Relação entre o bem-estar subjetivo e o autocuidado em cuidadores em tempos pandêmicos / Relationship between subjective well-being and self-care among caregivers in pandemic times

O autocuidado se refere à práticas de prevenção de doenças e de manutenção da saúde. A capacidade de autocuidado pode ser um fator importante em diferentes condições e contextos, e associados aos aspectos da saúde do indivíduo podem proporcionar benefícios diretos a esta pessoa. Durante a Pandemia (COVID-19) esta capacidade pode estar diminuída ou prejudicada, e ainda imbricado com aspectos afetivos, de forma a influenciar o seu bem-estar subjetivo, ou seja, afetar sua avaliação do próprio bem-estar. Assim, esse Estudo teve como Objetivo avaliar a relação entre capacidade de autocuidado e a atribuição afetiva (positiva ou negativa) de adultos que atuam como cuidadores, dos gêneros masculino e feminino. Os instrumentos utilizados foram um questionário sociodemográfico, a Escala de Afetos Positivos e Negativos e a Escala para Avaliar as Capacidades de Autocuidado. Após a análise de frequência das variáveis, passou-se a análise de correlação de Pearson e por meio dela foram reveladas associações moderadas (médio altas) entre a medida de afeto positivo e autocuidado (r=0,62; p=0,000) e afeto negativo e autocuidado, neste caso, com coeficiente negativo (r=-0,42; p=0,000), indicando assim, que pessoas tendem a ter mais autocuidado, apresentar melhor afeto positivo e menor afeto negativo, respectivamente. Pôde-se inferir a ideia de que bem-estar e qualidade de vida estão imbricados à atribuição afetiva do indivíduo.

Self-care refers to disease prevention and health maintenance practices. Self-care capacity can be an important factor in different conditions and contexts and when associated with aspects of an individual's health it can provide direct benefits to this person. During the Pandemic (COVID-19) this ability may be diminished or impaired, and even hindered with affective aspects, influencing their subjective well-being, that is, affecting their assessment of their own well-being. Thus, this study aimed to evaluate the relationship between self-care capacity and the affective attribution (positive or negative) of male and female adults who act as caregivers. The instruments used were a sociodemographic questionnaire, the Positive and Negative Affect Schedule and the Scale to Assess Self-Care Capabilities. After analyzing the frequency of the variables, Pearson's correlation analysis was performed and through it, moderate associations (medium high) were revealed between the measure of positive affect and self-care (r=0.62; p=0.000) and negative affect and self-care, in this case, with a negative coefficient (r=-0.42; p=0.000); thus, this indicates that people tend to have more self-care, have better positive affects and less negative affects, respectively. It was possible to infer the idea that well-being and quality of life are intertwined with the affective attribution of the individual.

Reduced bone mineral content and density in neurofibromatosis type 1 and its association with nutrient intake.

BACKGROUND Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disease characterized by multisystem involvement including low bone mineral density (BMD). OBJECTIVE To assess the bone phenotype of individuals with NF1 and verify its association with nutrient intake. METHODS Twenty-six adults with NF1 underwent bone phenotype assessments using dual-energy X-ray absorptiometry (DXA) and food intake evaluations. They were compared to 26 unaffected matched control patients. Weight, height, and waist circumference (WC) were measured. DXA provided total body, spine, and hip BMDs and bone mineral content (BMC) for all patients. Food intake was evaluated for energy, macro- and micro-nutrients. RESULTS Height (1.68 ± 0.1; 1.61 ± 0.1 cm; P = 0.003) and BMC (2.3 ± 0.4; 2.0 ± 0.5 kg; P = 0.046) were lower in the NF1 group. Individuals with NF1 also presented lower total body and spine BMDs (g/cm2) (1.1 ± 0.1, 1.0 ± 0.1, P = 0.036; 1.0 ± 0.1, 0.9 ± 0.1; P = 0.015, respectively). The frequency of total body bone mass below the expected level for patients' ages was higher in the NF1 group (7.7%; 34.6%, P = 0.016). There were no differences in energy consumption. No correlations between BMC and BMD with nutrient intake were observed in the NF1 group. CONCLUSIONS The NF1 group presented lower BMCs and BMDs. Although a lower consumption of calcium, iron, and vitamin A, and a higher intake of sodium and omega-6 were observed, there was no relationship between bone phenotype and nutrient intake.

