Ke hopohopo nei ʻoe e pili ana i nā puʻupuʻu a i ʻole nā ​​puʻupuʻu ma kou kino? He neurofibromas paha kēia. E kamaʻilio kākou e pili ana iā lākou!

Ua ʻike anei ʻoe i nā puʻupuʻu liʻiliʻi a i ʻole nā ​​puʻupuʻu ma kou kino, ma lalo a ma luna paha o ka ʻili? A i ʻole ua ʻike anei ʻoe i kekahi āu i ʻike ai he puʻupuʻu ko lākou? He mea maʻamau ka makaʻu iki a me ka hopohopo ke ʻike ʻoe i kēia mau mea. Akā, ʻaʻole nā ​​puʻupuʻu a pau he mea weliweli. I kēia lā, e kamaʻilio mākou e pili ana i kekahi ʻano puʻupuʻu, ʻo ia hoʻi ke ʻano i kapa ʻia ʻo neurofibroma. Mai hopohopo, e kamaʻilio mākou e pili ana i kēia me nā kikoʻī a me ka maʻalahi.

He aha ka Neurofibroma?

I ka ʻōlelo maʻalahi, he puʻupuʻu maikaʻi ʻole ka neurofibroma e ulu ana ma kou mau hunaola aʻalolo. ʻO ia kekahi o nā hui o nā maʻi genetic kakaikahi i kapa ʻia ʻo neurofibromatosis. Hiki i kekahi poʻe i hānau ʻia me ka neurofibromatosis ke hoʻomohala i kēia mau puʻupuʻu ma ka ʻili, ma lalo o ka ʻili, a i loko paha o ke kino.

Akā eia ka mea, ʻo ka hapa nui o nā neurofibromas ʻaʻole ia e hoʻoulu i nā pilikia olakino nui . Eia nō naʻe, i kekahi manawa, inā nui iki lākou, hiki iā lākou ke hoʻopilikia i nā aʻalolo he nui a hoʻoulu i nā pilikia olakino koʻikoʻi. I kēlā hihia, e wehe nā kauka iā lākou me ke ʻoki ʻana.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia kūlana?

ʻO kaʻoiaʻiʻo, ua hānau ʻia nā kānaka me kēia mau neurofibromas. Akā ʻo ka mea kupaianaha, i ka wā e hoʻomaka ai kēia mau puʻupuʻu e ʻike ʻia, hiki ke hala nā makahiki. ʻO ka hapa nui o ka manawa, ʻike maopopo ʻia lākou i ka wā ʻōpio.

Wahi a nā helu helu, ma kahi o hoʻokahi i loko o 3,000 mau keiki e loaʻa paha kahi maʻi i kapa ʻia ʻo Neurofibromatosis Type 1 (NF1) . Hoʻomaopopo pinepine ʻia ma mua o ka piha ʻana o 10 mau makahiki. Ma kahi o 25% o nā keiki me NF1, a i ʻole ma kahi o hoʻokahi i loko o ʻehā, e loaʻa paha nā puʻupuʻu i hiki ke lilo i mea nui e hoʻoulu ai i nā pilikia olakino koʻikoʻi.

Pehea ka hopena o ka Neurofibroma i ko mākou kino?

ʻOkoʻa loa ke ʻano o ka hoʻopilikia ʻana o nā neurofibromas i kēlā me kēia kanaka. Aia ia i ke ʻano o ka puʻupuʻu, kona nui, a me kona wahi ma ke kino.

• No kekahi poʻe, ʻike ʻia kēia mau mea he mau puʻupuʻu liʻiliʻi a i ʻole nā ​​​​​​ʻāpana o ka ʻili mānoanoa.
• Eia nō naʻe, no kekahi poʻe me nā neurofibromas nui, hiki iā lākou ke hoʻopilikia i ko lākou mau kino kūloko a me ke kaula spinal.

E noʻonoʻo e pili ana i ia mea, i kekahi manawa ua like ia me kahi pōpō liʻiliʻi e hana ana i loko o ke kino. Aia ka hopena i kona wahi.

Hiki paha i kēia mau puʻupuʻu ke lilo i kanesa?

