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Introduction Meningiomas are common tumors of the central nervous system that represent around 30% of primary tumors. However, the incidence of atypical meningiomas (AMs) is lower, of approximately 15% of all meningiomas, and they present high rates of relapse and mortality. Aim To review peculiarities of AMs. Methodology A literature review of articles published in English between 2009 and 2020 on the PubMed and Biblioteca Virtual em Saúde (BVS) databases using the terms meningioma and atypical. Results Atypical meningiomas are rare and more prevalent in older males. The clinical condition depends on the site of the tumor, and a definitive diagnosis of AM is only possible via anatomical pathology. Moreover, prominent studies have indicated a relationship between the presence of the anti-Ki67 antibody/mindbomb (Ki -67/MIB1) marker as an aid in the definition of AM and one of the determinants of tumor aggressiveness. Imaging studies have also advanced in terms of seeking criteria using magnetic resonance that may suggest the malignancy of a lesion. As far as treatment is concerned, total resection remains the main therapeutic option, and it has a direct relationship with survival and time until disease progression. Lastly, there are many factors involved in the prognosis of AM. Conclusion Atypical meningiomas continue to be a challenge, requiring further and more specific studies to provide a better understanding of it.
Introdução Os meningiomas são tumores comuns do sistema nervoso central, representando cerca de 30% dos tumores primários. Contudo, a incidência de meningiomas atípicos (MA) é menor, cerca de 15% do total dos meningiomas, e eles apresentam altas taxas de recidiva e mortalidade. Metodologia Revisão da literatura, nas bases de dados PubMed e Biblioteca Virtual em Saúde (BVS), utilizando os termos "meningioma" e "atypical", publicados em língua inglesa entre 2009 e 2020. Objetivo Revisar as particularidades dos MA. Resultados MA são raros, mais prevalentes em homens de idade avançada. O quadro clínico depende da localização do tumor e o diagnóstico definitivo de MA só se dá por meio do anatomopatológico. Ademais, estudos importantes têm apontado para a relação da presença do marcador anticorpo monoclonal/mindbomb (Ki-67/MIB1) como auxiliador na definição de MA e um dos determinantes de agressividade tumoral. Os estudos de imagem também avançam no sentido de buscar critérios na ressonância magnética que possam sugerir a malignidade de uma lesão. Em relação ao tratamento, a cirurgia de ressecção total se mantém como principal terapêutica, com relação direta na sobrevida e no tempo livre de progressão de doença. Por fim, o prognóstico em MA é multifatorial. Conclusão MA continuam como um desafio, necessitando de mais estudos específicos para compreendê-los melhor.
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Objetivo: Avaliar o impacto orçamentário da inclusão da cladribina oral no tratamento de esclerose múltipla remitente-recorrente em alta atividade da doença (EMRR HDA) no Sistema de Saúde Suplementar (SSS). Métodos: Foi conduzida uma análise de impacto orçamentário, sob a perspectiva do SSS, com horizonte temporal de quatro anos, considerando a abordagem de coorte aberta na qual o número de pacientes elegíveis para tratamento varia em cada ano com a introdução de novos pacientes diagnosticados de EMRR HDA e a retirada de indivíduos prevalentes devido a morte ou progressão secundária. Foram considerados custos médicos diretos, incluindo a aquisição e administração de medicamentos, monitoramento, eventos adversos e surtos. Os comparadores utilizados na análise foram: alentuzumabe, fingolimode, natalizumabe e ocrelizumabe. Os custos foram apresentados em real brasileiro (BRL). Resultados: O custo incremental da inclusão da cladribina oral para o SSS foi estimado em 463.265 BRL, 739.691 BRL, -1.414.963 BRL, -3.719.007 BRL, nos anos 1, 2, 3 e 4, respectivamente. Isso resultou em um custo incremental total de -3.931.015 BRL no período analisado, representando 1,5% da redução orçamentária total no tratamento de EMRR HDA. Conclusão: A inclusão da cladribina oral para o tratamento de pacientes com diagnóstico de EMRR HDA poderia gerar uma economia substancial para o sistema brasileiro de saúde suplementar, atingindo um valor de cerca de 3,9 milhões de BRL em um período de quatro anos
