Ua ʻike anei ʻoe i nā kiko ʻulaʻula, ʻōpala, a i ʻole nā kiko ʻē ma ka ʻili o kāu pēpē ma hope koke o ka hānau ʻana, a i ʻole kekahi mau pule ma hope? I kekahi manawa, ma ke ʻano he makuahine, hiki ke hopohopo ka ʻike ʻana i kēia mau mea. I kēia lā, e kamaʻilio mākou e pili ana i kahi kūlana e hoʻoulu ai i nā hōʻailona like, he mea kakaikahi iki ia, akā he mea nui e ʻike. Ua kapa ʻia kēia ʻo Incontinentia Pigmenti, a i ʻole IP no ka pōkole.
He aha ka Incontinentia Pigmenti (IP)?
I ka ʻōlelo maʻalahi, he maʻi hoʻoilina kēia. Ma muli o kona ʻano X-linked dominant, ʻoi aku ka hopena o ia i nā kaikamahine ma mua o nā keikikāne.
No loko o kahi hui o nā maʻi i kapa ʻia ʻo "nā maʻi neurocutaneous". E noʻonoʻo, ʻo ia hoʻi, hiki i kēia maʻi ke hoʻopilikia i kā mākou ʻōnaehana nerve (ʻo ia hoʻi, ka lolo a me nā aʻalolo) a me ka ʻili i ka manawa like. ʻAʻole wale ia, akā i kekahi manawa hiki iā ia ke hoʻopilikia i nā maka, nā niho, a me ka ʻōnaehana iwi.
ʻIke nui ʻia kēia maʻi i ka wā hānau a i ʻole i nā pule mua ma hope o ka hānau ʻana, me nā kiko like ʻole a me nā pehu e kū mai ana ma ka ʻili. Hoʻokumu ʻia kēia e kahi mutation i loko o ka gene i kapa ʻia ʻo NEMO (NF-kappaB essential modulator), ka mea e kāohi ana i ka hana a me ka ulu ʻana o kā mākou mau cell. A hiki i kēia manawa, ʻaʻohe lāʻau lapaʻau piha no kēia, akā aia nā lāʻau lapaʻau maikaʻi e kāohi i nā hōʻailona.
He aha nā loli i ka ʻili? He 4 mau pae!
Hiki ke ʻike ʻia nā loli ʻili i ka IP i ʻehā mau pae nui. Akā e hoʻomanaʻo, ʻaʻole pono e hana ʻia kēia mau pae ma ke kaʻina. I kekahi manawa hiki iā lākou ke hoʻololi, a hiki ke ʻike ʻia ʻelua a ʻekolu paha mau pae i ka manawa like.
| Kahua | ʻAno a me ka wehewehe |
|---|---|
| Pae 1: Vesicular | Hoʻohālikelike ʻia kēia e ke ʻano o nā pehu ʻulaʻula piha i ka wai ma ke kino. ʻIke pinepine ʻia kēia mau mea ma nā lima a me nā wāwae. |
| Pae 2: Verrucal | ʻIke ʻia nā ʻeha hou e like me ka wart ma kahi o nā pehu. |
| Pae 3: Hyperpigmentation | ʻO kēia ka hiʻohiʻona ʻokoʻa loa o kēia maʻi. ʻIke ʻia ka ʻili ma ke ʻano he hina, uliuli, a ʻeleʻele paha, he kinikini a he ʻale paha. ʻIke pinepine ʻia kēia mau mea ma ke kino a me nā lālā. |
| Pae 4: Hypopigmentation | I ka hala ʻana o ka manawa, mae wale kēlā mau kiko ʻeleʻele, e waiho ana i nā ʻeha keʻokeʻo, e like me ka ʻōpala i ʻoi aku ka keʻokeʻo ma mua o ke kala o ka ʻili. |
He mau kiko ʻili wale nō paha? ʻAʻole, aia paha he mau hopena ʻē aʻe
ʻAʻole i kaupalena ʻia kēia maʻi i ka ʻili. Hiki i kekahi mau keiki ke loaʻa i nā pilikia ʻē aʻe. Eia nō naʻe, pono ʻoe e hoʻomaopopo ʻaʻole e ʻike ʻia kēia mau hōʻailona āpau i kēlā me kēia keiki.
Nā pilikia Neurological
Ma kahi o 20% o nā keiki me IP e loaʻa i nā pilikia neurological.
- Ka lohi o ka ulu ʻana o ke kino: No ka laʻana, ʻaʻole hiki iā lākou ke hoʻohuli i ko lākou ʻāʻī, ʻōwili i luna, a hele paha i ka wikiwiki like me nā pēpē ʻē aʻe.
- Ke kīnā noʻonoʻo: Nā mea e like me ke kīnā aʻo ʻana.