He pilikia kēia i loaʻa i nā poʻe he nui. ʻAʻole lilo ka hapa nui o nā neurofibromas i mea ʻino. He maikaʻi ʻole lākou.

Akā,Aia kekahi ʻano kūikawā i kapa ʻia ʻo Plexiform neurofibromas. Ma kahi o 10% o kēia ʻano, a i ʻole hoʻokahi i loko o ka ʻumi, hiki ke lilo i maʻi kanesa. No laila, he mea nui loa ke nānā ʻia ʻoe e ke kauka inā he puʻupuʻu kou e like me kēia.

He aha nā hōʻailona o Neurofibroma?

ʻOkoʻa nā hōʻailona o ka neurofibroma. E like me ka mea i ʻōlelo ʻia ma mua, hilinaʻi lākou i ke ʻano, ka nui, a me kahi o ka puʻupuʻu. ʻO ka mea kupanaha, ʻaʻole paha e hōʻike kekahi poʻe me ka neurofibroma i kekahi mau hōʻailona. Eia nō naʻe, i kekahi manawa hiki iā lākou ke hana i nā kūlana e like me ka maʻi lolo a i ʻole ka makapō.

E nānā kākou i kēia mau ʻano neurofibromas a me kā lākou mau hōʻailona pili:

Nā neurofibromas kūloko

Ua kapa ʻia hoʻi kēia he neurofibromas ʻili.

• Hiki i kēia ke ʻike ʻia he mau puʻupuʻu liʻiliʻi ma ke kino holoʻokoʻa .
• ʻIke pinepine ʻia kēia mau mea i nā kānaka ma waena o 20 a me 40 mau makahiki.
• Hiki i kēia mau puʻupuʻu ke maneʻo a hiki ke ʻeha ke kaomi ʻia .

Nā neurofibromas hoʻolaha

ʻO kēia kekahi no ke ʻano like o ka neurofibroma ʻili.

• ʻIke pinepine ʻia kēia ma ke poʻo a me ka ʻāʻī .
• ʻIke ʻia ia he wahi mānoanoa a hoʻokiʻekiʻe ʻia ma ka ʻili o ka ʻili.
• Hiki paha iā ʻoe ke manaʻo i kahi ʻano ʻūlū a i ʻole ka mae ʻana ke hoʻopā ʻoe.

Nā neurofibromas Plexiform

Hoʻomohala kēia ʻano neurofibroma ma nā hui o nā aʻalolo.

• ʻO kēia ka mea maʻamau i nā keiki me ka Neurofibromatosis Type 1.
• Hiki i kēia mau ulu ke ulu nui aʻe i ka hala ʻana o ka manawa a i kekahi manawa ʻike ʻia he mau puʻu nui loa ma luna a ma lalo paha o ka ʻili o nā keiki.
• Hiki i kēia ke kau i ke kaomi ma ke kaula kuamoʻo a i ʻole nā ​​​​aʻalolo peripheral, e hana ana i nā hōʻailona e like me ka maʻi lolo , nāwaliwali, a me ka numbness .
• I kekahi mau keiki me nā neurofibromas Plexiform, hiki i ke kaomi o kēia mau puʻupuʻu ma ka iwi kuamoʻo ke hana i kahi maʻi i kapa ʻia ʻo scoliosis . He mea nui e makaʻala i kēia, no ka mea, ma ke ʻano he mau mākua, pono mākou e akahele loa i ka wā e hoʻopilikia ai kekahi mea e like me kēia i kahi keiki.

Pehea ke ʻano o kēia mau puʻupuʻu? (ʻAno)

ʻO nā puʻupuʻu neurofibroma, i hana ʻia ma luna a ma lalo paha o ka ʻili, he ʻokoʻa loa ke ʻano o nā neurofibromas plexiform, i hana ʻia i loko o ke kino.

