Objective: To assess the association of sleep disorders with the findings of heart rate variability (HRV) in children and adolescents with cystic fibrosis (CF). Methods: Cross-sectional study including children and adolescents aged six to 18 years with a clinical diagnosis of CF. Sociodemographic and clinical data were collected. Sleep disorders were evaluated using baseline nocturnal polysomnography. The autonomic nervous system (ANS) was evaluated through resting HRV. Results: A total of 30 individuals (11.2 years) with a mean forced expiratory volume in the first second (FEV1) of 62.7% were included. The respiratory disturbance index presented a median of 2.6 and obstructive sleep apnea syndrome (OSAS) was identified in 30%. In the HRV analysis, a mean standard deviation of all inter-beat (RR) intervals (SDNN) of 60.8±45.9ms was found. There was a significant correlation between the HRV low-frequency/high-frequency (LF/HF) global modulation index and the minimum SpO2 during sleep in patients with FEV1<60% (r=0.71; p=0.02). The prevalence of sleep disorders and HRV abnormalities was higher in individuals with lesser pulmonary function (FEV1<60%). Conclusions: The results indicate a weak correlation of sleep disorders (minimum SpO2) with HRV parameters (LH/HF) in children and adolescents with CF. When pulmonary function was reduced, a stronger correlation was found, highlighting the influence of disease severity. A high prevalence of ANS disorders, nocturnal hypoxemia, and presence of OSAS was also found.
Introdução: Tumor estromal gastrointestinal (GIST) é uma doença relativamente rara e a cirurgia se constitui no tratamento principal deste tumor. Existem apenas poucos centros com experiência no tratamento desta neoplasia. O objetivo do trabalho é analisar os resultados do tratamento cirúrgico dos pacientes portadores de GIST gástrico operados em uma mesma instituição. Métodos: Estudo retrospectivo baseado na análise de 20 pacientes com diagnóstico confirmado de GIST gástrico operados no Instituto Nacional do Câncer (INCA- Rio de Janeiro) entre 1986 e 2000. Todos os dados foram revisados, enfocando-se as características histopatológicas (localização, número de mitoses e tamanho do tumor), as características dos pacientes (idade, sexo e apresentação clínica) e os resultados cirúrgicos (tipos de cirurgia, morbidade, mortalidade e sobrevida de acordo com a classificação proposta por Shiu). Resultados: Onze pacientes eram do sexo masculino e 9 do sexo feminino. A mediana de idade foi de 57 anos. O tamanho médio do tumor foi de 14,7 cm. Setenta por cento dos pacientes tinham tumor de alto grau e 65% dos tumores se localizavam na porção proximal do estômago. Gastrectomia subtotal foi realizada em 50% dos casos, gastrectomia total em 35% e gastrectomia atípica em 10%. Ressecção gástrica isolada foi realizada em seis casos (grupo 1), ressecção de dois órgãos foi feita em cinco casos (grupo 2) e ressecção de mais de dois órgãos em oito casos (grupo 3). A morbidade operatória global foi de 35%. O grupo 1 não teve morbidade, o grupo 2 teve morbidade de 20% e o grupo 3, de 70%. A mortalidade operatória foi de 10% (2 pacientes). Dez por cento dos pacientes foram classificados como estádio 0, 25% como estádio I e 65%, estádio II de acordo com a classificação de Shiu. A sobrevida em 5 anos para o estádio 0 foi de 100% enquanto que o estádio II apresentou sobrevida nula em 5 anos. Conclusão: A alta morbi-mortalidade desta casuística é devido à presença de doença avançada ao diagnóstico, havendo necessidade de ressecções multiorgânicas a fim de se obter cirurgia R0. A cirurgia é o tratamento principal, porém isolada é insuficiente para se atingir sobrevida longa em doença avançada.
Os schwannomas são tumores benignos, geralmente solitários, encapsulados e de crescimento lento, que têm sua origem nas células de Schwann neoplásicas diferenciadas, com desenvolvimento habitual extramedular intradural relacionado a raízes nervosas. O schwannoma melanocítico é variante dessas neoplasias cuja localização em quase um terço dos casos está na raiz nervosa posterior, com apresentação clínica inespecífica. A ressonância magnética é o exame de imagem mais utilizado em seu diagnóstico, revelando imagens hiperintensas em T1 e hipointensas em T2. A confirmação diagnóstica é obtida a partir do estudo histológico e imuno-histoquímico, em que se observa intensa pigmentação citoplasmática. Existem dois tipos distintos de schwannomas melanocíticos: o esporádico e o psammomatoso, este último relacionado ao chamado complexo de Carney, uma forma de neoplasia endócrina múltipla de caráter familiar. Existem poucos relatos dessas neoplasias, sendo a maior série composta de cinco casos. O objetivo deste relato é descrever um raro caso de schwannoma melanocítico da coluna lombar do tipo esporádico de localização extramedular. Apresenta-se breve revisão de literatura contendo as principais características do tumor, incluindo suas diferentes formas, diagnósticos diferenciais, estudo histológico e imuno-histoquímico, bem como a abordagem atualmente preconizada, a fim de colaborar para o melhor entendimento dessa neoplasia.