Reduced bone mineral content and density in neurofibromatosis type 1 and its association with nutrient intake

SUMMARY BACKGROUND Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disease characterized by multisystem involvement including low bone mineral density (BMD). OBJECTIVE To assess the bone phenotype of individuals with NF1 and verify its association with nutrient intake. METHODS Twenty-six adults with NF1 underwent bone phenotype assessments using dual-energy X-ray absorptiometry (DXA) and food intake evaluations. They were compared to 26 unaffected matched control patients. Weight, height, and waist circumference (WC) were measured. DXA provided total body, spine, and hip BMDs and bone mineral content (BMC) for all patients. Food intake was evaluated for energy, macro- and micro-nutrients. RESULTS Height (1.68 ± 0.1; 1.61 ± 0.1 cm; P = 0.003) and BMC (2.3 ± 0.4; 2.0 ± 0.5 kg; P = 0.046) were lower in the NF1 group. Individuals with NF1 also presented lower total body and spine BMDs (g/cm2) (1.1 ± 0.1, 1.0 ± 0.1, P = 0.036; 1.0 ± 0.1, 0.9 ± 0.1; P = 0.015, respectively). The frequency of total body bone mass below the expected level for patients' ages was higher in the NF1 group (7.7%; 34.6%, P = 0.016). There were no differences in energy consumption. No correlations between BMC and BMD with nutrient intake were observed in the NF1 group. CONCLUSIONS The NF1 group presented lower BMCs and BMDs. Although a lower consumption of calcium, iron, and vitamin A, and a higher intake of sodium and omega-6 were observed, there was no relationship between bone phenotype and nutrient intake.

RESUMO INTRODUÇÃO A Neurofibromatose tipo 1 (NF1) é uma doença genética autossômica dominante caracterizada por envolvimento neurocutâneo e multissistêmico, incluindo baixa densidade mineral óssea (DMO). OBJETIVOS Avaliar características ósseas em indivíduos com NF1 e verificar associação com a ingestão de nutrientes. METODOLOGIA 26 adultos com NF1 submeteram-se a avaliação dos parâmetros ósseos usando absorciometria com raios-X de dupla energia (DXA), além da avaliação da ingestão alimentar. O grupo NF1 foi comparado e pareado com 26 indivíduos sem a doença. Peso, estatura e circunferência da cintura foram avaliados. DXA forneceu o conteúdo mineral ósseo (CMO) e a DMO do corpo total, coluna e fêmur. A ingestão de calorias, macronutrientes e micronutrientes foi avaliada. RESULTADOS O grupo NF1 apresentou redução da estatura (1,68 ± 0,1; 1,61 ± 0,1 cm; P=0,003) e do CMO (2,3 ± 0,4; 2,0 ± 0,5 kg; P=0,046). Indivíduos com NF1 também apresentaram redução da DMO de corpo total e coluna (g/cm2) (1,1 ± 0,1, 1,0 ± 0,1, P=0,036; 1,0 ± 0,1, 0,9 ± 0,1; P=0,015, respectivamente). A frequência de indivíduos com massa óssea abaixo do esperado para a idade foi maior no grupo NF1 (7,7%; 34,6%, P=0,016). Não houve diferenças no consumo energético. Não houve correlação entre CMO e DMO com a ingestão de nutrientes no grupo NF1. CONCLUSÕES O grupo NF1 apresentou redução do CMO e da DMO. Apesar de menor consumo de cálcio, ferro e vitamina A, e maior consumo de sódio e ômega-6, não foi observada relação entre o fenótipo ósseo e a ingestão de nutrientes.

Increased resting metabolism in neurofibromatosis type 1.

