E noʻonoʻo e nānā ana i ke aniani a ʻike i kahi laina pololei i waenakonu o kou helehelena, ʻulaʻula loa kekahi ʻaoʻao a pulu, a ʻo kekahi ʻaoʻao maʻamau, malia paha he keʻokeʻo iki ... ʻAʻole anei he mea weliweli a kupanaha? A i ʻole pehea inā ʻike ʻoe i kekahi ʻaoʻao o ke kino o kāu pēpē hānau hou i ʻulaʻula koke a ʻo kekahi ʻaoʻao he maʻamau? ʻO kēia ka mea a mākou e kapa nei ʻo ka Harlequin Sign in medicine. No ka mea he ʻano ʻē iki ka inoa, e ʻike hou kākou e pili ana i ia mea a inā he mea maoli kēia e makaʻu ai.
He aha maoli kēia Hōʻailona Harlequin?
I ka ʻōlelo maʻalahi, ʻo ka Hōʻailona Harlequin ka wā e loli ai ke kala o ka ʻili i kekahi ʻaoʻao o kou kino a i ʻole kou helehelena (ʻulaʻula a ʻeleʻele paha), ʻoiai ʻo kekahi ʻaoʻao e mau ana ia, i kekahi manawa keʻokeʻo. Me he mea lā ua hoʻokaʻawale ʻia kēia mau ʻaoʻao ʻelua e kahi laina pololei, ma waenakonu o ke kino.
Me kēia ʻokoʻa o ke kala o ka ʻili, hiki ke ʻike ʻia nā hiʻohiʻona ʻē aʻe ma nā ʻaoʻao ʻelua.
- ʻUlaʻula: He hyperhidrosis paha kēia wahi (ka hou nui ʻana). Hiki ke ʻike ʻia ka ʻili ma ia wahi he mehana iki ke pā aku.
- ʻAoʻao Palai/Maʻamau: ʻAʻole hou kēia ʻaoʻao. E anuanu nō hoʻi ka ʻili ke pā aku.
ʻOiai hiki i kēia Hōʻailona Harlequin ke lilo i hōʻailona o kahi maʻi e like me ka 'Harlequin syndrome', i ka hapa nui o ka manawa he maikaʻi ʻole ia a nalo wale aku.
He aha nā kumu nui no kēia?
ʻAʻole hiki ke loaʻa i nā manawa a pau kahi kumu kikoʻī no kēia. ʻO ke kumu nui o kēia he pilikia kamaʻilio i loko o ka ʻōnaehana nerve autonomic o ko mākou kino. E noʻonoʻo i kēia me ka ʻōnaehana "auto-pilot" o ko mākou kino. Hoʻomalu kēia ʻōnaehana i nā mea a mākou i ʻike ʻole ai iā mākou, e like me ko mākou hanu ʻana, ka puʻuwai, a me ka hou ʻana.
No laila, inā he ālai a kīnā paha i ke ala o ke aʻalolo e hoʻouna ana i nā leka mai ka lolo (hypothalamus) ma o ke kaula spinal i ka maka a me ke kino o luna, ʻaʻole paha e hoʻouna pono ʻia ka leka i hoʻokahi ʻaoʻao o ke kino. ʻO ia ke kumu e hoʻonui ai nā kīʻaha koko ma kekahi ʻaoʻao a lilo i ʻulaʻula, ʻoiai ʻaʻole loaʻa i kekahi ʻaoʻao ka leka a noho maʻamau.
Ke hana nei kēia i nā pēpē hānau hou?
ʻAe, he mea kēia i ʻike ʻia i nā pēpē hānau hou. Wahi a nā noiʻi, ma kahi o 10% o nā pēpē olakino e loaʻa paha kēia Hōʻailona Harlequin. No laila, mai hopohopo inā loaʻa i kāu pēpē kēia.
ʻO ka maʻamau, hana ʻia kēia ma waena o ʻelua a ʻelima mau lā ma hope o ka hānau ʻana o ke pēpē, akā i kekahi manawa hiki ke ʻike ʻia a hiki i ʻekolu pule.
