Ke manaʻo nei ʻoe i kekahi manawa ua nāwaliwali kou mau wāwae, paʻakikī iā ʻoe ke ala aʻe mai kahi noho, luhi ʻoe i ka piʻi ʻana i nā alapiʻi? A i ʻole luhi ʻoe me nā hōʻailona e like me nā maka kaumaha, ka waha maloʻo, a me ka ʻike pālua? ʻAʻole paha kēia he luhi wale nō. I kēia lā e kamaʻilio mākou e pili ana i kahi kūlana āu i lohe ʻole ai, akā he mea pono ke ʻike e pili ana, ʻo Lambert-Eaton Myasthenic Syndrome, a i ʻole ``(LEMS)`` e like me kā mākou e kapa nei no ka pōkole.
He aha ka Lambert-Eaton Myasthenic Syndrome (LEMS)?
I ka ʻōlelo maʻalahi, ʻo Lambert-Eaton Myasthenic Syndrome (LEMS) he maʻi autoimmune. I kēia manawa ke noʻonoʻo nei paha ʻoe i ke ʻano o ka maʻi autoimmune . Loaʻa i ko mākou kino kahi ʻōnaehana pale. ʻO kēia ka mea e pale ai iā mākou ma ke kaua ʻana i nā germs e hoʻoulu ai i ka maʻi mai waho. Ua like ia me ka pūʻali koa ma ko mākou ʻāina. Akā i kekahi manawa hele hewa kēia ʻōnaehana pale. A laila hoʻomaka ia e hoʻouka i kā mākou mau cell olakino ponoʻī. ʻO ia ka mea e hana ʻia ma LEMS.
Ma kēia `(LEMS)`, hoʻouka kā mākou ʻōnaehana pale i ka pilina ma waena o ko mākou mau aʻalolo hoʻokele a me nā ʻiʻo . I ka pololei, ua kapa ʻia kēia pilina ʻo `neuromuscular junction`. Ke hōʻino ʻia kēia junction, ua pilikia ka hoʻoili ʻana o nā leka mai nā aʻalolo i nā ʻiʻo. ʻO kēia ka manawa e nāwaliwali ai nā ʻiʻo.
Hoʻomaka pinepine kēia nāwaliwali ma kou mau ʻūhā luna , akā hiki ke laha mālie i kou mau lima, ka hanu ʻana, ka moni ʻana, a me nā ʻiʻo e ʻōlelo ana.
ʻO wai ka mea i ʻoi aku ka paʻakikī ke loaʻa i kēia `(LEMS)`?
ʻElua mau hui nui e hana ana i ka `(LEMS)`.
1. Pili me ke kanesa: Ma kahi o 60% o ka poʻe me LEMS he ʻano kanesa ko lākou. ʻO ke ʻano maʻamau ka maʻi kanesa māmā liʻiliʻi . ʻO ka mea kupanaha, i kekahi manawa hiki ke ʻike ʻia nā hōʻailona o LEMS i nā mahina a i ʻole nā makahiki ma mua o ka ulu ʻana o ke kanesa. ʻO ka hapa nui o kēia poʻe ma luna o 60 mau makahiki, he kāne lākou, a ua hoʻohana i nā huahana paka.
2. ʻAʻohe maʻi kanesa: ʻO ke koena 40% o ka poʻe me LEMS ʻaʻole i ʻike ʻia ka maʻi kanesa. Hoʻomaopopo pinepine ʻia kēia poʻe ma kahi o 35 mau makahiki a ma kahi o 60 mau makahiki.
He maʻi laha ʻole ʻo LEMS i waena o nā keiki ʻōpio.
Ua pili wale nō anei ka maʻi ʻaʻai māmā liʻiliʻi (LEMS) me kēia?
ʻAʻole. ʻO ke kanesa māmā liʻiliʻi ke ʻano maʻamau o ke kanesa, akā hiki ke pili pū kekahi mau ʻano kanesa ʻē aʻe me LEMS. ʻO nā hiʻohiʻona e komo pū me ke kanesa prostate, thymoma, a me nā maʻi lymphoproliferative. No ka mea hiki ke ʻike ʻia nā hōʻailona o LEMS ma mua o ka ʻike ʻia ʻana o nā hōʻailona kanesa, he mea nui e loaʻa nā hoʻokolohua kūpono e nānā ai no ke kanesa inā ʻike kāu kauka iā ʻoe me LEMS.