Background: Autonomic nervous system balance is altered in cystic fibrosis (CF), although its influence on physical fitness has been poorly explored. Objective: This study aimed to evaluate the association of heart rate variability (HRV) with exercise capacity and levels of daily physical activity in children and adolescents with mild-to-moderate CF. Methods: A cross-sectional study including individuals with CF aged 6–18 years, not under CFTR modulator therapy, was performed. Sociodemographic (age, sex) and clinical information (airway colonization, pancreatic insufficiency, and genotyping) were collected. In addition, exercise capacity (modified shuttle test — MST), lung function (spirometry), body composition (bioimpedance), levels of daily physical activity (5-day accelerometer), and HRV (both at rest and during the MST) were evaluated. Results: 30 individuals (20 females) aged [Formula: see text] years, mean FEV[Formula: see text]%, were included. A sympathovagal balance (LF/HF) increase ([Formula: see text]) during the MST was shown, indicating a predominance of sympathetic modulation. The standard deviation of all RR intervals (SDNN) and the high frequency (HF) index during exercise correlated significantly with FEV1 ([Formula: see text], [Formula: see text] and [Formula: see text], [Formula: see text]; respectively). MST distance also correlated positively and significantly with SDNN ([Formula: see text], [Formula: see text]), square root of the mean of the sums of squares of frequencies between RR intervals greater than 50[Formula: see text]ms — RMSSD ([Formula: see text], [Formula: see text]), low frequency — LF ([Formula: see text], [Formula: see text]), HF ([Formula: see text], [Formula: see text]), dispersion of points perpendicular to the short-term identity line — SD1 ([Formula: see text], [Formula: see text]) and negatively with LF/HF ([Formula: see text], [Formula: see text]). Regarding daily physical activity, SDNN at rest ([Formula: see text], [Formula: see text]) and exercise ([Formula: see text], [Formula: see text]) showed positive correlations with time in moderate-to-vigorous activities. When normalizing the SDNN and classifying individuals as normal or altered, those presenting altered SDNN showed poorest FEV1 ([Formula: see text]) and lower exercise capacity ([Formula: see text]). Conclusion: HRV correlates with lung function, exercise capacity and levels of daily physical activity in children and adolescents with CF. The study highlights the influence of CF on autonomic function and suggests HRV measurement as an easy tool to be used in clinical settings as an alternative marker to monitor CF individuals.
Schwannomas are benign tumors, usually solitary, encapsulated, slow-growing, which have their origin in differentiated neoplastic Schwann cells with extramedullary intradural usual development related to nerve roots. The melanotic schwannoma is a variant of these tumors whose location in almost one third of cases is on the posterior spinal nerve root, with a nonspecific clinical presentation. Magnetic resonance imaging is the most widely used test for the diagnosis, revealing hyperintense T1-weighted sequences and hypointense T2-weighted sequences. Diagnostic confirmation is obtained by histological and immunohistochemical studies, in which there is intense cytoplasmatic pigmentation. There are two distinct types of melanotic schwannomas: sporadic and psammomatous, the latter related to the called Carney complex, a form of multiple endocrine neoplasm with familiar character. In literature we found few cases of these neoplasms, the largest series consisting of five cases. The objective of this study is to report a rare case of melanotic schwannoma of the lumbar spine of the sporadic type of extramedullary location. We also present a brief review of the literature containing the main characteristics of the tumor, including its different forms, differential diagnoses, data from histological and immunohistochemical studies as well as the currently recommended approach in order to contribute to a better understanding of this neoplasm.Keywords: Spine; Spinal neoplasms; Schwann Cells; Manipulation, spinal. RESUMO Os schwannomas são tumores benignos, geralmente solitários, encapsulados e de crescimento lento, que têm sua origem nas células de Schwann neoplásicas diferenciadas, com desenvolvimento habitual extramedular intradural relacionado com as raízes nervosas. O schwannoma melanocítico é uma variante dessas neoplasias cuja localização em quase um terço dos casos está na raiz nervosa posterior
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