Ke manaʻo koke nei ʻoe i ka poniuniu i kekahi manawa? A i ʻole ke manaʻo nei ʻoe he mea ʻē i loko o kou ʻōpū, ua maloʻo kou waha, a i ʻole ʻaʻole hiki iā ʻoe ke kāohi i kou hanu ʻana? ʻOiai ke ʻano o kēia mau mea he mau mea maʻamau, i kekahi manawa aia kekahi moʻolelo paʻakikī ma hope o lākou. ʻO ia kekahi o kēlā mau kūlana kakaikahi, akā he mea pono ke ʻike, a mākou e kamaʻilio ai i kēia lā.
He aha kēia "Autoimmune Autonomic Ganglionopathy - AAG"? E hoʻomaopopo maʻalahi kākou, ʻeā?
I ka ʻōlelo maʻalahi, ʻo ka "Autoimmune Autonomic Ganglionopathy" a i ʻole "(AAG)" he ʻano ia e hoʻouka hewa ai ka ʻōnaehana pale o ko mākou kino ponoʻī, ʻo ia hoʻi ka ʻōnaehana pale, i kā mākou ʻōnaehana nerve autonomic ponoʻī. E noʻonoʻo, ua like kēia me ka mea e hana ʻia inā hoʻouka hewa ka pūʻali koa i manaʻo ʻia e pale i ko mākou ʻāina i ko mākou mau wahi koʻikoʻi ponoʻī.
Ua like ka ʻōnaehana nerve autonomic o ko kākou kino me kā kākou "ʻōnaehana aunoa". ʻO ia hoʻi, hoʻomalu ia i nā mea he nui e hana aunoa ana, me ka ʻike ʻole ʻia e kākou. Eia kekahi laʻana:
- Kou hanu ʻana
- Ka helu o ka puʻuwai (ka helu o ka puʻuwai)
- Ke koko
- Ka hou ʻana
- ʻO ka hoʻoheheʻe ʻana
ʻAʻole ka nui o kēia mau mea he mea a mākou e noʻonoʻo nei. Hana koke lākou. ʻO ka ʻōnaehana nerve autonomic ka mea e kōkua ai i ka holo pono ʻana o kēlā mau hana aunoa. I loko o AAG, ua hōʻino ʻia kēia ʻōnaehana koʻikoʻi. He kūlana kēia i kapa ʻia ʻo autonomic neuropathy, a i ʻole dysautonomia.
No ke aha e hana ai ko kākou kino ponoʻī i kēia? He aha nā kumu?
ʻO ka ʻoiaʻiʻo, ʻaʻole hiki i nā kauka ke ʻōlelo pololei i ke kumu pololei o kēia `(AAG)`. Akā ua ʻike ʻia kekahi mau ʻoiaʻiʻo.
- ʻO ka hapa nui o ka manawa, ʻo ia hoʻi , ma kahi o ʻekolu o kēlā me kēia ʻelima mau kānaka e loaʻa ana i ka "AAG", kū mai kēia ʻano ma hope o ka hoʻōla ʻana mai kahi maʻi ʻē aʻe, a i ʻole ma hope o kahi maʻi. Ua like ia me ka hopena ʻaoʻao o kēlā maʻi.
- Hiki i kekahi poʻe ke loaʻa i kahi maʻi i kapa ʻia ʻo paraneoplastic AAG. ʻO kēia ka wā e hana ai kekahi mau antibodies a kā mākou ʻōnaehana pale ma ke ʻano he pane i kahi maʻi e like me ke kanesa e hele a hoʻouka i ka ʻōnaehana nerve autonomic.
He aha ka hopena o kēia i ko kākou kino? E nānā pono kākou.
Loaʻa i kā mākou ʻōnaehana nerve autonomic nā hui o nā hunaola nerve i kapa ʻia ʻo autonomic ganglia . Ua like kēia me nā hui kaʻaahi. Hoʻouna ʻia a loaʻa ʻia nā leka like ʻole ma o kēia mau mea. He mea nui kēia hoʻololi ʻana o nā leka no nā hana aunoa o ko mākou kino, ʻo ia hoʻi, nā hana a mākou e kāohi ʻole ai (reflexes), e kāohi ʻia.