BACKGROUND & AIMS: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disease that is characterized by neurocutaneous changes with multisystem involvement. A previous study with adults with NF1 revealed that changes in total energy expenditure were related to food consumption and body composition. Resting energy expenditure (REE), a measure of energy that the body expends to maintain vital functions, has not been assessed in NF1 populations. This study aimed to assess REE in individuals with NF1 using indirect calorimetry (IC) and evaluate its correlation with body composition and muscle strength. METHODS: Twenty-six adults with NF1 (14 men) aged 18-45 years underwent IC for assessing REE, respiratory quotient (RQ), and substrate utilization. Body composition was assessed by dual energy X-ray absorptiometry. Weight, height, and waist circumference (WC) were also measured. Maximum muscular strength (Smax) was measured by handgrip test using a dynamometer. Patients in the NF1 group were compared to 26 healthy controls in the control group, who were matched by sex, age, body mass index (BMI), and physical activity level. RESULTS: There were no differences in weight, WC, fat mass, and body fat percentage (BFP). Appendicular lean mass (ALM) adjusted by BMI (ALMBMI) (0.828 ± 0.161 versus 0.743 ± 0.190; P = 0.048) and Smax (37.5 ± 10.6 versus 31.1 ± 12.2; P = 0.035) was lower in the NF1 group than in the control group. No differences in body composition, strength, and anthropometric parameters were observed in men, but women with NF1 presented lower body surface area (BSA), lean body mass (LBM), ALM, ALMBMI, and Smax. REE adjusted by weight, LBM, or ALM was higher in the NF1 group than in the control group (medians, 21.9 versus 26.3, P = 0.046; 36.5 versus 41.1, P = 0.012; and 82.3 versus 92.4, P = 0.006, respectively), and these differences were observed only among women. RQ was lower in the NF1 group than in the control group (0.9 ± 0.1 versus 0.8 ± 0.1; P = 0.008), revealing that individuals with NF1 oxidized more lipids and fewer carbohydrates than controls. REE correlated negatively with BFP and positively with weight, height, BMI, WC, BSA, LBM, ALM, ALMBMI, bone mineral content, and Smax. CONCLUSIONS: Individuals with NF1, particularly women, presented with increased REE (adjusted by weight, LBM, or ALM) and lower RQ compared to healthy controls. These findings were associated with lower ALMBMI and Smax, possibly indicating premature sarcopenia in this population. Further investigation concerning energy metabolism in NF1 and gender differences may be helpful in explaining underlying mechanisms of these changes.

Comportamento da pressão intraocular no tonômetro de aplanação de Goldman e pneumático durante o teste de sobrecarga hídrica / Intraocular pressure behavior in the Goldmann and pneumatic tonometer during water drinking test

Resumo Objetivo: Avaliar a concordância entre os tonômetros de aplanação de Goldman e pneumático na realização do teste de sobrecarga hídrica (TSH). Métodos: Estudo descritivo transversal composto por uma amostra de 102 olhos proveniente de um hospital particular em Goiânia (GO) entre 2013 a 2016, com avaliação das diferentes pressões intraoculares (PIO) nos tonômetros de aplanação de Goldman e pneumático quando submetidos ao TSH. Resultados: A média de idade foi de 52,17 (± 15,21) anos, sendo que60,8% dos pacientes pertenciam ao sexo feminino e 39,2% ao masculino. A média da espessura da córnea foi de 531,9(± 72,75) micra. Por regressão linear as variaveis idade e espessura da cornea central não ocorreram significancia estatistica entre os dois dispositivos analisados. Conclusão: Observou-se boa concordância nas medições entre os aparelhos de aplanação e o pneumático durante o teste de sobrecarga hídrica, porém necessita-se de novos estudos de maior impacto epidemiológico para confirmação desta assertiva.

Abstract Objective: To evaluate the consonance betweenthe Goldman and pneumática planation to nometers under the effect of the water drink test. Methods: Cross-sectional descriptive study consisting of a sample of 102 eyes from a private hospital in Goiânia (GO) from 2013 to 2015,with na evaluation of different intraocular pressures (IOP) in the Goldman and pneumatic flattening to nometers when submitted to TSH. Results: The average age was 52.17 (± 15.21) years old, 60.8% of the patients were female and 39.2% were male.The mean corneal thickness was 531.9(± 72.75) By linear regression the variables age and corneal thickness did not occur statistical significance between the two devices analyzed. Conclusion: Good agreement was observed in the measurements between the applanation devices and the tire during the water drink test, but new studies with a greater epidemiological impact were required to confirm this assertion.

Pileflebite: relato de caso / Pylephlebitis: case report

A Pileflebite é uma rara e grave complicação de diversas infecções intra-abdominais e pélvicas. Inicia-se através de uma tromboflebite de pequenos vasos que drenam uma área infectada e tem, como principais manifestações, a dor abdominal e a febre. É descrito caso de trombose séptica de veia porta em uma paciente do sexo feminino, 70 anos, hipertensa, obesa, portadora de neoplasia maligna de uretra e tabagista, que apresentou episódio de diverticulite aguda complicada com pileflebite. Tratamento realizado com antibioticoterapia e anticoagulação.

The pylephlebitis is a rare and serious complication of various abdominal and pelvic infections. It begins with thrombophlebitis of small vessels that drain an infected area, and has abdominal pain and fever as main manifestations. It describe the case of portal vein septic thrombosis in a female patient, 70 years, smoker, suffering from systemic hypertension, obesity and malignant neoplasm of urethra, who had an episode of acute diverticulitis complicated by pylephlebitis. Treatment instituted with antibiotics and anticoagulation.

