastasize) ma kēia ala.
• ʻO ka paraganglioma hou: Ua hoʻi mai ke kanesa ma hope o ka mālama ʻia ʻana a me ka hoʻōla ʻia. Hiki ke noho ma kahi like me ia ma mua, a i ʻole hiki ke noho ma kahi ʻāpana ʻē aʻe o ke kino.

Pehea ka wikiwiki o ka ulu ʻana o kēia mau nati?

ʻO ka maʻamau, ulu mālie loa nā paragangliomas. Eia nō naʻe, hiki ke ʻokoʻa kēia mai kēlā kanaka a kēia kanaka. Hiki i kekahi poʻe ke ulu wikiwiki iki.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia kūlana?

Hiki i kēia kūlana, i kapa ʻia ʻo paraganglioma, ke ulu i kēlā me kēia makahiki. Eia nō naʻe, ʻoi aku ka maʻamau i nā poʻe ma waena o 30 a me 50 mau makahiki. Eia kekahi, ma kahi o 10% o nā hihia i hōʻike ʻia i nā keiki.

Pehea ka maʻamau o Paraganglioma?

He ʻōʻō laha ʻole kēia. Ma ke ʻano helu helu, ua manaʻo ʻia he ʻelua wale nō kānaka i loko o hoʻokahi miliona e loaʻa i nā paragangliomas. No laila hiki iā ʻoe ke noʻonoʻo i ka laha ʻole o kēia.

He aha nā hōʻailona o Paraganglioma?

ʻO nā hōʻailona o nā paragangliomas i hoʻokumu ʻia e ka puʻupuʻu e hoʻokuʻu nui ana i ka adrenaline a i ʻole noradrenaline i ʻōlelo ʻia ma luna i loko o kou koko. Eia nō naʻe, ʻaʻole hana kekahi mau paragangliomas i kēia hormone keu, a no laila ʻaʻole lākou e hana i kekahi mau hōʻailona (ua kapa ʻia lākou he asymptomatic).

Nā hōʻailona i ʻike pinepine ʻiaʻOiaʻiʻo:

• Ka hoʻomaka koke ʻana o ke koko kiʻekiʻe (hypertension).
• ʻEha poʻo.
• Ka hou nui ʻana me ke kumu ʻole.
• He puʻuwai wikiwiki a kūpono ʻole hoʻi e hiki ai ke lohe ʻia ka puʻuwai e kuʻi ana.
• Manaʻo e haʻalulu ana kou kino.

Ma waho aʻe o kēia mau mea, aia kekahi mau hōʻailona i emi pinepine ʻole :

• ʻOi aku ka keʻokeʻo ma mua o kou ʻano maʻamau.
• Ka luaʻi a me/a i ʻole ka luaʻi ʻana.
• Ka maʻi pākī.
• Ka paʻa ʻana o ka ʻōpū.
• Ke kiʻekiʻe o ke kō koko (hyperglycemia).
• ʻO ka hāʻule koke ʻana o ke koko e hoʻoulu ai i ka poniuniu ke kū ʻoe i luna (ua kapa ʻia kēia he orthostatic hypotension).
• Ka wiwi ʻana me ke kumu ʻole.

Hiki i kekahi poʻe ke ʻike pinepine i kēia mau hōʻailona, ​​a i ʻole i ka pahū ʻana. E noʻonoʻo, i kekahi manawa ʻaʻohe hewa, a laila ʻike koke ʻia kēia mau hōʻailona, ​​a laila nalowale lākou ma hope o kekahi manawa. ʻO ia ke kumu i lohi ai ka ʻike ʻana i kēia i kekahi manawa.

He aha ke kumu o ka paraganglioma?

ʻO ka hapa nui o ka manawa, ʻaʻohe kumu kikoʻī no nā paragangliomas. ʻO ia hoʻi, hiki wale mai lākou. Eia nō naʻe, ma kahi o 25% a 35% o ka poʻe e loaʻa i nā paragangliomas ma muli o kahi kūlana genetic e holo ana i loko o nā ʻohana (kūlana hoʻoilina). ʻO kekahi o kēia mau kūlana:

• ʻO ka maʻi neoplasia endocrine lehulehu 2, nā ʻano A a me B (MEN2A a me MEN2B)).
• ʻO ka maʻi Von Hippel-Lindau (VHL).
• ʻO ka Neurofibromatosis ʻano 1 (NF1).
• ʻO ka maʻi paraganglioma hoʻoilina.
• ʻO Carney-Stratakis dyad (kahi e hiki ai i ka paraganglioma ke hele pū me ka gastrointestinal stromal tumor (GIST)).
• ʻO Carney triad (hiki ke komo pū me ka paraganglioma, GIST, a me kahi ʻano puʻupuʻu māmā i kapa ʻia ʻo pulmonary chondroma).

