Neurofibromatosis nyaéta gangguan multi-sistem manusa kompléks disababkeun ku mutasi neurofibromin. Neurofibromatosis ngabalukarkeun tumor sapanjang sistim saraf nu bisa tumuwuh di mana waé dina awak. Neurofibromatosis nyaéta salah sahiji gangguan genetik anu paling umum sareng gangguan dominan autosomal.
Gejala umum tina neurofibromatosis ngawengku bintik-bintik beureum semu coklat dina bagian panon anu warnana disebut nodul Lisch sareng neurofibroma. Scoliosis (curvature tina tulang tonggong), cacad diajar, gangguan visi, cacad méntal, sababaraha café au lait spot jeung epilepsy bisa ogé dibarengan.
Neurofibromatosis type I (NF-1) is a complex multi-system human disorder caused by the mutation of a gene on chromosome 17 that is responsible for production of a protein, called neurofibromin, which is needed for normal function in many human cell types.
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Cafe au lait macule katingal dina Neurofibromatosis type 1 (NF-1)
Neurofibromatosis mangrupikeun kaayaan anu nyababkeun tumor dina sistem saraf sareng kulit. Tipe 1 diwariskeun sacara dominan sareng nunjukkeun gejala sapertos neurofibromas, café-au-lait spots, freckling, and optic gliomas. Diagnosis dumasar kana tanda klinis. Tipe 2, di sisi séjén, ditandaan ku bilateral vestibular schwannomas (VS) and meningiomas , ogé diwariskeun dominan. Manajemén ngalibatkeun pangawasan rutin sareng perawatan médis upami diperyogikeun pikeun duanana jinis neurofibromatosis. Neurofibromatosis is a neurocutaneous disorder characterized by tumors in the nervous system and skin. Neurofibromatosis types 1 and 2 are the most common and are distinct entities. Neurofibromatosis type 1, or von Recklinghausen disease, is an autosomal dominant disease. Neurofibromatosis type 1 presents with neurofibromas, cafe-au-lait macules, freckling, and optic gliomas. It is a clinical diagnosis. Neurofibromatosis type 2 (NF2) is a disease characterized by bilateral vestibular schwannomas (VS) and meningiomas. It has an autosomal dominant inheritance pattern. Treatment for neurofibromatosis types 1 and 2 is clinical monitoring and medical intervention when appropriate.
Gejala umum tina neurofibromatosis ngawengku bintik-bintik beureum semu coklat dina bagian panon anu warnana disebut nodul Lisch sareng neurofibroma. Scoliosis (curvature tina tulang tonggong), cacad diajar, gangguan visi, cacad méntal, sababaraha café au lait spot jeung epilepsy bisa ogé dibarengan.
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