Pneumatose intestinal: relato de caso / Pneumatosis intestinalis: case report

Pneumatose intestinal (PI) consiste em um achado de imagem correspondente à presença de ar na parede intestinal. É um sinal infrequente, mais comum em idosos e sem predileção por sexo. Existem várias causas relatadas na literatura, sendo alguns casos reportados como idiopáticos. A modalidade diagnóstica de maior sensibilidade é a tomografia computadorizada de abdome. A maioria dos casos é assintomática, porém, uma pequena parcela pode evoluir com complicações graves, necessitando de intervenções imediatas. A inexistência de um consenso a respeito do manejo dos pacientes com PI, somada ao pouco conhecimento dos médicos acerca dessa enfermidade, resulta em um cenário clínico delicado. O objetivo deste artigo é relatar o caso de um paciente com PI, destacando o manejo terapêutico adotado.

Pneumatosis intestinalis (PI) consists in an image finding corresponding to the presence of air in the intestinal wall. It is an infrequent sign, more common in the elderly and without gender predilection. There are numerous causes described in the literature, and some cases are referred as idiopathic. The diagnostic modality with the highest sensibility is the computed tomography of the abdomen. The majority of cases are asymptomatic, but a small portion can evolve with severe complications, needing immediate interventions. The lack of a consensus regarding PI patients' management, along with the low knowledge level of physicians about the existence of this disorder, results in a delicate clinical scenario. The objective of this article is to report the case of a patient with PI, highlighting the adopted therapeutic management.

Neurofibromatosis: part 2 – clinical management / Neurofibromatoses: parte 2 – manejo clínico

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 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.

Neurofibromatoses: part 1 diagnosis and differential diagnosis / Das neurofibromatoses: parte 1 diagnostico e diagnostico diferencial

Neurofibromatoses (NF) are a group of genetic multiple tumor growing predisposition diseases: neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis (SCH), which have in common the neural origin of tumors and cutaneous signs. They affect nearly 80 thousand of Brazilians. In recent years, the increased scientific knowledge on NF has allowed better clinical management and reduced complication morbidity, resulting in higher quality of life for NF patients. In most cases, neurology, psychiatry, dermatology, clinical geneticists, oncology and internal medicine specialists are able to make the differential diagnosis between NF and other diseases and to identify major NF complications. Nevertheless, due to its great variability in phenotype expression, progressive course, multiple organs involvement and unpredictable natural evolution, NF often requires the support of neurofibromatoses specialists for proper treatment and genetic counseling. This Part 1 offers step-by-step guidelines for NF differential diagnosis. Part 2 will present the NF clinical management.

Neurofibromatoses (NF) constituem um grupo de doenças genéticas com predisposição ao crescimento de múltiplos tumores: tipo 1 (NF1), tipo 2 (NF2) e schwannomatose (SCH). Estas doenças têm em comum a origem neural dos tumores e os sinais cutâneos. Afetam cerca de 80 mil brasileiros. O maior conhecimento científico sobre as NF tem permitido melhor manejo clínico, redução da morbidade das complicações e melhor qualidade de vida. Na maioria dos casos, os especialistas em neurologia, dermatologia, genética clínica, oncologia e medicina interna estão capacitados a realizar o diagnóstico diferencial e identificar suas principais complicações. Devido à sua variabilidade fenotípica, curso progressivo, multiplicidade de órgãos acometidos e evolução imprevisível, as NF frequentemente necessitam de especialistas em NF para o acompanhamento. A Parte 1 deste texto oferece orientações para o diagnóstico de cada tipo de NF e discute os diagnósticos diferenciais com outras doenças. A Parte 2 oferecerá orientações em relação ao manejo clínico das NF.

[Neurofibromatosis type 1: more frequent and severe then usually thought]. / Neurofibromatose tipo 1: mais comum e grave do que se imagina.

