He aha ka Glucagonoma? E kamaʻilio kākou e pili ana i kēia maʻi laha ʻole!

He ʻōkana liʻiliʻi akā koʻikoʻi loa ka pancreas i loko o kou kino. Hoʻopuka ia i kekahi mau hormones e pono ai ko kākou kino. No laila, i kekahi manawa, hiki i kahi ʻano maʻi kanesa kakaʻikahi e ulu ana i loko o ka pancreas ke hana i nā pilikia. ʻO ia ka mea i kapa ʻia ai kēia maʻi ʻo Glucagonoma . Malia paha ua loaʻa koke iā ʻoe ka maʻi diabetes, a ua loaʻa paha iā ʻoe kahi mea e like me ka ʻulaʻula ʻē ma kou ʻili, he mea nui loa ia e makaʻala i kēia.

He aha ka glucagonoma? I ka ʻōlelo maʻalahi...

He puʻupuʻu kanesa laha ʻole ka Glucagonoma e ulu ana i loko o kou pancreas. Hoʻopuka kēia puʻupuʻu i ka nui o ka hormone i kapa ʻia ʻo glucagon. I kēia manawa ke noʻonoʻo nei paha ʻoe i ke ʻano o kēia glucagon.

He hormone koʻikoʻi loa ka Glucagon e kōkua ana i ka hoʻomalu ʻana i ke kiʻekiʻe o ke kō o ko mākou kino, ʻo ia hoʻi, ka pae glucose koko . Hana ʻia ia e nā cell alpha, kahi ʻano cell kūikawā i loko o kou pancreas. E noʻonoʻo inā ʻaʻole ʻoe i ʻai no kekahi manawa, inā ʻoe e hoʻoikaika kino nui, a inā ʻoe e ʻai i kahi meaʻai momona i ka protein, hiki i kou pae kō koko ke hāʻule, ʻeā? ʻO ia ka manawa e komo mai ai kēia glucagon, e hoʻokuʻu i ke kō i mālama ʻia i loko o kou ake, ʻo ia hoʻi, ka glycogen , a hoʻihoʻi i ke kiʻekiʻe o ke kō koko. Ua like ia me kekahi mea e kōkua ana i ka mālama ʻana i ke kaulike kō i loko o ko mākou kino.

Eia nō naʻe, i ka wā e loaʻa ai ka glucagonoma, hana nui ʻia kēia hormone glucagon. A laila ʻaʻole hiki i ko mākou kino ke hoʻokele iā ia. ʻO ka hui pū ʻana o nā hōʻailona e kū mai ana ma muli o kēia ua kapa ʻia ʻo Glucagonoma Syndrome . Hiki i kēia ke alakaʻi i nā pilikia he nui e like me nā pilikia ʻili a me ka maʻi diabetes.

ʻO kēia ʻano maʻi kanesa, i kapa ʻia ʻo glucagonoma, no loko ia o ka māhele o nā tumors neuroendocrine pancreatic, a i ʻole nā ​​tumors cell islet . ʻO ka hapa pinepine, kū mai kēia mau mea ma ka huelo a i ʻole ka ʻāpana waena o ka pancreas.

He maʻi ʻaʻai maoli anei kēia?

ʻAe, ʻo ka hapa nui o ka manawa, he maʻi kanesa ka glucagonoma. ʻO ka mea kaumaha, ʻoiai ke ʻike ʻia, ma kahi o 50% o nā hihia, ʻo ia hoʻi, ma kahi o hoʻokahi i loko o ʻelua, ua laha paha kēia maʻi kanesa (metastasized) i nā ʻāpana ʻē aʻe o ke kino, ʻoi aku hoʻi ke akepaʻa .

Pehea ka maʻamau o kēia ʻano?

He maʻi laha ʻole ka Glucagonoma. ʻAʻole i ʻike ʻia ma mua o hoʻokahi i loko o hoʻokahi miliona mau hihia hou i kēlā me kēia makahiki. Hoʻopilikia pinepine ia i nā poʻe ma waena o 50 a me 70 mau makahiki .

He aha nā hōʻailona o kēia?

Ulu lohi pinepine nā Glucagonomas. No laila, inā ʻaʻole hana kou kino i ka glucagon lawa e hoʻoulu ai i nā pilikia, ʻaʻole paha ʻoe e loaʻa i kekahi mau hōʻailona.

