Neurofibromatosis: ke eng, mefuta, lisosa le kalafo

Litaba

• Mefuta e meholo ea neurofibromatosis
• Ke eng e bakang neurofibromatosis
• Kalafo e etsoa joang

Neurofibromatosis, eo hape e tsejoang e le lefu la Von Recklinghausen, ke lefu le futsitseng le iponahatsang le le lilemo li ka bang 15 mme le baka kholo e sa tloaelehang ea lisele tsa methapo 'meleng oohle, li etsa maqhutsu a manyane le lihlahala tse kantle, tse bitsoang neurofibromas.

Ka kakaretso, neurofibromatosis e kotsi ebile ha e hlahise kotsi ea bophelo bo botle, leha ho le joalo, kaha e baka ponahalo ea lihlahala tse nyane tsa kantle, e ka lebisa ho senyeheng 'meleng, ho etsang hore batho ba anngoeng ba ikemisetse ho etsa opereishene ho ba tlosa.

Le ha neurofibromatosis e se na pheko, kaha lihlahala li ka hola hape, kalafo ka ho buuoa kapa kalafo ea radiation e ka lekoa ho leka ho fokotsa boholo ba lihlahala le ho ntlafatsa ponahalo ea botle ba letlalo.

Mefuta e meholo ea neurofibromatosis

Neurofibromatosis e ka aroloa ka mefuta e meraro:

• Mofuta oa 1 oa Neurofibromatosis: e bakoang ke liphetoho tse bang teng ho chromosome 17 tse fokotsang tlhahiso ea neurofibromine, protheine e sebelisoang ke 'mele ho thibela ponahalo ea lihlahala. Mofuta ona oa neurofibromatosis o ka baka tahlehelo ea pono le ho hloka matla;
• Mofuta oa 2 oa Neurofibromatosis: e bakiloeng ke liphetoho tse chromosome 22, e fokotsang tlhahiso ea merlina, protheine e 'ngoe e hatellang kholo ea lihlahala ho batho ba phetseng hantle. Mofuta ona oa neurofibromatosis o ka baka tahlehelo ea kutlo;
• Schwannomatosis: ke mofuta oa lefu o sa tloaelehang haholo oo lihlahala li hlahang lehata, lesapo la mokokotlo kapa methapo ea kutlo. Ka kakaretso, matšoao a mofuta ona a hlaha pakeng tsa lilemo tse 20 le 25.

Ho latela mofuta oa neurofibromatosis, matšoao a ka fapana. Kahoo, sheba matšoao a atileng haholo bakeng sa mofuta o mong le o mong oa neurofibromatosis.

Ke eng e bakang neurofibromatosis

Neurofibromatosis e bakoa ke liphetoho tsa liphatsa tsa lefutso liphatseng tse ling tsa lefutso, haholo-holo chromosome 17 le chromosome 22. Ho feta moo, maemo a sa tloaelehang a Schwannomatosis a bonahala a bakoa ke liphetoho liphatseng tsa lefutso tse kang SMARCB1 le LZTR. Liphatsa tsohle tsa lefutso tse fetotsoeng li bohlokoa ho thibela tlhahiso ea lihlahala, ka hona, ha li ameha, li lebisa ponahalong ea lihlahala tse khethollang neurofibromatosis.

Le ha boholo ba linyeoe tse fumanoeng li fetisoa ho tloha ho batsoali ho ea ho bana, ho boetse ho na le batho bao e kanna eaba ha ba so ka ba ba le mathata a lefu lena ka lapeng.

Kalafo e etsoa joang

Kalafo ea neurofibromatosis e ka etsoa ka ho buuoa ho tlosa lihlahala tse behang khatello lithong kapa kalafo ea radiation ho fokotsa boholo ba tsona. Leha ho le joalo, ha ho na kalafo e netefatsang pheko kapa e thibelang ho hlaha ha lihlahala tse ncha.

Maemong a boima ka ho fetisisa, moo mokuli a tšoaroang ke mofetše, ho ka hlokahala hore a alafshoe ka chemotherapy kapa kalafo ea radiation e lebisitsoeng ho lihlahala tse kotsi. Fumana lintlha tse ling tsa kalafo ea neurofibromatosis.