Ua hoʻokumu ʻia ʻo LEMS e nā loli genetic?
Manaʻo nā kānaka ʻepekema he pilina genetic paha ko nā maʻi autoimmune ma ke ʻano laulā. ʻOiai he maʻi autoimmune nō hoʻi ʻo LEMS, hiki nō hoʻi ke loaʻa iā ia kahi mana genetic, akā ʻaʻole i maopopo loa kēia.
Pehea ka maʻamau o ka `(LEMS)`?
He maʻi laha ʻole ʻo LEMS. Ua manaʻo ʻia ua hoʻopilikia kēia maʻi i kahi 2.8 miliona mau kānaka ma ka honua holoʻokoʻa. Ma ʻAmelika Hui Pū ʻIa, aia ma kahi o 400 mau hihia i hōʻike ʻia o LEMS. ʻOiai ʻaʻohe helu kikoʻī e pili ana i ka nui o nā kānaka ma ko mākou ʻāina, ua maopopo he maʻi laha ʻole kēia.
He aha nā hōʻailona o ka `(LEMS)`?
E nānā kākou i nā hōʻailona o `(LEMS)` pākahi. ʻAʻole hiki mai kēia mau hōʻailona a pau i ka manawa like i nā kānaka a pau, hiki ke ʻike mua ʻia kekahi mau hōʻailona, a hiki ke ʻike hope ʻia kekahi.
- Ka nāwaliwali o nā ʻiʻo a me ka luhi: ʻoi aku hoʻi ma nā wāwae o luna.
- Paʻakikī ke hele wāwae: Paʻakikī ke piʻi i nā alapiʻi a i ʻole ke ala ʻana mai kahi noho.
- ʻEha a ʻoʻoleʻa paha nā ʻiʻo.
- Manaʻo o ka mae ʻana o nā lālā.
- Nā maka maka e hāʻule ana (ptosis): Hiki i kēia ke nānā aku e like me ka hiamoe.
- Ke ʻike ʻana i ʻelua mau mea i ka manawa hoʻokahi (diplopia).
- Waha maloʻo a me nā maka (xerostomia): emi ka salivation, emi ka waimaka ma nā maka.
- Ka paʻa ʻana o ka ʻōpū.
- Ua emi ka hou ʻana.
- Ka emi ʻana o ke kaumaha.
- Paʻakikī i ka mimi ʻana.
- Ka hana ʻino ʻana o ka erectile i nā kāne.
- Paʻakikī i ka ʻōlelo ʻana (dysarthria) a me ka paʻakikī i ka moni ʻana (dysphagia): ʻO kēia nā hōʻailona e ulu aʻe ma hope o ka maʻi.
- Ka paʻakikī o ka hanu ʻana (dyspnea) a me ka pilikia hanu: He hōʻailona koʻikoʻi kēia a kakaikahi hoʻi e hiki ke kū mai i nā pae hope o ka maʻi.
Mea nui: Inā loaʻa iā ʻoe kekahi a ʻoi aku paha o kēia mau hōʻailona, mai manaʻo he LEMS ia. Eia nō naʻe, inā hoʻomau kēia mau hōʻailona, he mea naʻauao ke ʻimi i ke aʻo lapaʻau.
Pehea ka ʻike pinepine ʻia ʻana o ka LEMS a me ka holomua?
ʻO ka maʻamau, hoʻopilikia mua ʻo LEMS i nā ʻiʻo o nā ʻūhā . A laila hiki iā ia ke hoʻopilikia i nā ʻiʻo poʻohiwi, nā ʻiʻo o nā lima a me nā wāwae, nā ʻiʻo e kōkua ai i ka ʻōlelo a me ka moni ʻana, a me nā ʻiʻo o nā maka.
I ka hoʻomaka ʻana o ka maʻi, paʻakikī paha iā ʻoe ke ala aʻe mai kahi noho, piʻi i nā alapiʻi a i ʻole nā puʻu, a i ʻole e hana i nā hana ikaika e pono ai ka hoʻohana ʻana i kou mau wāwae.