Pehea e ʻike ʻia ai ʻo Paraganglioma?

Hiki ke paʻakikī ke ʻike i ka paraganglioma no ka mea he puʻupuʻu laha ʻole ia a i kekahi manawa ʻaʻole ia e hōʻike i nā hōʻailona. I kekahi manawa, ʻike nā kauka i nā paragangliomas i ka wā e hana ana i nā hoʻokolohua no kekahi kumu ʻē aʻe.

Hiki i ke kauka ke kānalua i ka paraganglioma ma hope o ka noʻonoʻo ʻana i nā mea aʻe:

• ʻO kāu mōʻaukala lapaʻau piha, ʻoiai inā paha i loaʻa i kekahi o kou ʻohana ka pheochromocytoma a i ʻole paraganglioma.
• He hoʻokolokolo kino a me ka hoʻokolokolo lapaʻau piha.
• ʻO ke ʻano o kekahi mau hōʻailona. Eia kekahi laʻana, inā he koko kiʻekiʻe kou i kāohi ʻole ʻia e nā lāʻau lapaʻau maʻamau.

He aha nā hoʻokolohua i hana ʻia no kēia?

Hiki i kāu kauka ke hoʻohana i kēia mau hoʻokolohua a me nā kaʻina hana e hoʻoholo ai inā he paraganglioma kou:

• Hoʻokolokolo kino: E nānā kāu kauka iā ʻoe a nānā i kou olakino holoʻokoʻa, e like me kou koko. E nīnau pū ʻo ia e pili ana i kou mōʻaukala olakino a me kou ʻohana, ʻoiai kekahi mau pilikia e pili ana i ka hormone.
• Hoʻāʻo mimi 24-hola: Pili kēia i ka hōʻiliʻili ʻana i kahi hāpana o kāu mimi ma luna o kahi manawa 24-hola a me ke ana ʻana i ka nui o nā hormones adrenal i kapa ʻia ʻo catecholamines. Ke nānā nei hoʻi ia i nā mea i hana ʻia i ka wā e haki ai kēia mau hormones. Inā loaʻa kēia mau catecholamines i loko o kāu mimi ma nā pae kiʻekiʻe ma mua o ka maʻamau, he hōʻailona paha ia o kahi paraganglioma.
• Nā hoʻāʻo catecholamine koko: Ke ana nei kēia mau hoʻāʻo i nā pae o nā catecholamine i loko o kou koko. E like me ka mea i ʻōlelo ʻia ma mua, ke nānā nei lākou i nā mea i hana ʻia i ka wā e haki ai kēia mau hormones. Inā loaʻa kēia mau mea i nā nui kiʻekiʻe ma mua o ka maʻamau i loko o ke koko, hiki nō hoʻi ke lilo i hōʻailona o ka paraganglioma.
• ʻImi PET (positron emission tomography scan): ʻO ka scan PET e pili ana i ka hoʻokomo ʻana i kahi kemika radioactive palekana (radiotracer) i loko o kou kino a me ka lawe ʻana i nā kiʻi o kou mau ʻōpū a me nā ʻiʻo me ka hoʻohana ʻana i kahi mea i kapa ʻia he scanner PET. ʻIke kēia scan i nā cell hana nui a me nā puʻupuʻu e omo ana i ka kemika. Hiki i kēia ke kōkua i ka hoʻoholo ʻana inā he pilikia olakino. He mea maikaʻi loa kēia scan no ka kuhikuhi ʻana i kahi o kahi paraganglioma.
• CT scan (computer tomography scan): ʻO ka CT scan kahi hana e lawe ana i kahi moʻo o nā kukuna X mai nā kihi like ʻole e hana i nā kiʻi kikoʻī o loko o kou kino. Hiki i kāu kauka ke paipai i kahi CT scan e kōkua i ke kuhikuhi ʻana i kahi o kahi puʻupuʻu (ma ka ʻāʻī).
• ʻO ke kiʻi MRI (magnetic resonance imaging): Hoʻohana kahi MRI i kahi magnet, nā nalu lekiō, a me kahi kamepiula e hana i kahi moʻo o nā kiʻi kikoʻī o loko o kou kino. Hiki i kāu kauka ke paipai i kahi MRI e nānā pono i kahi i loaʻa ai ka puʻupuʻu.