Eia nō naʻe, i ka wā e hana nui ai ka glucagon i ka glucagon a ʻaʻole hiki i ke kino ke lawelawe iā ia, e kū mai ka maʻi glucagonoma i ʻōlelo mua ʻia.Hiki mai ia. A laila, e ʻike ʻoe i nā hōʻailona maopopo. Inā loaʻa iā ʻoe kēia mau hōʻailona, ​​​​he mea nui loa e ʻike koke i ke kauka.

ʻŌhūhū ʻili (Necrolytic Migratory Erythema - NME)

ʻO kēia ka hōʻailona nui a maʻamau o ka glucagonoma syndrome. Ma kahi o 90% o ka poʻe me kēia syndrome e loaʻa i kēia pilikia ʻili. Ua kapa ʻia ʻo ia he necrolytic migratory erythema (NME) .

E noʻonoʻo, ʻo kou ʻili, ʻoiai ma nā wahi pilikino, nā puʻupuʻu, a me nā wāwae haʻahaʻa, e hoʻomaka ana e ulu i nā wahi ʻulaʻula, maneʻo, a i kekahi manawa ʻeha. Ma hope aku, lilo kēia mau mea i mau pehu, māwae, a me ka ʻili. ʻOiai ma hope o ko lākou ho'ōla ʻana, hiki ke waiho ʻia kahi wahi ʻeleʻele. Hiki i kēia ke hana ma kekahi wahi, ho'ōla, a laila e ʻike hou ʻia ma kahi ʻē aʻe. Ua like ia me kahi ʻulaʻula e neʻe ana mai kēlā wahi a i kēia wahi.

Nā hiʻohiʻona ʻē aʻe o NME:

• Hiki i kēia ke hele mai a hele aku, a hiki ke neʻe a puni ke kino .
• Hiki ke ʻeha loa a me ka ʻāʻī i ka wā mua.
• Lilo ka ʻulaʻula i ka ʻōpala a laila pehu a hana i kahi ʻili. Ma hope o ka ho'ōla ʻana, hiki ke waiho ʻia kahi wahi ʻeleʻele.
• Hiki i kēia ke alakaʻi i nā mea e like me nā ʻeha alelo (glossitis ), nā ʻeha waha, nā lehelehe māwae, a me ka angular cheilitis .
• ʻO ka pehu ʻana o nā lihilihi ( blepharitis ), ka nalowale o ka lauoho, a me nā pilikia kui e hiki mai ana.

Nā hōʻailona o ka maʻi diabetes

Hoʻoulu kēia maʻi i ke kiʻekiʻe o ke kō koko ( hyperglycemia ), hiki ke alakaʻi i ka maʻi diabetes. ʻO nā hōʻailona o ka maʻi diabetes:

• Ka wiwi ʻana me ke kumu ʻole.
• Ka makewai pinepine a nui (Polydipsia) .
• ʻO ka mimi pinepine ʻana ( polyuria ), ʻoi aku hoʻi i ka pō.
• Ka pōloli nui ( Polyphagia ).

Nā hōʻailona ʻē aʻe o (glucagonoma syndrome)

Aia kekahi mau hōʻailona ʻē aʻe:

• Ka maʻi pākī mau .
• Haʻahaʻa ka hemoglobin i loko o ke koko (anemia ). Hiki i kēia ke hana i ka luhi a me ka poniuniu.
• ʻO nā ʻūpā koko, ʻoi aku hoʻi ma nā aa koko hohonu o nā wāwae ( Deep Vein Thrombosis (DVT) ).

Nā hōʻailona e pili ana i ka ʻōnaehana noʻonoʻo a me ke kino

I kekahi manawa, ʻike paha ʻoe i nā hōʻailona e pili ana i ka ʻōnaehana noʻonoʻo a me ke kino, e like me:

• Kaumaha
• Dementia
• Hoʻonāukiuki
• Hyperreflexia (ka wikiwiki ʻana o nā pane a ke kino)
• He hele wāwae (ataxia )
• Nā maʻi noʻonoʻo ( Psychosis))
• Nā makaʻu a me nā kānalua kūpono ʻole ( Paranoid Delusions )

He aha ke kumu o ka glucagonoma?

ʻO ka hapa nui o ka manawa, ʻaʻole ʻike nā kauka i ke kumu kikoʻī o ka glucagonoma. Eia nō naʻe, ma kahi o 10% o nā hihia, ua pili kēia maʻi kanesa i kahi kūlana genetic i kapa ʻia ʻo Multiple Endocrine Neoplasia type 1 (MEN type 1) .

Eia naʻe, ʻaʻole nā ​​​​kānaka a pau me MEN type 1 e loaʻa i ka glucagonoma. He pakeneka liʻiliʻi loa kēlā.