ʻO ka maʻamau, ulu mālie nā hōʻailona, ma luna o kekahi mau pule a i ʻole mau mahina . Eia nō naʻe, inā loaʻa iā ʻoe ka LEMS me ka maʻi kanesa, hiki ke ulu koke nā hōʻailona.
He aha ke kumu o LEMS?
E like me kā mākou i kūkākūkā ai ma mua, he maʻi autoimmune ʻo LEMS. ʻO ia hoʻi, hoʻouka nā pale ponoʻī o kou kino (nā antibody) i kāu mau ʻiʻo olakino ponoʻī. I loko o LEMS, hoʻouka kēia mau antibodies i kahi e hui ai nā hunaola nerve a me nā olonā ʻiʻo, ʻo ia hoʻi ka neuromuscular junction. ʻO ke kikoʻī, hoʻouka lākou i nā kahawai calcium ma laila. He mea nui kēia mau kahawai calcium no ka hoʻouna ʻana i nā leka nerve.
E noʻonoʻo e like me kahi lālani o nā domino. Hoʻomaka kēia hoʻouka kaua i kahi kaulahao o nā hanana:
1. Hoʻopaʻa nā antibodies i nā kahawai calcium ma nā wēlau o nā hunaola nerve a ālai iā lākou.
2. Ke hoʻemi ʻia nā kahawai calcium, e emi ana ka nui o ka acetylcholine, kahi neurotransmitter, i hoʻokuʻu ʻia. He kemika koʻikoʻi kēia acetylcholine no ka hana ʻana o nā ʻiʻo.
3. Ke hoʻokuʻu ʻole ʻia ka acetylcholine lawa, ʻaʻole e hele pono nā "leka" i nā olonā ʻiʻo. A laila ʻaʻole hiki i kou mau ʻiʻo ke hana piha.
4. ʻO ka hopena, ʻaʻole hana maʻamau nā ʻiʻo, e hopena ana i ka nāwaliwali.
Eia ke ʻano o ka hoʻomohala ʻana o ka poʻe me ka maʻi kanesa māmā liʻiliʻi i ka LEMS: Ke loaʻa iā ʻoe ka maʻi kanesa i loko o kou kino, loaʻa i nā antibodies kahi hōʻailona e "hele i ke kaua." Akā ma kahi o ka hoʻouka ʻana i nā hunaola kanesa (ʻo ia hoʻi, nā kahawai calcium ma ka ʻili o nā hunaola kanesa), hoʻouka hewa kēia mau antibodies i nā kahawai calcium ma nā wēlau o nā hunaola nerve. A laila hana ʻia ke kaulahao like o nā hanana.
Manaʻo nā kānaka ʻepekema ʻo ke kumu o LEMS i ka poʻe me ka maʻi ʻaʻai māmā he hana hewa wale nō ia o ka ʻōnaehana pale. Akā, ʻaʻole maopopo i ke kumu o kēia.
Pehea e ʻike ai i ka `(LEMS)`?
ʻO ka hana mua i ka ʻike ʻana iā LEMS, ʻo ia ke nānā pono ʻana o kāu kauka iā ʻoe. E pili pinepine kēia i kahi neurologist, a i ʻole ke kauka loea i nā maʻi o nā aʻalolo a me nā ʻiʻo. ʻO kahi hapa o kēia kaʻina hana e pili ana i ka nīnau ʻana e pili ana i kāu mau hōʻailona, kou mōʻaukala olakino, a me nā lāʻau lapaʻau āu e lawe ai. Hana ʻia kahi hoʻokolokolo neurological kikoʻī e nānā i nā ʻano o ka nāwaliwali a me nā hōʻailona e kūlike me LEMS.
ʻO ka hana aʻe, ʻo ia ka hoʻāʻo ʻana. ʻO nā hoʻāʻo no `(LEMS)` e komo pū ana me:
- Hoʻāʻo koko: Hiki i kēia ke nānā inā he mau antibodies anti-calcium channel kou i loko o kou koko. Ma kahi o 85% o ka poʻe me LEMS he mau antibodies kēia i loko o ko lākou koko.
- Electromyography (EMG): Nānā kēia hoʻāʻo i ka maikaʻi o ka hana pū ʻana o kou mau ʻiʻo a me nā aʻalolo. Ma LEMS, loaʻa i ka hoʻāʻo EMG nā ʻike kikoʻī loa, no laila hiki ke kōkua i ka hōʻoia ʻana i ka hōʻailona.