Ma hope o ka nānā ʻana o kāu kauka iā ʻoe me ka paraganglioma, hiki iā lākou ke hana i nā hoʻokolohua hou aʻe e ʻike ai inā ua pālahalaha aku i nā ʻāpana ʻē aʻe o kou kino.

He mea pono anei ka hoʻāʻo ʻana i ka genetic?

Inā ua ʻike ʻia ʻoe me ka paraganglioma, e ʻōlelo paha kāu kauka e hele ʻoe i ke aʻo ʻana i nā genetics e ʻike ai inā he maʻi hoʻoilina kou a ua pilikia ʻoe no ka hoʻomohala ʻana i nā maʻi ʻē aʻe e pili ana me ia.

Hiki i kāu kauka ke ʻōlelo i ka hoʻāʻo genetic i kēia mau hihia:

• Inā he mau hōʻailona kou a i ʻole kekahi o kou ʻohana e pili ana i ka pheochromocytoma hoʻoilina a i ʻole ka maʻi paraganglioma.
• Inā he mau hōʻailona o ke kiʻekiʻe o nā catecholamines i loko o kou koko a i ʻole nā ​​​​hōʻailona o kahi paraganglioma cancerous.
• Inā ua loaʻa iā ʻoe ka paraganglioma ma mua o 40 mau makahiki.

Inā ʻike kāu kākāʻōlelo genetic i kekahi mau loli o ka gene i kāu mau hopena hoʻāʻo, e ʻōlelo paha ʻo ia e hoʻāʻo ʻia nā lālā ʻē aʻe o kou ʻohana (ʻoiai ka poʻe i asymptomatic akā i ka pilikia).

Pehea e mālama ʻia ai ʻo Paraganglioma?

ʻO ka mālama ʻana i ka paraganglioma e pili ana i kekahi mau kumu, e like me:

• Ka nui o ka puʻupuʻu.
• Inā he maʻi kanesa (malignant) a i ʻole benign (benign) ka puʻupuʻu.
• Inā he mau hōʻailona kāu ma muli o ke kiʻekiʻe o nā catecholamines.
• Aia wale nō anei ka puʻupuʻu ma kahi hoʻokahi, a ua pālahalaha paha (metastasized) i nā ʻāpana ʻē aʻe o ke kino?
• Inā paha ua ʻike ʻia ka puʻupuʻu no ka manawa mua, a inā paha ua ho'ōla mua ʻia a laila hoʻi hou mai.

Inā loaʻa iā ʻoe kahi paraganglioma e hoʻoulu ai i nā hōʻailona ma muli o ka nui o nā hormones, e kuhikuhi kāu kauka i nā lāʻau lapaʻau e kāohi i kēlā mau hōʻailona. Hiki i kēia mau lāʻau lapaʻau ke komo pū me:

• Nā lāʻau lapaʻau e kaohi ai i kou koko, no ka laʻana nā alpha-blockers .
• Nā lāʻau lapaʻau e mālama i ka helu o kou puʻuwai ma kahi pae maʻamau, no ka laʻana nā beta-blockers .
• Nā lāʻau lapaʻau e ālai ai i nā hopena o nā hormones i hoʻokuʻu nui ʻia e kāu mau kelepa adrenal.

ʻO nā koho lapaʻau no ka paraganglioma:

• Wehe ʻana i ka puʻupuʻu ma ke ʻoki kino.
• Hoʻōla radiation.
• Kemoterapi.
• Lapaʻau ablation.
• Hoʻōla i kuhikuhi ʻia.

E hui pū, ʻo ʻoe a me kāu hui lapaʻau e hoʻoholo i ka hoʻolālā lapaʻau e kūpono loa iā ʻoe a me kou kūlana.