OBJECTIVE: To study prevalence of clinical features among Brazilian patients diagnosed with neurofibromatosis type 1(NF1), comparing these features with international data to evaluate the severity and visibility of the disease and quantify less frequent manifestations such as short stature, macrocephaly, muscle strength, voice abnormalities and oral motor disorders. METHODS: 183 patients diagnosed with NF1, attended at the Neurofibromatosis Outpatient Reference Center, were evaluated for clinical manifestations and complications of NF1. Severity and visibility were verified using the Riccardi and Ablon scales respectively. Voice abnormalities and oral motor disorders were quantified using the Vox-Metria software and maximal voluntary muscle strength (MVMS) was quantified using a handgrip dynamometer. RESULTS: Clinical manifestations of NF1 observed were comparable to those described in literature. However, more then 50% of patients presented severity and visibility classified as moderate and severe. The incidence of macrocephaly and short stature was higher among the Brazilian patients. Voice abnormalities and oral motor disorders were quantified for the first time, with hoarseness and oral motor disorders observed in more then 60% the patients. Maximal voluntary muscle strength was found to be reduced in 67% of patients. CONCLUSION: The main clinical features of these patients are similar to those reported in previous studies. More then one-half of the patients presented moderate and severe levels of NF1 (severity and visibility), including short stature, macrocephaly, voice abnormalities and oral motor disorders and decreased muscle strength. These results are in disagreement with the traditional concept that NF1 is a benign disease and also disclosed some clinical aspects not previously reported.

Neurofibromatose tipo 1: mais comum e grave do que se imagina / Neurofibromatosis type 1: more frequent and severe then usually thought

OBJETIVO: Determinar a prevalência das características clínicas da neurofibromatose tipo 1 (NF1), avaliar sua gravidade e visibilidade e quantificar baixa estatura, macrocrania, força muscular e as alterações da motricidade oral e da voz nesta enfermidade. MÉTODOS: 183 pacientes atendidos no Centro de Referência em Neurofibromatose de Minas Gerais (CRNF-MG) foram avaliados quanto à frequência de manifestações clínicas e complicações da NF1. A gravidade e a visibilidade foram avaliadas com o uso de escalas de Riccardi e Ablon respectivamente. As alterações da motricidade oral e da voz foram verificadas com uso do software Vox-Metria, e a medida quantitativa da força muscular realizada com uso de dinamômetro. RESULTADOS: As manifestações clínicas habituais da NF1 observadas neste estudo são semelhantes às descritas na literatura. Entretanto, mais de 50 por cento dos pacientes apresentaram gravidade e visibilidade moderada e grave. A incidência de macrocrania e baixa estatura foi maior nos pacientes do CRNF-MG. As alterações da voz e da motricidade oral foram pela primeira vez quantificadas, com rouquidão e distúrbios da motricidade oral observados em mais de 60 por cento dos pacientes. A força muscular estava reduzida em 67 por cento dos pacientes. CONCLUSÃO: O perfil clínico destes pacientes é semelhante aos relatos prévios da literatura. Mais da metade dos pacientes avaliados apresenta as formas moderada e grave da doença (gravidade e visibilidade), incluindo baixa estatura, macrocrania, distúrbios da voz e da motricidade oral e redução da força muscular. Os resultados desta pesquisa contrariam o conceito tradicional de que a NF1 é uma doença benigna e demonstram alguns aspectos clínicos, ainda não descritos na literatura.

OBJECTIVE: To study prevalence of clinical features among Brazilian patients diagnosed with neurofibromatosis type 1(NF1), comparing these features with international data to evaluate the severity and visibility of the disease and quantify less frequent manifestations such as short stature, macrocephaly, muscle strength, voice abnormalities and oral motor disorders. METHODS: 183 patients diagnosed with NF1, attended at the Neurofibromatosis Outpatient Reference Center, were evaluated for clinical manifestations and complications of NF1. Severity and visibility were verified using the Riccardi and Ablon scales respectively. Voice abnormalities and oral motor disorders were quantified using the Vox-Metria software and maximal voluntary muscle strength (MVMS) was quantified using a handgrip dynamometer. RESULTS: Clinical manifestations of NF1 observed were comparable to those described in literature. However, more then 50 percent of patients presented severity and visibility classified as moderate and severe. The incidence of macrocephaly and short stature was higher among the Brazilian patients. Voice abnormalities and oral motor disorders were quantified for the first time, with hoarseness and oral motor disorders observed in more then 60 percent the patients. Maximal voluntary muscle strength was found to be reduced in 67 percent of patients. CONCLUSION: The main clinical features of these patients are similar to those reported in previous studies. More then one-half of the patients presented moderate and severe levels of NF1 (severity and visibility), including short stature, macrocephaly, voice abnormalities and oral motor disorders and decreased muscle strength. These results are in disagreement with the traditional concept that NF1 is a benign disease and also disclosed some clinical aspects not previously reported.