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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.

A sudorese é o principal mecanismo autonômico termorregulatório na espécie humana. A taxa de sudorese (TS) durante o exercício pode ser influenciada por diversos fatores, entre eles doenças, como o diabetes mellitus tipo 1, cujos pacientes são vítimas potenciais de distúrbios termorregulatórios. Os exercícios regulares são recomendados para diabéticos e, inclusive, alguns deles tornam-se atletas. No entanto, ainda não foi medida a TS de diabéticos durante exercício progressivo até a exaustão (GXT) e comparada com a de indivíduos sadios com características antropométricas semelhantes. O presente estudo comparou a TS de sete voluntários diabéticos (DM) e sete controles (GC), semelhantes quanto à idade, gênero, composição corporal e capacidade aeróbica. Antes e após o GXT, foram medidos a gravidade específica da urina (GEU), o peso e a glicemia capilar. A TS global foi calculada dividindo-se a variação do peso pelo tempo de coleta e área de superfície corporal. A TS local do antebraço foi calculada utilizando-se a variação do peso de um filtro absorvente corrigido pela sua área e tempo de coleta. As TS locais do antebraço e da perna foram induzidas por iontoforese com pilocarpina (0,5%) no grupo DM. A freqüência cardíaca (FC) foi registrada antes, durante e após GXT. A temperatura média da pele (TMP) e as condições térmicas ambientais foram medidas durante o experimento. A glicemia capilar foi maior no DM, como o esperado. As TS global e local foram semelhantes entre os grupos, enquanto que a FC no repouso e a TMP no repouso e no exercício foram maiores no DM. Não houve diferença entre as TS do antebraço e da perna no DM com a pilocarpina. Concluiu-se que a resposta sudorípara foi semelhante entre diabéticos jovens e grupo controle durante exercício progressivo em ambiente temperado. Sudoresis is the main autonomic human thermoregulatory mechanism. It can be influenced by several factors, including diseases, among them diabetes mellitus Type 1 (DM1), whose patients are potential victims of thermoregulatory disturbances. Regular physical exercises are recommended to diabetic people and some of them even become athletes. However, up to now, the sweat rate (SR) of diabetic patients during graded exercise until exhaustion (GXT) has not been compared to the SR of healthy individuals with similar physical characteristics. The present study measured global (SRglobal) and local (SRlocal) sweat rates during GXT in 14 diabetic (DM) and control subjects (CG), similar in age, gender, body composition and aerobic capacity. Urine specific gravity (Gu), body mass and blood glucose (Gblood) were measured before and after GXT. Besides that, Srlocal (forearm and leg) was measured by iontophoresis with pilocarpine (0.5%) in the DM group. Heart rate (HR), skin temperature (Tsk) and environmental thermal conditions were measured during the experiment. Both initial and final Gblood were higher in the DM, as expected. The Gu were similar between groups. Exercise induced similar SRglobal and SRlocal in both groups, whereas the rest HR and rest and exercise Tsk were higher in the DM. Forearm and leg SR were not different in the DM. It was concluded that the sweating response was not different between young diabetic patients and control group during GXT in a temperate environment.