Wehe ʻoki kino i ka puʻupuʻu

ʻO ke ʻoki kino ka lāʻau lapaʻau nui no ka paraganglioma. I ka wā o ke ʻoki kino e wehe ai i ka puʻupuʻu, e nānā kāu kauka ʻoki i nā ʻiʻo a puni a me nā lymph node e ʻike ai inā ua pālahalaha ka puʻupuʻu. Inā ua pālahalaha, e wehe ke kauka ʻoki i nā ʻiʻo i hoʻopilikia ʻia, inā hiki.

Hiki ke wehe ʻia ka hapa nui o nā paragangliomas me ka hoʻohana ʻana i nā ʻano hana minimally invasive, e like me ke ʻoki laparoscopic . Pili kēia i ka hana ʻana i kekahi mau ʻoki liʻiliʻi i kou ʻili a me ka wehe ʻana i ka puʻupuʻu me nā mea hana kūikawā. Eia nō naʻe, pono paha i nā puʻupuʻu nui ke ʻoki wehe kuʻuna.

Ma hope o ke ʻoki kino, e nānā kāu kauka i nā pae o nā catecholamines i loko o kou koko a i ʻole ka mimi. Inā hoʻi nā pae o nā catecholamines i ka maʻamau, he hōʻailona kēia ua wehe ʻia nā hunaola paraganglioma āpau.

Hoʻōla Pāhawewe

ʻO ka radiation therapy kahi lāʻau lapaʻau maʻi kanesa e hoʻohana ana i nā kukuna ikehu kiʻekiʻe o ka radiation e pepehi i nā hunaola kanesa a i ʻole e hoʻōki iā lākou mai ka ulu ʻana. Hana ʻia kēia me ka hoʻēmi ʻana i ka hōʻino ʻana i nā ʻiʻo olakino a puni.

Aia ʻelua ʻano o ka radiation therapy:

• ʻO ka lāʻau lapaʻau radiation waho: Pili kēia i ka hoʻohana ʻana i kahi mīkini ma waho o kou kino e kuhikuhi i ka radiation i kahi i loaʻa ai ke kanesa.
• Lapaʻau radiation kūloko: Pili kēia i ke kau ʻana i kahi mea radioactive i loko o nā nila, nā hua, nā uea, a i ʻole nā ​​​​catheters, a laila e kau pololei ʻia i loko a kokoke paha i ka puʻupuʻu e ke kauka.

ʻO ke ʻano o ka radiation therapy a kāu kauka e ʻōlelo nei e pili ana i ka mea inā he kūloko kou maʻi kanesa, ʻāpana, mamao, a ua hoʻi mai paha. Hoʻohana pinepine nā kauka i ka radiation therapy beam waho a/a i ʻole ka 131I-MIBG therapy e mālama i nā paragangliomas cancerous. ʻO ka 131I-MIBG therapy kahi ʻano o ka hāʻawi ʻana i kahi mea radioactive i kekahi mau ʻano o nā cell cancer, a laila e hoʻokomo ʻia i loko o ke kino a ʻo ka radiation āna e hoʻopuka ai e pepehi i nā cell.

Kemoterapia

ʻO ke ʻano lapaʻau maʻamau no ka metastatic paraganglioma ʻo ia ka chemotherapy. Pili kēia i ka hoʻohana ʻana i nā lāʻau lapaʻau e pepehi i nā hunaola kanesa, e hoʻōki iā lākou mai ka māhele ʻana, a i ʻole e hoʻōki iā lākou mai ka ulu ʻana. Hāʻawi pinepine ʻia ka chemotherapy ma ke ʻano intravenous. ʻOiai he lāʻau lapaʻau maikaʻi kēia, hiki iā ia ke hana i nā hopena ʻaoʻao.

Lapaʻau Ablation

ʻO ka Ablation therapy kahi koho lapaʻau minimally invasive e hoʻohana ana i nā mahana kiʻekiʻe loa a haʻahaʻa loa paha e luku i nā puʻupuʻu. ʻO nā lāʻau lapaʻau ablation e kōkua i ka pepehi ʻana i nā hunaola kanesa a me nā hunaola maʻamau ʻole:

• ʻO ka hoʻopau ʻana i ke alapine radio: Pili kēia i ka hoʻohana ʻana i nā nalu radio e hoʻomehana a luku i nā hunaola kanesa a me nā hunaola ʻē. Hoʻouna ʻia nā nalu radio ma o nā electrodes (nā mea liʻiliʻi e alakaʻi i ka uila).
• Cryoablation: Hoʻohana kēia lāʻau i ka naikokene wai a i ʻole ke kalapona ʻokikene wai e hoʻopaʻa a luku i nā ʻōpū kanesa a me nā ʻōpū maʻi ʻē.