- ʻO ke kiʻi X-ray māmā `(X-ray)` a i ʻole CT scan `(CT scan)` a i ʻole MRI scan `(MRI)` (o ka umauma): No ka mea, hiki ke ulu kahi kūlana i kapa ʻia ʻo `(LEMS)` i loko o ka poʻe me ka maʻi ʻaʻai māmā liʻiliʻi, e ʻimi kēia mau hoʻokolohua i nā hōʻailona o ka maʻi ʻaʻai māmā.
Hiki i nā hōʻailona o LEMS ke hoʻomaka i nā mahina a i ʻeono mau makahiki ma mua o ka ulu ʻana o ke kanesa. No laila, ma hope o ka ʻike ʻia ʻana o LEMS, pono ʻoe e hana i nā hoʻokolohua kanesa i kēlā me kēia ʻekolu a ʻeono mahina no ka liʻiliʻi o ʻelua mau makahiki. E paipai kāu kauka i kahi papa hana hoʻokolohua kūpono iā ʻoe.
Pehea e mālama ʻia ai ʻo LEMS?
Aia kekahi mau lāʻau lapaʻau no LEMS.
- Ke mālama ʻana i ke kanesa kumu: Inā pili ʻo LEMS i kahi kanesa, e like me ke kanesa māmā cell liʻiliʻi, hiki i ka mālama ʻana i kēlā kanesa ke hōʻemi i nā hōʻailona LEMS. Hiki i nā lāʻau lapaʻau kanesa ke komo pū me ka chemotherapy, radiation therapy, a/a i ʻole ke ʻoki kino. Aia kēia i ke ʻano o ke kanesa a me nā ʻōlelo a kāu kauka.
- Nā lāʻau lapaʻau kūikawā no (LEMS): ʻO kēia mau mea nā lāʻau lapaʻau e hōʻoluʻolu i nā hōʻailona a me nā lāʻau lapaʻau e kaohi ai i ka ʻōnaehana pale.
Lapaʻau no nā hōʻailona
- ʻAmifampridine (Firdapse®), Guanidine: Hoʻonui kēia mau lāʻau i ka ikaika o nā ʻiʻo. Hoʻonui lākou i ka hoʻokuʻu ʻana o ka neurotransmitter acetylcholine ma ka neuromuscular junction, e hoʻomaikaʻi ana i nā hōʻailona i nā ʻiʻo.
- Pyridostigmine (Pyridostigmine - Mestinon®): Hana ʻo Pyridostigmine ma ka pale ʻana i ka haki ʻana o ka acetylcholine ma ka neuromuscular junction. Hoʻonui pū kēia i ka hōʻailona acetylcholine a hoʻomaikaʻi i ka ikaika o nā ʻiʻo. Hāʻawi ʻia ʻo Pyridostigmine i kekahi manawa me ka amifampridine e hoʻomaikaʻi i ka hana ʻana o nā ʻiʻo a mālama i nā hōʻailona o LEMS e like me ka waha maloʻo, nā maka maloʻo, ka paʻa ʻana o ka ʻōpū, ka hana moekolohe, a me ka emi ʻana o ka hou ʻana. Eia nō naʻe, ʻo ka hoʻohana ʻana i ka pyridostigmine no LEMS he hoʻohana ma waho o ka lepili, ʻo ia hoʻi, ʻaʻole i ʻae ka U.S. Food and Drug Administration (FDA) i ka lāʻau no kēia kūlana.
Lapaʻau hoʻololi i ka pale ʻana
- Nā lāʻau pale kino:Kōkua kēia mau lāʻau lapaʻau i ka hoʻōki a i ʻole kaohi ʻana i ka hana "pale" o kāu ʻōnaehana pale. Hiki i kāu kauka ke kuhikuhi i kēia mau lāʻau lapaʻau inā ʻaʻole nā lāʻau lapaʻau ʻē aʻe e kaohi i kāu mau hōʻailona. No ka laʻana, hiki ke hāʻawi ʻia kahi lāʻau i kapa ʻia ʻo prednisone me a me ka ʻole o ka azathioprine a i ʻole cyclosporine.