• Nā neurofibromas kūloko / Nā neurofibromas ʻili:He mau puʻupuʻu ʻili kēia (nodules) . Loaʻa pinepine lākou ma ke kino, ke poʻo, ka ʻāʻī, nā lima, a me nā wāwae. Ua like ko lākou nui me ka pī . He palupalu lākou i ka pā ʻana a he ʻano palupalu iki ko lākou . Inā ʻoe e kaomi i kahi puʻupuʻu e like me kēia, e piholo ia i loko o ka ʻili, a i kou wehe ʻana i kou lima, e puka hou mai ia. Ua kapa ʻia kēia e nā kauka ʻo "buttonhole sign." Ua like ia me kahi lua i loko o ka pālule kahi i kau ʻia ai kahi pihi.
• Nā neurofibromas diffuse: Hiki ke ʻike ʻia kēia ma ke ʻano he ʻulaʻula, i hoʻokiʻekiʻe ʻia ma ka ʻili .
• Nā neurofibromas Plexiform: Hiki i kēia ke like me nā puʻu ʻiʻo nui i hoʻokuke ʻia i waho o ke kino. Ua wehewehe nā kauka iā lākou he "ʻeke ilo ma lalo o ka ʻili." Inā ʻike ʻoe i kahi mea e like me kēia, ʻoi aku ka maikaʻi o ka ʻimi koke ʻana i ke kōkua lapaʻau.

He aha ka Neurofibromatosis ʻAno 1 (NF1)?

ʻO ka Neurofibromatosis Type 1 (NF1) kahi maʻi e hoʻopilikia ana ma kahi o 1 i loko o 3,300 mau pēpē hānau hou. ʻO Neurofibroma kekahi hōʻailona wale nō o kēia maʻi (NF1). Aia kekahi mau hōʻailona ʻē aʻe:

• Nā wahi Café-au-lait: He mau wahi ʻeleʻele māmā kēia, he kala kofe, a he nui. Hiki i kēia mau wahi ke nui aʻe i ka hala ʻana o ka manawa.
• Nā ulu ʻino ʻole ma ka ʻaoʻao kala o ka maka (iris): Ua kapa ʻia kēia he Lisch nodules.
• Nā puʻupuʻu o ke aʻalolo optic: Ua kapa ʻia kēia he optic pathway gliomas.

Inā kānalua ʻoe ua loaʻa i kāu keiki kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​​​​​he mea nui e nīnau i kahi pediatrician no ka ʻōlelo aʻo.

He aha nā kumu o ka neurofibroma?

ʻO ka Neurofibroma kahi hōʻailona o ke ʻano neurofibromatosis type 1 (NF1). Hoʻokumu ʻia kēia ʻano e kahi mutation a i ʻole ka loli ʻana o kahi gene i kapa ʻia ʻo ka gene NF1. Loaʻa i ka gene NF1 nā ʻōlelo kuhikuhi no ka hana ʻana i kahi protein i kapa ʻia ʻo neurofibromin.

E nānā, he mea nui loa kēia protein i kapa ʻia ʻo Neurofibromin. He protein hoʻopau tumor ia. ʻO ia hoʻi, ʻo ia ka mea e pale maʻamau ai i nā cell mai ka ulu wikiwiki ʻana a me ka māhele ʻana me ke kāohi ʻole ʻia. Pehea e hana ai i kēlā? Aia kekahi protein ʻē aʻe i kapa ʻia ʻo Ras protein, nāna e kōkua i nā cell e ulu a māhele. Hoʻokele ʻia kēlā protein Ras e Neurofibromin.

No laila, ke hoʻololi ʻia kēlā gene `(NF1)`, e pau ia i ka hoʻōki ʻana i ka ulu ʻana o nā cell. A laila hoʻonui nui nā cell me ke kāohi ʻole ʻia a hana i nā tumors. Maopopo iā ʻoe?

Hiki ke hoʻoili ʻia kēia maʻi `(NF1)` i nā keiki inā loaʻa i kekahi o nā mākua kēia hoʻololi ʻana o ka genetic. Ua hoʻoilina paha kēlā mau mākua mai ko lākou mau mākua mai. Eia naʻe, he mea kupanaha, ʻo 50% o ka poʻe me `(NF1)`, ʻo ia hoʻi, ma kahi o ka hapalua, ʻaʻohe mōʻaukala ʻohana o ka maʻi. ʻO ke ʻano kēia, hiki ke loaʻa kēia maʻi ʻoiai inā loaʻa kahi hoʻololi genetic hou.