Objective: To evaluate the budget impact of adopting cladribine tablets as a treatment strategy for relapsing remitting multiple sclerosis with high disease activity (RRMS HDA), from the Brazilian private healthcare system perspective. Methods: A budget impact analysis, under private healthcare system perspective, with a 4-years time horizon was conducted, considering the open cohort approach in which the number of patients eligible for treatment varies each year with the introduction of newly diagnosed RRMS HDA patients and the drop out of prevalent individuals due to death or secondary progression. Direct medical costs, including acquisition, drug administration, monitoring, adverse events and relapses were considered. Comparators used in the analysis were: alentuzumab, fingolimod, natalizumab and ocrelizumab. Costs were presented in Brazilian real (BRL). Results: The incremental cost of incorporating cladribine tablets into the private healthcare system was estimated at 463,265BRL, 739,961BRL, -1,414,963 BRL, -3,716,007 BRL, in years 1, 2, 3 and 4, respectively. This resulted in a total incremental cost of -3,931,015 BRL over the period analyzed, representing 1.5% of the total budget reduction in the treatment of RRMS HDA. Conclusion: Incorporation of cladribine tablets for the management of RRMS HDA could generate substantial savings for the private healthcare system, reaching a value of approximately 3.9 million BRL in a 4-years period
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Esclerose Múltipla Recidivante-Remitente , Saúde Suplementar , Análise de Impacto Orçamentário de Avanços Terapêuticos , Esclerose MúltiplaRESUMO
OBJECTIVE: ANO5-related myopathy is an important cause of limb-girdle muscular dystrophy (LGMD) and hyperCKemia. The main descriptions have emerged from European cohorts, and the burden of the disease worldwide is unclear. We provide a detailed characterization of a large Brazilian cohort of ANO5 patients. METHODS: A national cross-sectional study was conducted to describe clinical, histopathological, radiological, and molecular features of patients carrying recessive variants in ANO5. Correlation of clinical and genetic characteristics with different phenotypes was studied. RESULTS: Thirty-seven patients from 34 nonrelated families with recessive mutations of ANO5 were identified. The most common phenotype was LGMD, observed in 25 (67.5%) patients, followed by pseudometabolic presentation in 7 (18.9%) patients, isolated asymptomatic hyperCKemia in 4 (10.8%) patients, and distal myopathy in a single patient. Nine patients presented axial involvement, including one patient with isolated axial weakness. The most affected muscles according to MRI were the semimembranosus and gastrocnemius, but paraspinal and abdominal muscles, when studied, were involved in most patients. Fourteen variants in ANO5 were identified, and the c.191dupA was present in 19 (56%) families. Sex, years of disease, and the presence of loss-of-function variants were not associated with specific phenotypes. INTERPRETATION: We present the largest series of anoctaminopathy outside Europe. The most common European founder mutation c.191dupA was very frequent in our population. Gender, disease duration, and genotype did not determine the phenotype.
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Anoctaminas/genética , Doenças Musculares/patologia , Adolescente , Adulto , Idoso , Brasil , Criança , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/diagnóstico por imagem , Músculo Esquelético/patologia , Doenças Musculares/diagnóstico por imagem , Distrofia Muscular do Cíngulo dos Membros , Mutação , Fenótipo , Adulto JovemRESUMO
Sporadic inclusion body myositis (sIBM) is considered the most common acquired myopathy aged over 50 years. The disease is characterized by a particular process of muscle degeneration characterized by abnormal deposit of protein aggregates in association with inflammation. The aim of this study was to present clinical and muscle histopathological findings, including immunostaining for LC3B, p62, α-synuclein, and TDP-43, in 18 patients with sIBM. The disease predominated in males (61%) and European descendants, with onset of clinical manifestations around 59 years old. The most common symptoms were muscle weakness, falls, dysphagia, and weight loss. Hypertension was the main comorbidity. Most of the cases presented with paresis predominantly proximal in lower limbs and distal in upper limbs. Immunosuppressive treatment showed to be not effective. Muscle histological findings included dystrophic changes, endomysial inflammation, increased lysosomal activity, and presence of rimmed vacuoles and of beta-amyloid accumulation, in addition to high frequency of mitochondrial changes. There was increased expression of LC3B, p62, α-synuclein, and TDP-43 in muscle biopsies. The sIBM has characteristic clinical and histological findings, and the use of degeneration and autophagic markers can be useful for the diagnosis.