I loko o AAG, hoʻouka kā mākou ʻōnaehana pale i nā mea loaʻa kikoʻī i loko o kēia mau ganglia. ʻO nā mea loaʻa nā wahi e loaʻa ai nā leka. No laila, ke hōʻino ʻia kēia mau mea loaʻa, ʻaʻole e hele pono kēlā mau leka aʻalolo. ʻO ka hopena, ʻo nā mea e hana koke ʻia, e like me ke kaohi ʻana i ke koko a me ka hou ʻana, e hoʻomaka e hele hewa.
E noʻonoʻo, inā e hemo kahi `pili` i loko o ka `papa kuapo nui` o kou home, ʻaʻole e ʻā nā kukui, ʻaʻole e milo ka peʻahi, a pēlā aku. Pēlā nō ia me kēia.
Pehea ka maʻamau o kēia kūlana i kapa ʻia ʻo `(AAG)`?
He maʻi laha ʻole kēia. No ka laʻana, ma ʻAmelika Hui Pū ʻIa, he 100 wale nō kānaka i ʻike ʻia me kēia maʻi i kēlā me kēia makahiki. No laila, he mea laha ʻole ia ma Sri Lanka. No laila, mai makaʻu ʻoe i kou lohe ʻana i kēia, ʻaʻole kēia he mea e hana ʻia i nā poʻe he nui.
He aha nā hōʻailona o `(AAG)`? Pehea mākou e ʻike ai iā lākou?
Hiki ke ʻokoʻa nā hōʻailona o kēia maʻi mai kēlā kanaka a kēia kanaka. ʻAʻole e ʻike nā kānaka a pau i nā hōʻailona like. ʻO nā hōʻailona maʻamau:
- Paʻa ʻōpū: ʻO ke ʻano kēia o ka paʻa ʻōpū a me ka paʻakikī e hele i ka lua.
- Nā haumāna i hoʻonui ʻia (nā haumāna a Adie): I kekahi manawa hoʻokahi wale nō ka haumāna o hoʻokahi maka e hoʻonui ʻia a hiki ke maʻalahi i ka mālamalama.
- Waha maloʻo a maka maloʻo paha: He manaʻo me he mea lā ʻaʻohe lawai a waimaka paha i loko o nā maka.
- Nāwaliwali / Syncope: Hiki paha iā ʻoe ke nawaliwali koke me ke kumu ʻole.
- Neurogenic orthostatic hypotension: He manaʻo o ka poniuniu a me ke poʻo māmā i ke kū ʻana mai kahi noho a i ʻole ke ala ʻana mai kahi moe. Hana ʻia kēia e ka emi koke ʻana o ke koko .
- Paʻakikī i ka mimi / Paʻa ʻana o ka mimi: ʻO ka manaʻo o ka hiki ʻole ke mimi ʻoiai inā makemake ʻoe e mimi.
Ma kahi o ʻelua o kēlā me kēia ʻekolu kānaka me AAG ua ʻike ʻia he kiʻekiʻe nā pae o nā antibodies i kapa ʻia ʻo ganglionic acetylcholine receptor antibodies (g-AChR antibodies) i loko o ko lākou koko. Manaʻo nā kānaka noiʻi aia kahi pilina ma waena o nā pae o kēia mau antibodies g-AChR a me ke koʻikoʻi o nā hōʻailona.
Pehea e ʻike ai ke kauka i kēia maʻi `(AAG)`?
Inā loaʻa iā ʻoe nā hōʻailona e like me kēia, ʻo ka mea mua a ke kauka e hana ai, ʻo ia ke nīnau iā ʻoe e pili ana i kāu mau hōʻailona a nānā iā ʻoe. A laila, hana paha lākou i kekahi mau hoʻokolohua kūikawā e nānā i ka hana o kāu ʻōnaehana nerve autonomic.
Eia kekahi o lākou:
- Nā hoʻāʻo ʻōnaehana hoʻoheheʻe: No ka laʻana, he hoʻāʻo "ʻOki ʻana o ka ʻōpū". Hoʻāʻo kēia i ka wikiwiki o ka haʻalele ʻana o ka meaʻai i kou ʻōpū.
- ʻO ka hoʻāʻo reflex axon sudomotor quantitative (QSART): Ke ana kēia i ka maikaʻi o ka hana ʻana o kāu mau kelepa hou.
- `Hoʻāʻo hou wela`: Hana ʻia kēia hoʻāʻo e ʻike ai inā ua emi a piʻi paha ka hou ʻana o ke kino, a he aha ke kumu.