Hoʻōla i kuhikuhi ʻia

ʻO ka lāʻau lapaʻau i kuhikuhi ʻia kahi koho lapaʻau e hoʻohana ana i nā lāʻau lapaʻau a i ʻole nā ​​​​mea ʻē aʻe e hoʻouka wale i nā hunaola kanesa kikoʻī, me ka ʻole o ka hōʻino ʻana i nā hunaola olakino. Hoʻohana nā kauka i ka lāʻau lapaʻau i kuhikuhi ʻia e mālama i nā paragangliomas mamao a me nā paragangliomas hou.

Ke noiʻi nei nā kānaka noiʻi i kahi mea hoʻopaʻa tyrosine kinase i kapa ʻia ʻo sunitinib.Ke aʻo ʻia nei kekahi ʻano lāʻau lapaʻau e ʻike i ke ʻano o ka mālama ʻana i nā paragangliomas mamao. ʻO ka lāʻau lapaʻau inhibitor Tyrosine kinase kahi ʻano lāʻau lapaʻau i hoʻopaʻa ʻia e hoʻōki i ka ulu ʻana o ka maʻi puʻupuʻu.

Hiki ke pale ʻia ka ulu ʻana o ka paraganglioma?

ʻO ka mea pōʻino, ʻaʻole hiki ke pale ʻia nā paragangliomas. Eia nō naʻe, inā loaʻa iā ʻoe kekahi mau syndromes hoʻoilina a me nā genes e hoʻoweliweli iā ʻoe no ka hoʻomohala ʻana i nā paragangliomas, hiki i ke aʻo ʻana i nā genetics ke kōkua iā ʻoe e hoʻāʻo ʻia no nā paragangliomas a loaʻa paha iā lākou i ka wā mua.

E kamaʻilio me kāu kauka inā ua loaʻa i kou mau hoahānau mua (nā kaikaina a me nā mākua) ka paraganglioma a i ʻole pheochromocytoma, a/a inā loaʻa iā ʻoe kekahi o kēia mau kūlana genetic:

• ʻO ka maʻi endocrine neoplasia 2 he nui.
• ʻO ka maʻi Von Hippel-Lindau (VHL).
• ʻO ka neurofibromatosis ʻano 1.
• ʻO ka maʻi paraganglioma hoʻoilina.
• ʻO ke kaulahao ʻelua o Carney-Stratakis.
• ʻEkolu ʻo Carney.

He aha ka wānana ma kēia kūlana?

ʻOkoʻa ka nānā ʻana no nā paragangliomas, ʻo ia hoʻi, ka manawa e hoʻōla ai a ola ai, ma muli o kekahi mau kumu. I waena o lākou:

• ʻAuhea ka puʻupuʻu i loko o kou kino a pehea kona nui.
• Inā paha he maʻi ʻaʻai a ua laha paha i nā ʻāpana ʻē aʻe o ke kino.
• Ua wehe ʻia ke ʻōpū ma ke ʻoki ʻana, a inā pēlā, ehia ka nui o ke ʻōpū i wehe ʻia i ka wā o ke ʻoki ʻana?

ʻO ka poʻe me kahi paraganglioma liʻiliʻi i laha ʻole i nā ʻāpana ʻē aʻe o ke kino (ʻaʻole i metastasized) he ʻelima mau makahiki o ka ola ʻana ma kahi o 95%. Eia nō naʻe, ʻo ka poʻe me kahi paraganglioma i hoʻi hou mai ma hope o ka mālama mua ʻana a i ʻole ua laha i nā ʻāpana ʻē aʻe o ke kino (metastasized) he ʻelima mau makahiki o ka ola ʻana ma waena o 34% a me 60% .

Eia kekahi, aia kekahi mau paragangliomas koʻikoʻi, ʻoiai inā ʻaʻole lākou i pālahalaha loa, ʻaʻole hiki ke wehe loa ʻia e ke ʻoki kino no ka mea ua pālahalaha nui lākou i loko o nā ʻiʻo a puni. I ia mau hihia, hiki i ka hoʻokuʻu nui ʻana o ka adrenaline a me ka noradrenaline ke lilo i mea weliweli a paʻakikī hoʻi e mālama.