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Autofagia , Biomarcadores/análise , Miosite de Corpos de Inclusão/patologia , Miosite/patologia , Adulto , Idoso , Biópsia , Feminino , Humanos , Inflamação/patologia , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/patologia , Vacúolos/metabolismoRESUMO
BACKGROUND: The number of patients with chronic kidney disease is increasing worldwide, as well as the number of patients in kidney transplant waiting lists. In order to prevent infections related to immunosuppressive therapy, immunization guidelines for CKD patients before transplantation have been proposed. The aim of the present study was to evaluate adherence to immunization in a cohort of CKD patients in transplant waiting list and their renal replacement therapy clinics. METHODS: CKD patients older than 18 years old, receiving renal replacement therapy longer than 12 months and included in kidney transplant waiting list at University of Campinas (Unicamp) were enrolled. RESULTS: From February 2014 to December 2015, 105 patients fulfilled the inclusion criteria. Complete hepatitis B vaccination was observed in 73% and influenza vaccine in 67%. None of the other vaccine protocols reached 50% of coverage. Patients receiving immunization at primary health units presented higher coverage for diphtheria, tetanus (dT), measles, mumps, rubella (MMR), and hepatitis B vaccines, while patients immunized at renal replacement therapy clinics showed higher prevalence of pneumococcus (pneumo23). CONCLUSION: The low rates of immunization could reflect the RRT's clinics knowledge about the vaccines guidelines and its application on daily care. We suggest an integration between transplant center and RRT clinics, through lectures, periodic checking of vaccination cards, and easy to follow guidelines in order to provide a better vaccine coverage and to obtain higher immunization rates.
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Imunização/métodos , Falência Renal Crônica/imunologia , Vacinação/efeitos adversos , Listas de Espera , Acesso à Informação , Adulto , Estudos Transversais , Difteria/prevenção & controle , Feminino , Vacinas contra Hepatite B/administração & dosagem , Vacinas contra Hepatite B/efeitos adversos , Humanos , Vacinas contra Influenza/administração & dosagem , Vacinas contra Influenza/efeitos adversos , Influenza Humana/prevenção & controle , Transplante de Rim/efeitos adversos , Masculino , Pessoa de Meia-Idade , Cooperação do Paciente , Tétano/prevenção & controle , Toxoide Tetânico/administração & dosagem , Toxoide Tetânico/efeitos adversos , Vacinação/estatística & dados numéricosRESUMO
OBJECTIVE: The purpose of this prospective study is to assess MRI findings in patients with sporadic inclusion body myositis (IBM) and correlate them with clinical and functional parameters. SUBJECTS AND METHODS: This study included 12 patients with biopsy-proven sporadic IBM. All patients underwent MRI of the bilateral upper and lower extremities. The images were scored for muscle atrophy, fatty infiltration, and edema pattern. Clinical data included onset and duration of disease. Muscle strength was measured using the Medical Research Council (MRC) scale, and functional status was assessed using the Modified Rankin Scale. Correlation between MRI and different clinical and functional parameters was calculated using the Spearman rank test and Pearson correlation. RESULTS: All patients showed MRI abnormalities, which were more severe within the lower limbs and the distal segments. The most prevalent MRI finding was fat infiltration. There was a statistically significant correlation between disease duration and number of muscles infiltrated by fat (r = 0.65; p = 0.04). The number of muscles with fat infiltration correlated with the sum of the scores of MRC (r = -0.60; p = 0.04) and with the Modified Rankin Scale (r = 0.48; p = 0.03). CONCLUSION: Our findings suggest that most patients with biopsy-proven sporadic IBM present with a typical pattern of muscle involvement at MRI, more extensively in the lower extremities. Moreover, MRI findings strongly correlated with clinical and functional parameters, because both the extent and severity of muscle involvement assessed by MRI and clinical and functional parameters are associated with the early onset of the disease and its duration.