- `Hoʻāʻo papa ʻaina hiʻohiʻona`: Ma kēia hoʻāʻo, e hoʻomoe ʻia ʻoe ma luna o kahi moena kūikawā a e hoʻohuli mālie ʻia ka moena i luna, e nānā ana i ke ʻano o ka loli ʻana o kou puʻuwai a me ke koko.
- Urinalysis: Hiki i kēia ke hāʻawi iā ʻoe i kahi manaʻo no ka hana maikaʻi o kāu ʻōpū mimi.
Eia kekahi, hiki ke hana ʻia kahi hoʻāʻo koko e nānā i ka pae o nā `(g-AChR antibodies)` i ʻōlelo ʻia ma luna . Inā kiʻekiʻe ka pae o kēia antibody, he hōʻike hou aʻe ia he `(AAG)` paha. Eia nō naʻe, ʻokoʻa kēia hoʻāʻo koko mai ka `AChR antibody test`, kahi i hoʻohana ʻia e nānā i kahi maʻi neurological ʻē aʻe i kapa ʻia ʻo `(Myasthenia Gravis).`
Aia kekahi lāʻau lapaʻau no kēia? He aha ka mea hiki ke hana ʻia?
No ka mea, he maʻi laha ʻole ʻo AAG, ʻaʻohe kūlana maʻamau, "ʻo kēia ka lāʻau lapaʻau". ʻO ka hapa nui o ka manawa , ʻo ke kiko ka mālama ʻana i nā hōʻailona.
Hoʻokumu nā kauka i kahi hoʻolālā lapaʻau ma ka hoʻohui ʻana i nā ʻano lapaʻau like ʻole. ʻO kekahi o lākou:
- Hoʻololi plasma / plasmapheresis: Pili kēia i ka lawe ʻana i kekahi o kou koko, wehe i nā mea ʻino e like me nā antibodies mai ka plasma, a laila hoʻihoʻi i ke koko i hoʻomaʻemaʻe ʻia i kou kino.
- ʻO ka lāʻau lapaʻau intravenous immunoglobulin (IVIG): ʻO kēia ka hāʻawi ʻana i kou kino i ka immunoglobulin (kahi ʻāpana o ka plasma e hakakā ana i nā maʻi) i lawe ʻia mai nā poʻe olakino. Kōkua kēia i ka hōʻoluʻolu ʻana i ka ʻōnaehana pale.
- ʻO nā corticosteroids intravenous: Hāʻawi ʻia nā dosis kiʻekiʻe o nā steroid anti-inflammatory, e like me ka methylprednisolone (Solu-Medrol®) a i ʻole hydrocortisone (Solu-Cortef®), ma o ka inikini.
- Nā lāʻau lapaʻau immunosuppressive: Hana kēia ma ka hōʻemi ʻana i ka hana nui o ka ʻōnaehana pale. No ka laʻana, nā lāʻau lapaʻau e like me rituximab (Rituxan®).
Aia kekahi ala e pale aku ai i kēia kūlana `(AAG)`?
ʻAʻohe ala i hōʻoia ʻia e pale aku ai i kēia, no ka mea, he mea kakaikahi loa ia. Eia nō naʻe, hiki i kekahi mau maʻi, e like me ke kanesa, ke hoʻonui iki i kou pilikia o ka loaʻa ʻana. Inā ua loaʻa iā ʻoe ke kanesa, eia kekahi mau mea hiki iā ʻoe ke hana e mālama i kou olakino holoʻokoʻa:
- Hoʻoikaika kino mau: ʻoi aku ka hoʻoikaika kino aerobic.
- Inā loaʻa iā ʻoe ka `(Dysautonomia)`, hiki i ka ʻai ʻana i ka meaʻai kiʻekiʻe i ka paʻakai a me nā wai ke kōkua i ka kaohi ʻana i kou koko. (He mea kēia āu e kūkākūkā ai me kāu kauka.)
- Ka Hoʻokele ʻana i ke Koʻikoʻi: E hoʻohana i nā ʻano olakino e like me ka hoʻoikaika kino, ke kamaʻilio ʻana me kahi hoaaloha, a me ke kākau ʻana i loko o kahi puke pai.
- E hiamoe maikaʻi: E hiamoe ma ka liʻiliʻi he 7-8 mau hola i ka lā.
- Ke komo ʻana i nā lole hoʻopili: Kōkua kēia i ka hoʻomaikaʻi ʻana i ke kahe koko a hōʻemi i ka pehu (ʻoi aku hoʻi i nā wāwae).