Pehea e ʻike ai nā kauka i kēia?

ʻO ka maʻamau, hana mua nā kauka i kahi hoʻokolohua kino e ʻike ai i ka neurofibroma. ʻO ia hoʻi ke nānā aku iā ʻoe a nānā i nā puʻupuʻu.

Eia kekahi, hiki ke hoʻohana ʻia nā hoʻokolohua kiʻi e like me kēia:

• ʻO ke scan CT (Computed Tomography scan) a me ke scan MRI (MRI - Magnetic Resonance Imaging): Hoʻohana ʻia kēia mau hoʻokolohua e ʻike i nā puʻupuʻu liʻiliʻi loa. Kōkua pū lākou i ka ʻike ʻana i kahi o kahi puʻupuʻu a inā paha e hoʻopilikia ke ʻoki ʻana i nā ʻiʻo a i ʻole nā ​​​​​​kino e puni ana.
• PET scan (Positron Emission Tomography scan): Kōkua kēia hoʻāʻo i nā kauka e hoʻoholo inā he maikaʻi ʻole a ʻino paha kahi puʻupuʻu.

He aha nā lāʻau lapaʻau no ka neurofibroma?

He nui nā ala a nā kauka e mālama ai i ka neurofibroma:

• Nānā ʻana: Inā maikaʻi ʻole kāu neurofibroma a ʻaʻole ia e hoʻopilikia nui, e haʻi aku kāu kauka iā ʻoe e hele mai no nā nānā maʻamau e ʻike inā he mau loli.
• ʻO ke ʻoki plastik: Hiki i kāu kauka ke ʻōlelo aku i ke ʻoki plastik e wehe i nā ulu ʻino ʻole ma luna a ma lalo paha o ka ʻili.
• ʻO ke ʻoki kino: Inā loaʻa iā ʻoe kahi neurofibroma e kaomi ana i kou mau iwi a i ʻole nā ​​​​​​ʻōpū, hiki i kāu kauka ke ʻōlelo i ke ʻoki kino e wehe i ka nui o ka puʻupuʻu, a i ʻole ka nui o ka puʻupuʻu e like me ka hiki, me ka ʻole o ka hōʻino ʻana i kou mau ʻōpū a i ʻole nā ​​​​​​ʻiʻo.

Aia kekahi mau hopena ʻaoʻao o ke ʻoki kino?

ʻOkoʻa nā hopena ʻaoʻao o ke ʻoki ʻana i ka neurofibroma mai kēlā me kēia ʻoki. No ka laʻana, ʻokoʻa nā hopena ʻaoʻao o ke ʻoki ʻana e wehe i nā puʻupuʻu ʻili mai nā hopena o ke ʻoki ʻana e wehe i nā puʻupuʻu kuamoʻo. Inā ʻoe e hoʻolālā ana e hana i ke ʻoki neurofibroma, he mea nui e nīnau i kāu kauka e pili ana i nā hopena ʻaoʻao o ke ʻoki ʻana.

Aia kekahi ala e pale aku ai i kēia mau mea mai ka hana ʻana?

He maʻi hoʻoilina ka Neurofibroma, no laila he paʻakikī ke pale aku i ka ulu ʻana o kēia mau ʻōpū .

He aha ka mea e hana ai inā loaʻa iaʻu kahi neurofibroma?

Ma ke ʻano laulā, ʻaʻole i hoʻopilikia ʻia ka hapa nui o ka poʻe me nā neurofibromas e ke ʻano . Eia nō naʻe, hopohopo paha ka poʻe me nā puʻupuʻu he nui a nui paha e pili ana i ko lākou helehelena a manaʻo lākou e hoʻopilikia ana ia i ko lākou ola. Inā loaʻa iā ʻoe kēia ʻano, e kamaʻilio me kāu kauka e pili ana i ke ʻoki ʻana e wehe i nā puʻupuʻu i ʻike ʻia e hoʻoluhi a hilahila iā ʻoe iho. He mea kākaʻikahi loa ka hoʻi ʻana mai o nā Neurofibromas ma hope o ke ʻoki ʻana.

