Neurofibromatosis: part 2 – clinical management / Neurofibromatoses: parte 2 – manejo clínico
Arq. neuropsiquiatr
;
73(6): 531-543, 06/2015. tab, graf
Article
in English
| LILACS
| ID: lil-748178
ABSTRACT
Part 1 of this guideline addressed the differential diagnosis of the neurofibromatoses (NF) neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis (SCH). NF shares some features such as the genetic origin of the neural tumors and cutaneous manifestations, and affects nearly 80 thousand Brazilians. Increasing scientific knowledge on NF has allowed better clinical management and reduced rate of complications and morbidity, resulting in higher quality of life for NF patients. Most medical doctors are able to perform NF diagnosis, but the wide range of clinical manifestations and the inability to predict the onset or severity of new features, consequences, or complications make NF management a real clinical challenge, requiring the support of different specialists for proper treatment and genetic counseling, especially in NF2 and SCH. The present text suggests guidelines for the clinical management of NF, with emphasis on NF1.
RESUMO
A primeira parte desta diretriz abordou o diagnóstico diferencial das neurofibromatoses (NF) neurofibromatose do tipo 1 (NF1), neurofibromatose do tipo 2 (NF2) e schwannomatose (SCH). As NF compartilham algumas características, como a origem neural dos tumores e sinais cutâneos, e afetam cerca de 80 mil brasileiros. O aumento do conhecimento científico sobre as NF tem permitido melhor manejo clínico e redução da morbidade das complicações, resultando em melhor qualidade de vida para os pacientes com NF. A maioria dos médicos é capaz de realizar o diagnóstico das NF, mas a variedade de manifestações clínicas e a dificuldade de se prever o surgimento e a gravidade de complicações, torna o manejo da NF um desafio para o clínico e envolve diferentes especialistas para o tratamento adequado e aconselhamento genético, especialmente a NF2 e a SCH. O presente texto sugere algumas orientações para o acompanhamento dos portadores de NF, com ênfase na NF1.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Skin Neoplasms
/
Neurofibromatosis 1
/
Neurofibromatoses
/
Neurilemmoma
Type of study:
Etiology study
/
Practice guideline
/
Prognostic study
/
Risk factors
Limits:
Humans
Language:
English
Journal:
Arq. neuropsiquiatr
Journal subject:
Neurology
/
Psychiatry
Year:
2015
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Universidade Federal de Minas Gerais/BR
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