Hiki ke ho'ōla loa ʻia kēia kūlana `(AAG)`? He aha ka mea e hiki mai ana?
Pono wau e ʻōlelo, ʻoiai ke kaumaha nei au,ʻAʻole hiki ke ho'ōla ʻia ka AAG. Eia nō naʻe, hiki i ka nui o ka poʻe ke hoʻokele maikaʻi i ko lākou mau hōʻailona i ka wā lōʻihi. ʻO ka mea kupanaha, ma kahi o hoʻokahi o ʻekolu poʻe me AAG e hoʻomaikaʻi mālie me ka ʻole o ka lāʻau lapaʻau. Eia nō naʻe, pono ka hapa nui o ka poʻe e kū i kekahi mau hōʻailona i ko lākou ola.
He aha hou kāu e makemake ai e nīnau aku i ke kauka?
Inā kānalua ʻoe he ʻano kou e like me kēia, he mea nui loa e ʻike i ke kauka maikaʻi a nīnau e pili ana i kēia mau mea:
- He aha ke kumu kūpono loa o koʻu mau hōʻailona?
- He aha nā hoʻokolohua e pono iaʻu e hana e ʻike pono ai inā loaʻa iaʻu ka `(AAG)`?
- He aha nā koho lapaʻau no ka `(AAG)`?
- He aha ka likelika e emi ai nā hōʻailona o `(AAG)` ʻoiai me ka ʻole o ka lāʻau lapaʻau?
- He aha nā hoʻololi o ke ʻano o ka nohona e pono iaʻu e hana ai e hoʻomaikaʻi i koʻu olakino holoʻokoʻa?
He aha nā kūlana palekana ʻē aʻe e pili ana me ka neuropathy autonomic?
He mea kūikawā ʻo ``(AAG)`` no ka mea, ma kekahi ʻaoʻao, he maʻi ia e pili ana i ka ʻōnaehana pale kino, a ma kekahi ʻaoʻao, he maʻi autoimmune o ka ʻōnaehana nerve.
Eia kekahi, aia kekahi mau maʻi ʻē aʻe e pili ana i ka ʻōnaehana pale e hoʻonui ai i ka pilikia o ka hoʻomohala ʻana i ka "dysautonomia" (nā maʻi o ka ʻōnaehana nerve autonomic). ʻO kekahi mau laʻana:
- Maʻi Celiac
- `(Guillain-Barré syndrome)` (Guillain-Barré syndrome)
- `(Lupus / systemic lupus erythematosus)` (Maʻi Lupus)
- `(ʻO ka rumatika)` (ʻO ka rumatika)
- `(Sjögren's Syndrome)` (Sjögren's Syndrome)
No laila hiki iā ʻoe ke ʻike, he mau kūlana kēia e hiki ke hoʻopili ʻia.
ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)
ʻAe, i kēia manawa ua ʻike ʻoe he maʻi laha ʻole ka `(Autoimmune Autonomic Ganglionopathy - AAG)` kahi e hoʻouka ai kā mākou ʻōnaehana pale ponoʻī i kā mākou ʻōnaehana nerve autonomic. Hiki i nā hōʻailona ke komo pū me ka nalowale koke o ka ʻike, ka emi ʻana o ke `kaomi` i ke kū ʻana, a me nā pilikia ʻōpū.
Mai hopohopo ke lohe ʻoe i kēia. He mea kākaʻikahi loa kēia. Eia kekahi, hoʻohana ʻia nā lāʻau immunosuppressant a me ka 'plasmapheresis' ma ke ʻano he lāʻau lapaʻau no kēia. ʻOiai ʻaʻole hiki ke ho'ōla piha ʻia, hiki i ka hapa nui o ka poʻe ke kāohi pono i nā hōʻailona a ola i kahi ola maʻamau.
ʻO ka mea nui loa, ʻo ia ke ʻike koke i ke kauka kūpono inā loaʻa iā ʻoe kēia mau hōʻailona. Inā ʻike mua ʻoe iā lākou, hiki iā ʻoe ke hoʻomaka i ka mālama ʻana a hōʻemi i ka hopena i kou ola. ʻAʻole ʻoe hoʻokahi, a aia nā kauka e hiki ke kōkua.
ʻO Autoimmune Aneurysmal Ganglionopathy, AAG, ʻŌnaehana Nervous Autoimmune, ʻŌnaehana Palekana, Maʻi Neurological, Nā Hōʻailona, Lapaʻau
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