ʻO ka mea nui loa, ʻo nā paragangliomas, inā he maʻi kanesa a ʻaʻole paha, inā waiho ʻole ʻia, hiki ke hana i nā pilikia koʻikoʻi, a hiki i ke ola ma muli o ka nui o ka adrenaline a me ka noradrenaline a lākou e hūnā ai.

ʻO kekahi o kēia mau pilikia:

• Ka maʻi ʻiʻo puʻuwai (cardiomyopathy).
• Ka mumū ʻana o kou mau ʻiʻo puʻuwai ('myocarditis').
• Ke kahe koko ʻole i loko o kou lolo (cerebral hemorrhaging).
• Hōʻiliʻili ʻana o ka wai i loko o kou mau māmā (edema pulmonary).
• Hoʻouka puʻuwai ('myocardial infarction').
• Hahau.
• Koma.
• Ka make.

Āhea wau e ʻike ai i ke kauka?

Inā ua ʻike ʻia ʻoe me ka paraganglioma a ʻike ʻoe i nā hōʻailona kānalua, e ʻike koke i kāu kauka.

Inā ʻike ʻoe i nā hōʻailona o ka paraganglioma, e like me ke koko kiʻekiʻe a me ke poʻo ʻeha, e kamaʻilio me kāu kauka. ʻOiai he mea kakaikahi ka paraganglioma, he mea nui nō hoʻi e mālama i ke koko kiʻekiʻe, ʻoiai inā ʻaʻole paha e loaʻa iā ʻoe.

Inā ʻike ʻoe ua loaʻa i kekahi o kou mau hoahānau mua (nā kaikaina, nā mākua) kahi maʻi genetic e like me ka "Multiple endocrine neoplasia 2 syndrome" a i ʻole ka "maʻi Von Hippel-Lindau (VHL)", e nīnau i kāu kauka e pili ana i ka hoʻāʻo genetic, no ka mea, hiki i kēia ke hoʻonui i kou pilikia o ka loaʻa ʻana o ka paraganglioma.

He aha nā nīnau āu e nīnau ai i kāu kauka?

Inā ua ʻike ʻia ʻoe me ka paraganglioma, he mea kōkua paha ke nīnau i kāu kauka i kēia mau nīnau:

• No ke aha i loaʻa ai iaʻu ka paraganglioma?
• Hiki i kaʻu mau keiki a/a i ʻole nā ​​​​hoahānau ke loaʻa i ka paraganglioma?
• He aha nā koho lapaʻau e loaʻa iaʻu?
• He aha nā hopena ʻaoʻao o nā lāʻau lapaʻau like ʻole?
• Pehea e hiki ai iaʻu ke mālama i kaʻu mau hōʻailona?

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)

He maʻi laha ʻole ka Paraganglioma, akā he mea nui e makaʻala i ia mea. Inā loaʻa iā ʻoe nā hōʻailona mau e paʻakikī ke hoʻomaopopo, ʻoiai ke koko kiʻekiʻe koke, ka hou ʻana, a i ʻole ka wikiwiki o ka puʻuwai, he mea naʻauao ke ʻimi i ka ʻōlelo aʻo lapaʻau ma mua o ka hoʻokuʻu ʻana iā lākou he mea maʻamau wale nō.

ʻOiai ʻaʻole ʻike pinepine ʻia ke kumu o ka paraganglioma, ua pili ia i kekahi mau kūlana hoʻoilina. No laila, inā ua loaʻa i kekahi o kou ʻohana ka paraganglioma a i ʻole pheochromocytoma, hiki i ka hoʻāʻo genetic ke kōkua iā ʻoe e ʻike i kou pilikia, a me ka loaʻa ʻana o nā pilikia olakino ʻē aʻe. Inā he mau nīnau kāu e pili ana i kēia, mai kānalua e kamaʻilio me kāu kauka. Eia lākou e kōkua iā ʻoe.

ʻO Paraganglioma, Paraganglioma, Pheochromocytoma, Nā pūnaewele Chromaffin, Catecholamines, Hormones, Tumors, Kanesa, Nā Hōʻailona, ​​​​Kikokikona Koko, Nā Maʻi Genetic