ʻO ka mea nui loa, ʻo ia kēia Hōʻailona Harlequin i loaʻa i nā pēpē hānau hou.ʻAʻohe mea ʻino loa. Nalo koke kēia hoʻololi kala i loko o 30 kekona wale nō. I kekahi manawa hiki ke mau a hiki i 20 mau minuke. Hiki ke ʻike hou ʻia ke waiho ʻia ke pēpē ma kona ʻaoʻao.
He mea nui loa: ʻAʻole pono e huikau ʻia kēia kūlana i kapa ʻia ʻo Harlequin Sign me ka 'Harlequin ichthyosis', kahi kūlana hoʻoilina weliweli loa, hoʻoweliweli i ke ola a kakaikahi loa. ʻO 'Harlequin ichthyosis' kahi kūlana i uhi ʻia ai ke kino holoʻokoʻa o ke pēpē i nā unahi mānoanoa e like me ka papa, me nā māwae hohonu ma waena. He mea ʻokoʻa loa ia.
Nā maʻi pili ʻē aʻe
Hiki i ka hōʻailona Harlequin ke ʻike ʻia i kekahi manawa ma ke ʻano he hōʻailona o nā pilikia olakino ʻē aʻe.
ʻO ka maʻi Harlequin
He kūlana kēia e hoʻopilikia ai i kā mākou ʻōnaehana nerve sympathetic. Hoʻomalu kēia ʻōnaehana nerve i kā mākou pane "hakakā a lele paha", ʻo ia hoʻi nā loli e kū mai ana i ke kino ke pīhoihoi mākou, makaʻu, a hoʻoikaika kino paha. I kēlā me kēia manawa e hoʻāla ʻia ai kēia ʻōnaehana i loko o ke kanaka me ka Harlequin Syndrome (no ka laʻana, i ka wā o ka hoʻoikaika kino), e lilo kekahi ʻaoʻao o ka maka a me ke kino i ʻulaʻula a hou, ʻoiai ʻaʻole ka ʻaoʻao ʻē aʻe.
ʻO ka maʻi Horner
He maʻi neurological laha ʻole kēia. Hoʻopilikia ia i ka maka a me nā ʻiʻo a puni ma kekahi ʻaoʻao o ka maka. Ua kapa ʻia hoʻi ʻo 'oculosympathetic palsy'. Hoʻokumu ʻia ia e kahi ālai ʻana i ke ala o ke aʻalolo mai ka lolo a i ka maka a me ka maka. Maanei pū kekahi, hiki i ka ʻaoʻao i hoʻopilikia ʻia o ka maka ke lilo i ʻulaʻula.
| Kūlana | ʻO wai ka mea i hoʻopilikia ʻia? | Nā hiʻohiʻona nui | ʻOiaʻiʻo? |
|---|---|---|---|
| Hōʻailona Harlequin (i nā pēpē hānau hou) | Nā pēpē hānau hou olakino (ma kahi o 10%) | ʻUlaʻula ma kekahi ʻaoʻao o ke kino no ka manawa pōkole. | ʻAʻole. ʻAʻohe mea ʻino loa a e nalo wale aku nō ia. |
| ʻO ka maʻi Harlequin | No kekahi o kēlā me kēia makahiki | ʻUlaʻula a me ka hou ʻana ma kekahi ʻaoʻao i ka wā e hoʻoikaika kino ai a i ʻole e pīhoihoi ana. | Aia nō ia ma ke kumu kumu. ʻO ka hapa pinepine, ʻaʻole ia he mea ʻino. |
| ʻO ka maʻi Horner | No kekahi o kēlā me kēia makahiki | ʻO ka lihilihi o lalo ma kekahi ʻaoʻao o ka maka, ka maka ʻeleʻele liʻiliʻi, ka emi ʻana o ka hou ʻana, a me ka ʻulaʻula o ka maka. | Hiki ke koʻikoʻi ke kumu nui (e.g., he puʻupuʻu). He mea nui ka hoʻokolokolo lapaʻau. |
Pehea e mālama ʻia ai?