- Nāwaliwali o nā ʻiʻo: Manaʻo o ka nāwaliwali.
- Nā hopu ʻana: ʻO ka hiki ʻana mai o nā kūlana like me ke kūpono.
Hiki ke hana ʻia kēia e nā mea e like me ka emi ʻana o ka ʻiʻo o ka lolo (cerebral atrophy) a me ke kūkulu ʻia ʻana o nā lua liʻiliʻi i loko o ka mea keʻokeʻo o ka lolo.
Nā hopena ma nā maka a me nā niho
- Nā pilikia maka: Loaʻa paha i kekahi mau keiki nā kūlana e like me ka strabismus, cataracts, a i ʻole ka nalowale nui o ka ʻike.
- Nā pilikia niho: Hiki ke ʻike ʻia nā niho nalowale a me nā niho ʻano kui.
ʻO ka mea nui loa, ʻaʻole e hopohopo koke ke ʻike ʻoe i kahi hōʻailona e like me kēia, akā e ʻike i kahi pediatrician no ka ʻōlelo aʻo.
Pehea e mālama ʻia ai kēia?
Hoʻōla ʻia ʻo IP ma muli o nā hōʻailona o ke keiki. Hiki ke ʻokoʻa nā koho lapaʻau kūpono no kēlā me kēia keiki.
| Pilikia (Hōʻailona) | ʻAno Hana Lapaʻau |
|---|---|
| Nā ʻōuli ʻili a me nā pehu | ʻO ka nūhou maikaʻi, ʻaʻole pono kēia mau kiko ʻili i kekahi mālama kūikawā. E mae wale lākou i ka wā e ulu ai ke keiki a i ka wā makua. |
| Nā pilikia o ka ʻōnaehana nerve (nā hopu ʻana, nāwaliwali o nā mākala) | No nā mea e like me kēia, pono ʻoe e ʻike i kahi neurologist . A laila, hiki ke hoʻomalu ʻia kēia mau kūlana me ka lāʻau lapaʻau a i ʻole, inā pono, nā mea lapaʻau ʻē aʻe. |
| Ka hemahema o ka ʻike | No nā pilikia ʻike, hiki iā ʻoe ke ʻike i kahi kauka maka a loaʻa nā aniani, nā lāʻau lapaʻau, a i ʻole, i nā hihia koʻikoʻi, ke ʻoki ʻana. |
| Nā pilikia niho | Inā he mau pilikia me ke ʻano a i ʻole ka helu o nā niho, hiki iā ʻoe ke ʻimi i ka lāʻau lapaʻau e pono ai mai ke kauka niho. |
No laila, pono anei kākou e makaʻu i ka wā e hiki mai ana?
He nīnau kēia a nā mākua he nui. ʻO kaʻoiaʻiʻo, i ka hapanui o nā hihia, inā ʻaʻohe pilikia koʻikoʻi i ka wā kamaliʻi, hiki i nā keiki me IP ke ola i kahi ola maʻamau a alakaʻi i nā ola olakino a maʻamau . I ka hala ʻana o ka manawa, e mae paha kēlā mau wahi ʻeleʻele ma ka ʻili, a nalowale loa paha.
Eia nō naʻe, e like me ka mea i ʻōlelo ʻia ma mua, aia nā keiki i loaʻa i nā pilikia neurological a i ʻole nā pilikia kino ʻē aʻe. No laila, ʻo ka mea maikaʻi loa e hana ai, ʻo ia ke kamaʻilio me kāu kauka e pili ana i ke kūlana o kāu keiki. A laila hiki iā ʻoe ke loaʻa kahi ʻike maopopo i ka hoʻolālā olakino maikaʻi loa no kāu keiki a me ka mea e hana ai ma hope.
Leka Lawe-Home
- ʻO ka Incontinentia Pigmenti (IP) kahi maʻi genetic kakaikahi e pili nui ana i nā kaikamahine.
- ʻO ka hōʻailona nui ka nui o nā loli o ka ʻili (nā pehu, nā ʻāpana ʻāʻī, nā ʻāpana wrinkled ʻeleʻele, a me nā ʻāpana keʻokeʻo).
- Ma waho aʻe o nā pilikia ʻili, hiki i kekahi mau keiki ke loaʻa nā pilikia e pili ana i ka ʻōnaehana nerve, nā maka, a me nā niho.
- E mae pinepine nā kiko ʻili i ka hala ʻana o ka manawa me ka ʻole o ka mālama ʻia.
- Hiki i ka hapa nui o nā keiki ke ola maʻamau a olakino me ka ʻole o nā pilikia koʻikoʻi.
- Inā loaʻa i kāu keiki kēia mau hōʻailona, he mea nui loa ia e hopohopo ʻole a e ʻike i kahi pediatrician no ka ʻōlelo aʻo kūpono.
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