I-Neurofibromatosis: yini, izinhlobo, izimbangela kanye nokwelashwa

-Delile

• Izinhlobo eziyinhloko ze-neurofibromatosis
• Yini ebangela i-neurofibromatosis
• Yelashwa kanjani

I-Neurofibromatosis, eyaziwa nangokuthi isifo sikaVon Recklinghausen, yisifo esizalwa njengefa esizibonakalisa sineminyaka yobudala eyi-15 futhi sidala ukukhula okungajwayelekile kwezicubu zemizwa emzimbeni wonke, kwakheka amaqhuqhuva amancane nezicubu zangaphandle, ezibizwa nge-neurofibromas.

Ngokuvamile, i-neurofibromatosis inobungozi futhi ayivezi ingozi yezempilo, noma kunjalo, njengoba ibangela ukuvela kwamathumba amancane angaphandle, kungaholela ekukhubazekeni komzimba, okwenza abantu abathintekayo bahlose ukuhlinzwa ukuze basuswe.

Yize i-neurofibromatosis ingenakho ukwelashwa, njengoba izimila zingakhula zibuyele emuva, ukwelashwa ngokuhlinzwa noma ukwelashwa ngemisebe kungazama ukuzama ukunciphisa ubukhulu bezimila nokwenza ngcono ukubukeka kwesikhumba.

Izinhlobo eziyinhloko ze-neurofibromatosis

I-Neurofibromatosis ingahlukaniswa ngezinhlobo ezintathu:

• Uhlobo lwe-Neurofibromatosis 1: kubangelwe ukuguqulwa kwe-chromosome 17 eyehlisa ukukhiqizwa kwe-neurofibromine, iphrotheni esetshenziswa ngumzimba ukuvimbela ukuvela kwezimila. Lolu hlobo lwe-neurofibromatosis lungadala ukulahleka kombono nokungabi namandla;
• Uhlobo lwe-Neurofibromatosis 2: kubangelwe ukuguquka kwezakhi zofuzo ku-chromosome 22, kunciphisa ukukhiqizwa kwe-merlina, enye iphrotheni ecindezela ukukhula kwezimila kubantu abaphilile. Lolu hlobo lwe-neurofibromatosis lungadala ukulahleka kokuzwa;
• I-Schwannomatosis: wuhlobo lwesifo oluyivelakancane lapho izicubu zikhula khona kugebhezi, umgogodla noma izinzwa ezisemaphethelweni. Ngokuvamile, izimpawu zalolu hlobo zivela phakathi kweminyaka engama-20 nengama-25.

Ngokuya ngohlobo lwe-neurofibromatosis, izimpawu zingahluka. Ngakho-ke, hlola izimpawu ezijwayelekile kunhlobo ngalunye lwe-neurofibromatosis.

Yini ebangela i-neurofibromatosis

I-Neurofibromatosis ibangelwa izinguquko zofuzo kwezinye izakhi zofuzo, ikakhulukazi i-chromosome 17 ne-chromosome 22. Ngaphezu kwalokho, izimo ezingavamile zeSchwannomatosis zibonakala zidalwa izinguquko ezakhiweni zofuzo ezinjengeSMARCB1 neLZTR. Zonke izakhi zofuzo ezishintshiwe zibalulekile ekuvimbeleni ukukhiqizwa kwezicubu futhi, ngakho-ke, uma zithinteka, ziholela ekubonakaleni kwezimila ezaziwa nge-neurofibromatosis.

Yize iningi lamacala atholakele lidluliselwa lisuka kubazali liye ezinganeni, kukhona nabantu okungenzeka ukuthi abakaze babe namacala esifo emndenini.

Yelashwa kanjani

Ukwelashwa kwe-neurofibromatosis kungenziwa ngokuhlinzwa ukuze kususwe izicubu ezifaka ingcindezi ezithweni noma ngokusebenzisa i-radiation therapy ukunciphisa usayizi wazo. Kodwa-ke, akukho muthi oqinisekisa ukwelashwa noma ovimbela ukuvela kwezimila ezintsha.

Ezimweni ezinzima kakhulu, lapho isiguli sihlaselwa ngumdlavuza, kungadingeka ukwelashwa ngamakhemikhali noma ngemisebe ebhekiswe kwizicubu ezimbi. Thola imininingwane eminingi yokwelashwa kwe-neurofibromatosis.