Pehea ʻoe e ʻike ai i kēia?

Inā loaʻa iā ʻoe ke ʻano ʻili necrolytic migratory erythema (NME) i ʻōlelo ʻia ma mua, hiki i ke kauka ke kānalua i ka glucagonoma. A laila hiki iā lākou ke hana i nā hoʻokolohua e like me:

• Hoʻāʻo Kō koko hoʻokē ʻai: Inā kiʻekiʻe ke kiʻekiʻe o ke kō, hiki ke kānalua ʻia ka glucagonoma a hiki ke hōʻoia ʻia ka maʻi diabetes pili.
• Hoʻāʻo Koko Glucagon Hoʻokē ʻai: Kiʻekiʻe loa nā pae Glucagon i ka glucagonoma - ʻoi aku ka nui ma mua o 500 picograms i kēlā me kēia milliliter (pg/mL). ʻOi aku ka liʻiliʻi o nā pae glucagon hoʻokē ʻai ma mua o 150 pg/mL.
• Hoʻāʻo Koko ʻAkika Amino: ʻO ka Glucagonoma ka mea maʻamau ke kumu o nā pae haʻahaʻa o nā waikawa amino i loko o ke koko ( hypoaminoacidemia ).
• Helu Koko Piha (CBC): Nānā kēia no ka anemia , no ka mea, hiki ke loaʻa ka anemia me ka glucagonoma.

Hiki i nā hoʻokolohua kiʻi e like me kēia ke kōkua i ka hōʻoia inā loaʻa kahi puʻupuʻu:

• CT Scan
• MRI Scan
• ʻO ka Ultrasound Endoskopika

Pehea e mālama ʻia ai?

ʻO ka mālama ʻana i ka glucagonoma e hilinaʻi nui ʻia i ka palena o ke kanesa i ka pancreas (localized) a i ʻole ua pālahalaha aku i nā ʻāpana ʻē aʻe o ke kino (metastasized) .

Pehea e kaohi mua ai i nā hōʻailona

ʻO ka hana mua, ʻo ia ke kāohi ʻana i nā hōʻailona a me nā pilikia i hoʻokumu ʻia e ka glucagon keu. Hana ʻia kēia penei:

• Nā lāʻau lapaʻau e kāohi i ka glucagon keu: ʻO nā inikini i kapa ʻia ʻo Octreotide a me Lanreotide e kōkua i ka hōʻemi ʻana i nā hopena o ka glucagon keu a hoʻōki i ka hana ʻana o ka glucagon. Hiki i kēia mau lāʻau lapaʻau ke hoʻolohi i ka ulu ʻana o nā puʻupuʻu. Hāʻawi pū lākou i ka hōʻoluʻolu mai nā hōʻailona o ka NME, ka maʻi diabetes, ka maʻi diarrhea, a me nā maʻi ʻōnaehana nerve.
• Ka mālama ʻana i ka maʻi diabetes: Pono paha ʻoe i ka lāʻau lapaʻau maʻi diabetes a i ʻole nā ​​​​inikini insulin e mālama i kāu mau pae kō koko i loko o ka pae kūpono.
• Kākoʻo meaʻai:Hiki ke hāʻawi ʻia ka Total Parenteral Nutrition (TPN) , nā waikawa amino a me nā mea hoʻopihapiha zinc e hōʻemi i nā hopena o ka ʻai ʻole.
• Ka mālama ʻana i ka maʻi ʻili NME: Hiki i nā antibiotics a me nā corticosteroids ke hoʻomaikaʻi i ka NME.
• Lapaʻau Anticoagulant: ʻO nā lāʻau hoʻokahe koko e like me ka heparin e kōkua i ka pale ʻana i ka deep vein thrombosis (DVT) .

Inā ua kaupalena ʻia i ka pancreas ...

Inā aia wale nō ke kanesa i loko o ka pancreas, ʻo ke ʻoki kino ke ʻano nui o ka lāʻau . Inā hiki i ke kauka ʻoki ke wehe loa i ka puʻupuʻu, hoʻōla pinepine ʻia ka maʻi . Ma hope o ka wehe ʻia ʻana o ka glucagonoma, pono e hoʻi hou kāu mau pae glucagon i ka maʻamau. Pono hoʻi e nalo aku kāu mau hōʻailona.

Inā ua pālahalaha aku i nā wahi ʻē aʻe (metastasized) ...

I nā manawa hiki, ʻōlelo nā kauka i ka wehe ʻana i nā puʻupuʻu i loko o ka pancreas, nā lymph node kokoke, a me ke akepaʻa.