Pehea wau e mālama ai iaʻu iho inā loaʻa iaʻu ka neurofibroma?

He mau puʻupuʻu maikaʻi ʻole nā ​​neurofibromas, a ʻaʻole pinepine lākou e hoʻoulu i nā pilikia olakino koʻikoʻi . Eia nō naʻe, ʻo ka poʻe me nā neurofibromas he nui, a i ʻole ka poʻe me nā puʻupuʻu nui, hiki ke hopohopo iā lākou iho i ko lākou helehelena. I ia mau hihia, he koho maikaʻi paha ke ʻoki ʻana.

ʻAʻole paha e like ka weliweli a me ka weliweli o ka ʻike ʻia ʻana me kahi puʻupuʻu maʻi ʻaʻai me ka ʻike ʻia ʻana me kahi puʻupuʻu maʻi ʻaʻai. Eia nō naʻe, hiki i kahi puʻupuʻu maikaʻi ʻole e like me ka neurofibroma ke hoʻopilikia i kou ola.

Hiki i kēia ke hoʻopilikia i kou helehelena. Hoʻomohala kekahi poʻe i nā neurofibromas, hiki ke ulu nui a hiki ke hoʻopilikia i nā ʻōpū a me nā ʻiʻo kokoke. Inā ua ʻike ʻia ʻoe me nā neurofibromas, e nīnau i kāu kauka e pili ana i ke ʻoki kino e wehe i kāu neurofibromas.

Eia kekahi, e hoʻomanaʻo he hiki i ka neurofibroma ke lilo i hōʻailona o kahi maʻi laha ʻole i kapa ʻia ʻo neurofibromatosis, hiki ke hoʻoulu i nā pilikia olakino koʻikoʻi. No laila, he mea nui loa e nīnau i kāu kauka inā he hōʻailona kou puʻupuʻu o ka neurofibromatosis.

Leka Lawe-Home

ʻAe, eia kekahi o nā mea nui e pono ai ʻoe e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai:

• ʻO ka Neurofibroma kahi ʻano puʻupuʻu e ulu ana mai nā pūnaewele nerve a ʻaʻole ia he mea ʻino .
• He hōʻailona paha kēia o kahi maʻi genetic i kapa ʻia ʻo neurofibromatosis.
• ʻOiai ʻo ka hapa nui o ka manawa ʻaʻole kēia mau mea e hoʻoulu i nā pilikia olakino nui, hiki i kekahi mau ʻano nui a plexiform paha ke hoʻoulu i nā pilikia .
• Loaʻa i kahi hapa liʻiliʻi o nā ʻano Plexiform ka pilikia o ka lilo ʻana i maʻi kanesa .
• ʻOkoʻa nā hōʻailona, ​​​​mai nā ʻōhū ʻili, ka maneʻo, ka ʻeha, a i kekahi manawa hiki i ka hōʻino nui ʻana o ke aʻalolo.
• Hana ʻia ka ʻike ma o nā hoʻokolohua lapaʻau a, inā pono, CT, MRI, a me PET scans.
• He nānā ʻana ka lāʻau lapaʻau, a i ʻole ke ʻoki ʻana inā pono.
• Kēia mau meaʻAʻole hiki ke pale ʻia no ka mea he mea hoʻoilina ia, akā hiki ke mālama ʻia nā hōʻailona.
• Inā he kānalua kāu e pili ana i kahi puʻupuʻu e like me kēia, ʻoi aku ka maikaʻi o ka hopohopo ʻole a ʻimi i ke kōkua lapaʻau .

E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. ʻO ka ʻike ʻana i kēia mau kūlana a me ka loaʻa ʻana o ke kōkua lapaʻau e pono ai e kōkua iā ʻoe e noho olakino.

ʻo neurofibroma, neurofibromatosis, NF1, nā puʻupuʻu ʻili, nā puʻupuʻu aʻalolo, nā kiko café-au-lait, nā maʻi hoʻoilina