ʻO ke ʻano o ka lāʻau lapaʻau e pili ana i ke kumu o ka Harlequin Sign.
- No nā pēpē hānau hou: ʻAʻohe pono o ka lāʻau lapaʻau. E nalo wale ia i loko o kekahi mau kekona a i ʻole mau minuke.
- No ka Harlequin Syndrome: ʻAʻole pono mau ka lāʻau lapaʻau. I kekahi manawa hiki i kēia kūlana ke hoʻomaikaʻi iā ia iho. Eia nō naʻe, inā manaʻo kāu kauka he pono ka lāʻau lapaʻau, e kuhikuhi lākou i nā lāʻau lapaʻau e pili ana i ke kumu. ʻO kēia mau mea:
- Hoʻohana ʻia kahi lāʻau pale i kapa ʻia ʻo 'Stellate ganglion block' e kāohi i ka hoʻololi ʻili.
- Nā inikini toxin botulinum e kāohi i ka hou ʻana.
- Inā he puʻupuʻu ma lalo, hiki ke wehe ʻia ma ke ʻoki ʻana a i ʻole ma o ka radiation therapy.
- Ke aʻo ʻana no ka hopohopo noʻonoʻo i hoʻokumu ʻia e kēia ʻano.
- No ka Horner Syndrome: Inā ʻaʻohe ʻeha, ʻaʻole pono paha ka lāʻau lapaʻau. I kēia hihia, e mālama ke kauka i ke kumu kumu.
Āhea ʻoe e ʻike ai i ke kauka?
Inā ʻike ʻoe a i ʻole kekahi o kou ʻohana i ka Hōʻailona Harlequin, he ʻulaʻula, hou, a wela hoʻi ma kekahi ʻaoʻao o ke kino, ʻo ka mea maikaʻi loa e hana ai, ʻo ia ke ʻike i ke kauka a hoʻāʻo e nānā iā ia iho.
E nānā kāu kauka iā ʻoe, e nīnau e pili ana i kāu mau hōʻailona, a inā pono, e hana i kekahi mau hoʻokolohua e kāpae i nā kūlana ʻē aʻe. ʻOiai he mea kakaikahi loa, he mea nui e kāpae i kahi puʻupuʻu a i ʻole kekahi kūlana ʻē aʻe, no ka mea, ʻo ia paha ke kumu.
Inā hele pū koke kēia mau hōʻailona me nā hōʻailona koʻikoʻi ʻē aʻe (e like me ke poʻo ʻeha nui, nā loli o ka ʻike, ka nalowale o ka ʻike), he mea nui loa ia e hele koke i ka Emergency Department (ETU) o ka haukapila kokoke loa .
Leka Lawe-Home
- ʻO ka Hōʻailona Harlequin kahi kūlana kahi i ʻulaʻula ai kekahi ʻaoʻao o ke kino a i ʻole ka maka a he keʻokeʻo kekahi ʻaoʻao. ʻAʻole maʻamau kēia he mea ʻino.
- He mea maʻamau kēia i nā pēpē hānau hou. Nalo wale ia, i loko o kekahi mau minuke, me ka ʻole o ka lāʻau lapaʻau, a ʻaʻohe mea e hopohopo ai. E hoʻomanaʻo ʻaʻole kēia ka maʻi weliweli ʻo 'Harlequin ichthyosis'.
- Inā loaʻa kēia i nā pākeke, he hōʻailona paha ia o kahi maʻi ʻōnaehana nerve e like me ka 'Harlequin syndrome' a i ʻole 'Horner syndrome'.
- Inā ʻike ʻoe a i ʻole kāu pēpē i kēia hōʻailona, he mea naʻauao ke ʻole e hopohopo, akā e ʻike i kāu kauka e hoʻoholo i ke kumu pololei a e hōʻoia ʻaʻohe mau maʻi koʻikoʻi ʻē aʻe.
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