- Hoʻololi plasma: Ma kēia kaʻina hana, hoʻokaʻawale kahi mīkini i ka ʻāpana wai o kou koko (plasma) mai nā hunaola koko. Aia kāu mau antibodies i loko o kēia plasma. Ma hope o ka hoʻokaʻawale ʻana, hoʻihoʻi ʻia ke koko i loko o kou kino. E noʻonoʻo paha kāu kauka i kēia lāʻau lapaʻau inā loaʻa iā ʻoe nā hōʻailona koʻikoʻi a pono ʻoe i ka lāʻau lapaʻau wikiwiki.
- Nā Immunoglobulins: No kekahi poʻe, hiki i ka mālama ʻana i ka immunoglobulin kiʻekiʻe ke kōkua i ka pale ʻana i ka maʻi mai ka ʻino ʻana.
Hiki ke pale ʻia ka LEMS?
ʻAʻole maopopo loa ke kumu kikoʻī o LEMS, no laila ʻaʻole maopopo ia inā he ala e pale aku ai i ka maʻi.
Eia nō naʻe, ʻoiai hiki i ka LEMS ke ulu i loko o ka poʻe me ka maʻi kanesa o ka māmā, hiki iā ʻoe ke kōkua i ka pale ʻana i ka maʻi kanesa o ka māmā ma o:
- Mai puhi paka. Inā ʻoe e hoʻohana i nā huahana paka, e noi i kāu kauka no ke kōkua e haʻalele.
- E pale i ka uahi lua.
- E nānā i kou home no ke kinoea radon. ʻO ka hoʻopili lōʻihi ʻana i ke kinoea radon hiki ke hoʻonui i kou pilikia o ka maʻi kanesa māmā.
Hiki iā ʻoe ke hana i nā hana e mālama ai i kahi nohona olakino:
- E ʻai i nā huaʻai a me nā mea kanu hou aʻe. E hahai i ka papaʻai Mediterranean a i ʻole DASH.
- E mālama i ke kaumaha olakino.
- E hoʻoikaika kino e like me ka ʻōlelo a kāu kauka.
- E hiamoe lawa - ʻehiku a ʻeiwa hola paha.
- E hōʻemi i kou kaumaha. Hiki i ke kaumaha ke hoʻonui i nā hōʻailona.
- Mai ʻauʻau i ka wai wela. Hiki i ka wela ke hoʻonui i nā hōʻailona o LEMS.
- E ʻike pinepine i kāu kauka, a e hoʻomaopopo koke iā lākou inā loli kou olakino a i ʻole inā loaʻa iā ʻoe nā hōʻailona hou.
Hiki ke ho'ōla loa ʻia ka LEMS?
ʻO ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no LEMS. Ua hōʻike ʻia ka mālama ʻana i ke kanesa ma lalo e pili ana me LEMS e hoʻomaikaʻi i nā hōʻailona o LEMS. Hiki i nā lāʻau immunosuppressive ke kōkua i ka kaohi ʻana i ka hoʻouka kaua autoimmune, a aia kekahi mau lāʻau lapaʻau e hiki ke kōkua i ka hōʻoluʻolu ʻana i nā hōʻailona ma ka hoʻonui ʻana i ka nui o ka acetylcholine i nā hopena nerve a me ka hoʻomaikaʻi ʻana i ka ikaika o nā ʻiʻo.
Inā loaʻa iā ʻoe ka LEMS me ka ʻole o ke kanesa, hiki iā ʻoe ke manaʻo i kahi ola maʻamau. Inā loaʻa iā ʻoe ka LEMS a me ke kanesa, hiki ke hoʻomaikaʻi ʻia kāu mau hōʻailona i ka hala ʻana o ka manawa (ʻoiai ʻaʻole i piha loa) inā ʻike ʻia a mālama ʻia ke kanesa i ka wā mua.
ʻO ke ʻano o kou ola e hilinaʻi nui ʻia ana i ke koʻikoʻi o kāu mau hōʻailona.
Āhea wau e hoʻokaʻaʻike aku ai i kaʻu kauka inā loaʻa iaʻu ka `(LEMS)`?
Inā he loli i kou olakino, a inā e ʻoi aʻe ka ʻino o kou mau hōʻailona, e kelepona i kāu kauka i kēlā me kēia manawa . Mai makaʻu, mai hopohopo. Aia nā kauka ma laila e kōkua iā ʻoe.
