Neurofibromatosis: he aha ia, ʻano, kumu a me ka mālama ʻana

Anter

• Nā ʻano nui o neurofibromatosis
• He aha ke kumu o ka neurofibromatosis
• Pehea e hana ai i ka lāʻau

ʻO Neurofibromatosis, i ʻike ʻia ʻo ka maʻi ʻo Von Recklinghausen, kahi maʻi hoʻoilina i hōʻike ʻia iā ia iho ma kahi o 15 mau makahiki a kumu i ka ulu pono ʻole ʻana o nā mea hopohopo ma loko o ke kino, e hana ana i nā nodule liʻiliʻi a me nā hikoko o waho, i kapa ʻia ʻo neurofibromas.

ʻO ka mea maʻamau, maikaʻi ʻole ka neurofibromatosis a ʻaʻole hāʻawi i kahi pilikia olakino, akā, no ka mea ke kumu o nā kūpoko liʻiliʻi o waho, hiki iā ia ke alakaʻi i ka disfigurement o ke kino, kahi e manaʻo ai ka poʻe i hoʻopilikia ʻia e hana i kahi ʻoki e wehe iā lākou.

ʻOiai ʻaʻohe lāʻau o neurofibromatosis, no ka mea hiki i nā tumors ke ulu hou, hiki ke hoʻāʻo ʻia me ka ʻoki ʻana a i ʻole radiation radiation e hoʻāʻo e hoʻēmi i ka nui o nā tumors a hoʻomaikaʻi i ka nani o ka ʻili.

Nā ʻano nui o neurofibromatosis

Hiki ke hoʻokaʻawale ʻia ʻo Neurofibromatosis i ʻekolu mau ʻano:

• ʻO Neurofibromatosis ʻano 1: ma muli o ka hoʻololi ʻana i ka chromosome 17 e hōʻemi ana i ka hana o neurofibromine, kahi protein i hoʻohana ʻia e ke kino e pale ai i ka ʻike ʻia o nā tumors. Hiki i kēia ʻano neurofibromatosis ke kumu o ka nalowale o ka hihiʻo a me ka ikaika ʻole;
• ʻO Neurofibromatosis ʻano 2: ma muli o ka hoʻololi ʻana i ka chromosome 22, e hoʻemi ana i ka hana o merlina, kahi protein ʻē aʻe e kaomi i ka ulu ʻana o nā tumors i nā kānaka olakino. Hiki i kēia ʻano neurofibromatosis ke kumu o ka hoʻolohe ʻana i ka nalo ʻana;
• ʻO Schwannomatosis: ʻo ia ka ʻano lahilahi loa o nā maʻi e hoʻomohala ai nā tumors i ka iwi poʻo, iwi kuamoʻo a i ʻole nā ​​ʻalolo ākea. ʻO ka maʻamau, ʻike ʻia nā ʻōuli o kēia ʻano ma waena o nā makahiki 20 a 25.

Kaukaʻi ʻia i ke ʻano o ka neurofibromatosis, ʻokoʻa paha nā ʻōuli. No laila, e nānā i nā hōʻailona maʻamau no kēlā me kēia ʻano o neurofibromatosis.

He aha ke kumu o ka neurofibromatosis

Hoʻokumu ʻia ʻo Neurofibromatosis e nā loli kūpuna i kekahi mau genes, keu hoʻi ka chromosome 17 a me ka chromosome 22. Eia kekahi, ʻike ʻia nā hihia kakaikahi o Schwannomatosis e nā loli i nā genes kikoʻī e like me SMARCB1 a me LZTR. He mea nui nā genes āpau āpau i ka pāpā ʻana i ka hana ʻana o nā tumors a, no laila, ke pili lākou, alakaʻi lākou i ke ʻano o nā tumors i ke ʻano o neurofibromatosis.

ʻOiai ʻo ka hapanui o nā hihia i ʻike ʻia ua hala ʻia mai nā mākua a i nā keiki, aia kekahi poʻe ʻaʻole i loaʻa kekahi hihia o ka maʻi i ka ʻohana.

Pehea e hana ai i ka lāʻau

Hiki ke hana i ka lāʻau no ka neurofibromatosis ma o ke kaha ʻana e wehe i nā tumors e hoʻokau ana i nā ʻokena a i ʻole ma o radiation radiation e hoʻoliʻiliʻi i kā lākou nui. Eia nō naʻe, ʻaʻohe lāʻau lapaʻau e hōʻoiaʻiʻo ai i kahi lāʻau lapaʻau a i ʻole pale i ke ʻano o nā tumors hou.

I nā hihia koʻikoʻi loa, kahi e hoʻomohala ai ka mea maʻi i ka maʻi ʻaʻai, pono paha e hana i ka lāʻau me chemotherapy a i ʻole radiation therapy i kuhikuhi ʻia i nā tumele ʻino. E ʻike i nā kikoʻī hou aʻe no ka mālama ʻana no neurofibromatosis.