Akā inā ʻaʻole hiki i ke kauka lapaʻau ke wehe i kekahi a i ʻole nā ​​​​​​mea āpau o ka puʻupuʻu, hiki i kāu hui lapaʻau ke ʻōlelo i hoʻokahi a ʻoi aku paha o kēia mau lāʻau lapaʻau:

• Chemotherapy: Hana kēia lāʻau lapaʻau ma ka luku ʻana i nā hunaola kanesa a pale iā lākou mai ka ulu ʻana.
• ʻO ka Ablation Radiofrequency: Hoʻohana kēia i ka wela mai nā nalu lekiō e pepehi i nā hunaola kanesa.
• ʻO ka Cryosurgical Ablation: Hoʻohana kēia i nā kemika anuanu loa, e like me ka naikokene wai, e luku i nā hunaola kanesa.
• Chemoembolization: Ke ālai nei kēia lāʻau i ke kahe koko i ka maʻi kanesa.

He aha ka wānana no ka ho'ōla ʻana mai kēia maʻi?

ʻO ke kūlana no kekahi me ka glucagonoma e hilinaʻi ʻia i kekahi mau kumu:

• Kou mau makahiki a me kou olakino holoʻokoʻa .
• Ka nui o ka puʻupuʻu kanesa .
• Inā ua pālahalaha ke kanesa a ʻaʻole paha .

Inā ua kaupalena ʻia ke kanesa i ka pancreas, maikaʻi ka nānā ʻana. ʻO ke ʻoki ʻana e wehe i ka puʻupuʻu he mea ho'ōla pinepine ia, akā ʻo kēia ka hihia no kahi 20% wale nō o nā maʻi glucagonoma.

Ma muli o ka laha ʻole o ka glucagonoma, ʻaʻole lawa ka ʻikepili e ʻōlelo pololei ai i ka lōʻihi o kou ola ʻana a i ʻole ka hopena inā laha ka maʻi ʻaʻai. E hiki i kāu hui lapaʻau ke hāʻawi i ka ʻike hou aku e pili ana i kou kūlana kikoʻī.

Hiki ke pale ʻia kēia?

No ka mea, ʻaʻole ʻike ka poʻe ʻepekema i ke kumu o ka hapa nui o nā hihia glucagonoma, ʻaʻohe mea hiki iā ʻoe ke hana e pale aku ai .

Eia nō naʻe, inā ua ʻike ʻia kekahi o kou mau hoahānau mua (nā mākua maoli a me nā kaikaina) me MEN (Multiple Endocrine Neoplasia) , e kamaʻilio me kāu kauka e pili ana i ka hoʻāʻo ʻana i ka genetic. Inā loaʻa iā ʻoe ka MEN, hiki i ka hoʻāʻo ʻana i ka genetic ke kōkua i ka ʻike ʻana i nā maʻi kanesa i ko lākou wā mua.

He mea hoʻonāukiuki loa ka ʻike ʻia ʻana he maʻi kanesa. Inā he mea laha ʻole e like me ka glucagonoma, ʻoi aku ka paʻakikī. Akā e hoʻomanaʻo, aia kāu hui lapaʻau ma laila e pane i kāu mau nīnau a kākoʻo iā ʻoe. Inā manaʻo ʻoe ua mehameha ʻoe ma ka noʻonoʻo a me ka naʻau me ka glucagonoma, e nīnau i kāu kauka e pili ana i nā polokalamu a me nā lawelawe e hiki ke kōkua iā ʻoe.

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

No laila, ʻoiai he maʻi kakaʻikahi loa ka glucagonoma, he mea nui e makaʻala iā ia. ʻOi loa aku inā loaʻa koke iā ʻoe nā hōʻailona e like me ka maʻi diabetes me kahi ʻulaʻula ʻili ʻē (NME), e like me ka mea i ʻōlelo ʻia, e ʻike koke i ke kauka. Inā ʻike mua ʻia, hiki ke mālama ʻia. ʻOiai he mea kakaʻikahi kēia, aia nā lāʻau lapaʻau.

Mai hopohopo, ʻaʻole ʻoe hoʻokahi. E kōkua kāu kauka a me ka hui lapaʻau iā ʻoe ma kēia huakaʻi. E noho ikaika a e hahai i nā ʻōlelo aʻoaʻo a lākou e hāʻawi aku ai iā ʻoe.

ʻO Glucagonoma, maʻi ʻaʻai pancreatic, glucagon, maʻi diabetes, nā hōʻailona ʻili, nā hormones, nā maʻi ʻaʻai laha ʻole