He aha ka ʻokoʻa ma waena o `(LEMS)` a me `(Myasthenia Gravis - MG)`?
ʻO ka Lambert-Eaton myasthenic syndrome (LEMS) a me ka myasthenia gravis (MG) kekahi mau hōʻailona like. Hoʻopilikia nā mea ʻelua i ka nāwaliwali o nā ʻiʻo a me ka luhi.
Eia nō naʻe, ʻo ka myasthenia gravis (MG) maʻamau e hoʻopilikia mua i nā ʻiʻo a puni kou mau maka a me nā ʻiʻo i hoʻohana ʻia no ka moni ʻana. Ma hope mai, hiki iā ia ke hoʻopilikia i nā ʻiʻo maʻamau, me nā ʻiʻo o ka hanu ʻana a me nā ʻiʻo o nā lālā.
Hoʻomaka pinepine ʻo LEMS me ka nāwaliwali ma kou mau ʻūhā luna, a laila neʻe i kou mau lima. Hiki i ka LEMS ke hoʻonāwaliwali i kou mau ʻiʻo maka, akā ʻoi aku ka maʻalahi ma mua o ka myasthenia gravis (MG).
I ka myasthenia gravis (MG), hiki i ka nāwaliwali nui o nā ʻiʻo hanu (nā ʻiʻo e kōkua iā ʻoe e hanu) ke make. He mea kākaʻikahi kēia ma LEMS.
ʻAʻole hōʻike pinepine ka poʻe me ka myasthenia gravis (MG) i nā hōʻailona he nui o ka hana ʻole o ka ʻōnaehana nerve autonomic (e.g., nā maka maloʻo, ka waha maloʻo, ka paʻa ʻana o ka ʻōpū, ka emi ʻana o ka hou ʻana, ka hana moekolohe). I ka hoʻohālikelike ʻana, loaʻa pinepine i ka poʻe me LEMS kēia mau hoʻopiʻi, a hiki i kēia mau hōʻailona ke kōkua i ka hoʻokaʻawale ʻana i nā maʻi ʻelua.
ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)
I kēia manawa, ua maopopo paha iā ʻoe ʻo ka Lambert-Eaton Myasthenic Syndrome (LEMS) kahi maʻi i hoʻokumu ʻia e kahi ʻōnaehana pale ikaika loa. ʻAʻole hoʻouna kāu mau hunaola aʻalolo i nā leka lawa i kou mau olonā ʻiʻo e "ʻaelike." ʻO ka hopena, he nāwaliwali nā ʻiʻo a me nā hōʻailona ʻē aʻe.
E hoʻomanaʻo, ma kahi o 60% o ka poʻe me LEMS he maʻi ʻaʻai māmā liʻiliʻi. Inā loaʻa iā ʻoe ka LEMS akā ʻaʻohe maʻi ʻaʻai, e hōʻoia e loaʻa iā ʻoe a me kāu kauka nā hoʻokolohua maʻi ʻaʻai i kēlā me kēia mau mahina no ʻelua mau makahiki ma hope o ka hōʻoia ʻana.
Aia nā lāʻau lapaʻau. ʻO kēia mau mea e pili ana i ka mālama ʻana i ke kanesa inā loaʻa, a me ka lawe ʻana i nā lāʻau lapaʻau e hoʻomaikaʻi ai i ka launa ʻana o nā cell nerve-muscle fiber. ʻO ka hoʻomaikaʻi ʻana i ke kamaʻilio ʻana hiki ke hoʻomaikaʻi i ka ikaika o nā ʻiʻo a me nā hōʻailona ʻē aʻe.
Manaʻolana wau he mea pono kēia ʻike iā ʻoe.E hoʻomanaʻo mau, inā he mau hopohopo olakino kou, ʻoi aku ka maikaʻi o ke kamaʻilio ʻana me ke kauka akamai e pili ana i ia mea.
ʻO ka maʻi myasthenic Lambert -Eaton, LEMS, nā maʻi autoimmune, nāwaliwali o nā ʻiʻo, ka maʻi ʻaʻai māmā liʻiliʻi, ka neuromuscular junction, acetylcholine











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