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Imageamento por Ressonância Magnética/métodos , Miosite de Corpos de Inclusão/diagnóstico por imagem , Idoso , Biópsia , Avaliação da Deficiência , Extremidades , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miosite de Corpos de Inclusão/fisiopatologia , Estudos ProspectivosRESUMO
Este estudo é uma discussão a partir do subprojeto de uma pesquisa que tem a intenção de investigar a relação público-privado a partir dos usos que a iniciativa privada estabelece sobre os espaços públicos. Analisaram-se os usos das orlas em projetos de intervenção com esporte e lazer e de como opera a política, de tal forma a identificar como estas ações incidem sobre os direitos constitucionalmente previstos de acesso ao esporte e ao lazer. Nos procedimentos metodológicos, utilizamos como planos de pesquisa os estudos descritivo e explicativo; mapeamentos e questionários com os responsáveis pelas intervenções. Os resultados apontam para concepções sobre espaço público como espaço para todos, mas, ocupado de acordo com interesses pessoais; concepção unívoca de benefícios nas parceiras e; oferta de serviços sem institucionalização e regulamentação, mostrando ausência de responsabilidades do Estado.
This study is a discussion from the subproject of a research that intends to investigate the public-private relationship of the uses of the private sector on public spaces. It was analyzed the uses of waterfront beaches in intervention projects with sports and leisure and how politics operates in such a way to identify how these actions affect the rights constitutionally previewed to this access to sport and leisure. In the methodological procedures, it was used as research plans the descriptive and explanatory studies; mapping and questionnaires with those responsible for interventions. The results indicate conceptions of public space as a space for everyone, but, used according to personal interests; univocal conception of the partner benefits and; offer without institutionalization and regulation services, showing the absence of responsibilities of the State.
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Atividades de LazerRESUMO
A miosite por corpos de inclusão (inclusion body myositis - IBM), na sua forma esporádica, é considerada a miopatia adquirida mais comum após os 50 anos de idade. Embora seja incluída no grupo das miopatias inflamatórias, estudos recentes mostram um processo particular de degeneração muscular caracterizado por deposição anormal de agregados de proteínas nas fibras musculares e funcionamento anormal dos principais sistemas de degradação proteica. O objetivo deste estudo foi o de avaliar os aspectos clínicos, histológicos e imunoistoquímicos de pacientes com IBM. Avaliamos 18 casos com diagnóstico de IBM de dois dos principais centros de doenças neuromusculares do Brasil (25 biópsias musculares). Na tentativa de diferenciar os casos de IBM das outras miopatias inflamatórias, determinamos o padrão de expressão tecidual da p-tau (p62), alfa-sinucleína e TDP-43. Também foi avaliada a função lisossomal através da reação da fosfatase ácida (marcação da atividade lisossomal global) e determinação da marcação para LC3B (marcador de autofagia). Foi observado que a IBM predominou no sexo masculino (61% dos casos), da cor branca, com início das manifestações clínicas ao redor dos 59 anos de idade e os sintomas mais frequentes foram fraqueza muscular, instabilidade postural com quedas da própria altura, disfagia e perda ponderal, podendo ainda apresentar dispneia. O diagnóstico demorou em média 7,4 anos após o início dos sintomas e frequentemente esteve associada às seguintes comorbidades: hipertensão arterial sistêmica, diabetes mellitus tipo 2, osteopenia / osteoporose, dislipidemia e hiperuricemia / gota. O padrão de comprometimento muscular na IBM foi caracterizado por tetraparesia de predomínio proximal em membros inferiores e distal em membros superiores. Os valores séricos da creatinofosfoquinase em pelo menos uma das medições foram elevados em todos os pacientes, porém sem ultrapassar 10 vezes o limite superior da normalidade. O uso de...
Sporadic inclusion body myositis (sIBM) is considered the most common acquired myopathy affecting adults aged over 50 years. Although included in the group of inflammatory myopathies, recent studies show a particular process of muscle degeneration characterized by abnormal deposit of protein aggregates in muscle fibers and abnormal operation of the main protein degradation systems. The aim of this study was to evaluate the clinical, histological and immunohistochemical patients with IBM. We evaluated 18 cases with IBM diagnostic of two of the main centers of neuromuscular diseases in Brazil (25 muscle biopsies). In an attempt to differentiate the IBM cases of other inflammatory myopathies, we determined the pattern of tissue expression of p-tau (p62), alfa-synuclein and TDP-43. Also evaluated the lysosomal function by acid phosphatase reaction (marking global lysosomal activity) and determining the markup for LC3B (autophagy marker). It was observed that IBM was predominant in males (61% of cases), white colored, with onset of clinical manifestations around 59 years old and the most common symptoms are muscle weakness, postural instability with high falls, dysphagia and weight loss, and may also present dyspnea. The diagnosis took an average of 7.4 years after the onset of symptoms and was often associated with the following comorbidities: hypertension, type 2 diabetes mellitus, osteopenia / osteoporosis, dyslipidemia and hyperuricemia / gout. The muscular damage pattern at IBM was characterized by tetraparesis predominantly proximal lower limbs and distal upper limbs. Serum creatine kinase levels in at least one of the measurements were elevated in all patients, but not exceeding 10 times normal. Immunosuppression was not effective in patients with IBM. The IBM histological findings included diversify dystrophic changes, endomysial inflammation, as well as the occurrence of rimmed vacuoles, in addition to high frequency of mitochondrial changes. Other...
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Humanos , Masculino , Feminino , Autofagia , Imuno-Histoquímica , Inflamação , Lisossomos , Mitocôndrias Musculares , Atrofia Muscular , Miosite , Miosite de Corpos de InclusãoRESUMO
BACKGROUND: Available prognostic scores for mortality after acute variceal bleeding are mainly based on logistic regression analysis but may have some limitations that can restrict their clinical value. AIMS: To assess the efficacy of a novel prognostic approach based on Classification and Regression Tree -CART- analysis to common easy-to-use models (MELD and Child-Pugh) for predicting 6-week mortality in patients with variceal bleeding. METHODS: Sixty consecutive cirrhotic patients with acute variceal bleeding. CART analysis, MELD and Child-Pugh scores were performed to assess 6-week mortality. Receiver operating characteristic (ROC) curves were constructed to evaluate the predictive performance of the models. RESULTS: Six-week rebleeding and mortality were 30% and 22%, respectively. Child-Pugh and MELD scores were clinically relevant for predicting 6 weeks mortality. CART analysis provided a simple algorithm based on just three bedside-available variables (albumin, bilirubin and in-hospital rebleeding), allowing accurate discrimination of two distinct prognostic subgroups with 3% and 80% mortality rates. All MELD, Child-Pugh and CART models showed excellent and comparable predictive accuracy, with areas under the ROC curves (AUROC) of 0.88, 0.84 and 0.91, respectively. CONCLUSIONS: A simple CART algorithm combining albumin, bilirubin and in-hospital rebleeding allows an accurate predictive assessment of 6-week mortality after acute variceal bleeding.
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Varizes Esofágicas e Gástricas/mortalidade , Hemorragia/mortalidade , Cirrose Hepática/mortalidade , Modelos Estatísticos , Adulto , Algoritmos , Bilirrubina/sangue , Varizes Esofágicas e Gástricas/sangue , Varizes Esofágicas e Gástricas/diagnóstico , Feminino , Hemorragia/sangue , Hemorragia/diagnóstico , Humanos , Estimativa de Kaplan-Meier , Cirrose Hepática/sangue , Cirrose Hepática/diagnóstico , Masculino , Pessoa de Meia-Idade , Prognóstico , Curva ROC , Estudos Retrospectivos , Albumina Sérica/metabolismo , Fatores de TempoRESUMO
Motor cortex stimulation oriented by functional cortical mapping is used mainly for treating otherwise intractable neurological disorders, however, its mechanism of action remains elusive. Herein, we present a new method for functional mapping of the rat motor cortex using non-invasive transdural electrical stimulation. This method allows a non-invasive mapping of the surface of the neocortex providing a differentiation of representative motor areas. This study may facilitate further investigation about the mechanisms mediating the effects of electrical stimulation, possibly benefiting patients who do not respond to this neuromodulation therapy.