Mepolizumab versus benralizumab for eosinophilic granulomatosis with polyangiitis (EGPA): A European real-life retrospective comparative study

Background: Following the results of the MANDARA trial, this real-life study aimed at comparing the effectiveness and safety profile of mepolizumab versus benralizumab in a European EGPA cohort. Methods: We conducted a retrospective observational comparative study including EGPA patients, who received mepolizumab or benralizumab at the asthma dose. Patients were matched 1:1 by sex, age, BVAS and oral corticosteroid (OCS) dosage at the treatment initiation (T0). Complete response (CR) and partial response (PR), disease activity, OCS, pulmonary parameters, eosinophil count, relapses, and safety outcomes were also compared at 3, 6 and 12 months. Results: Patients treated with mepolizumab or benralizumab (n = 88 each) were matched: 57 % were females, median age was 54 years (IQR 45–60), median OCS dose 10 (7.5–12.5) and 10 (7–13) mg/day, median BVAS 4 (2–7) and 3 (2–8), respectively. 45.4 % of patients in the mepolizumab group and 51.1 % in the benralizumab group achieved CR or PR at T3, with CR steadily increasing during follow-up for both treatments. At T12, a higher CR rate was found in the benralizumab group (48.1 % vs 32.4 %, p = 0.005). No differences in BVAS, OCS, and respiratory parameters were observed between groups at the different timepoints. Throughout the follow-up, both treatments reduced eosinophil count, although a deeper reduction was found in the benralizumab group at all timepoints (p < 0.0001). Safety profile was comparable between patient groups. Conclusion: Mepolizumab and benralizumab showed comparable overall effectiveness and safety in EGPA. However, benralizumab achieved a higher CR rate at T12, and a deeper peripheral eosinophil reduction

Influence of gender on Behçet's disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry

Objectives. - Gender impact on phenotypical expression of Behcet's disease (BD) has been specifically investigated only in a few large-scale studies. The main goal of the study was to examine gender differences in a large cohort of patients affected by BD.Methods. - Data were retrieved from the International AIDA Network Registry for BD. We assessed differences between males and females in terms of Behcet's syndrome Overall Damage Index (BODI), differences in the disease manifestations at onset and in the cumulative manifestations throughout diseasecourse, as well as differences in the cardiovascular risk. Finally, predictive factors leading to major organ involvement were investigated.Results. - In total, 1024 BD patients (567 males, 457 females) were enrolled in the study, with a male-to-female ratio of 1.24/1. Males displayed a significantly higher mean +/- SD BODI (1.92 +/- 2.09) at the last follow-up, compared to female patients (1.25 +/- 1.87) (P < 0.0001). Uveitis (P < 0.0001) and vascular involvement (P = 0.0076) were significantly more frequent among males whereas female patients were significantly over-represented in arthralgia (P < 0.0001), arthritis (P = 0.00025), isolated headache (P < 0.0001), central nervous system (CNS) involvement (P = 0.040), and gastrointestinal involvement (P = 0.00046). Regarding cardiovascular risk, no differences between the two groups emerged (P = 0.617). Four variables were associated with the development of major organ involvement: male gender (OR = 2.104, P = 0.001), current treatment with biologic agents (OR = 2.257, P = 0.0003), origin from endemic countries (OR = 2.661, P = 0.0009), and disease duration (OR = 1.002, P = 0.024).Conclusion. - BD displays a more severe course among males. This subgroup develops more irreversible damage and presents more frequently ocular and vascular involvement during disease course. On the other hand, female patients are prone to experience articular involvement, headache, CNS and gastrointestinal involvement. These data suggest the existence of a gender-driven disease expression

Comunicación interna para mejorar la gestión de la calidad en la farmacia INKAFARMA del distrito de Yungay, 2023.

Hoy en día las empresas enfrentan un mercado globalizado y exigente por lo que es necesario que se maneje un estilo de comunicación que se adecue a la realidad empresarial, y son pocas que notan esta variable como parte indispensable de la estructura, por otro lado, la gestión de calidad se convirtió un objetivo fundamental para cualquier actividad económica puesto que, es necesario la administración de los recursos materiales y el talento humano, sin embargo, se denota un desorden administrativo para inspeccionar sus operaciones. Por lo que se planteó como objetivo general: Implementar la comunicación interna para mejorar la gestión de la calidad en la Farmacia Inkafarma del distrito de Yungay, 2023. La metodología que se utilizó fue tipo cuantitativo, nivel descriptivo, diseño no experimental y corte transversal, la técnica fue la encuesta e instrumento el cuestionario, fue aplicado a una muestra de 6 empleados. Donde se obtuvo que, el 50% indicaron que siempre el supervisor les da conocer sobre los objetivos y metas y el 50% señalaron que siempre y casi siempre se maneja una comunicación adecuada. En conclusión, ha demostrado que la comunicación interna, en la empresa InkaFarma se maneja siempre y casi siempre favoreciendo al logro de objetivos en el tiempo determinado, respecto, a la gestión de calidad, se fomenta siempre la cultura de calidad y pese a ello no hay una supervisión suficiente lo que genera interés menor para el desempeño laboral

Clinical and laboratory features associated with macrophage activation syndrome in Still’s disease: data from the international AIDA Network Still’s Disease Registry

To characterize clinical and laboratory signs of patients with Still's disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. Patients with Still's disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still's Disease Registry. Clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still's disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). At multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9-52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9-97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still's disease onset (OR 0.6, 95% CI 0.4-0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01-0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0-0.2, p = 0.008) resulted to be protective. Clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data. © 2023, The Author(s)

Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry

: To characterize clinical and laboratory signs of patients with still's disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. patients with still's disease classified according to internationally accepted criteria were enrolled in the autoInflammatory disease alliance (AIDA) still's disease registry. clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still's disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). at multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9-52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9-97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still's disease onset (OR 0.6, 95% CI 0.4-0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01-0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0-0.2, p = 0.008) resulted to be protective. clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data

O teletrabalho em home office sob a perspectiva do direito fundamental da privacidade do trabalhador

Dissertação apresentada ao Programa de Pós-Graduação em Direito da Universidade do Extremo Sul Catarinense - UNESC, como requisito parcial para a obtenção do título de Mestre em Direito.A privacidade do teletrabalhador em home office está inserida dentro dos direitos da personalidade, erigida como direito fundamental sob o manto da dignidade da pessoa humana. Por tratar-se de características e atributos da pessoa humana, tem irradiação imediata no contrato de trabalho, podendo ser exercido ou reclamado durante uma relação de trabalho. Nesse contexto, quais os limites e as restrições do exercício do Teletrabalho em home office a serem observados pelo empregador de modo a não violar o direito fundamental à privacidade do trabalhador? Visando responder esse problema de pesquisa, primeiramente examina-se a teoria dos direitos fundamentais, acerca dos seus aspectos conceituais, dimensões e eficácia, bem como especialmente sua eficácia nas relações de emprego. Num segundo momento, fazse uma análise crítica dos diversos marcos normativos sobre teletrabalho em home office na legislação brasileira, como também trata-se do seu conceito, características, obrigações, vantagens e desvantagens. Ao final, busca-se defender que, independentemente dos avanços tecnológicos, ferramentas de trabalho ou modalidades de trabalho, os direitos fundamentais inespecíficos, por se tratarem de direitos vinculados a pessoa humana, devem ser respeitados, devendo ser estabelecidos limites no teletrabalho em home office a fim de preservar o direito fundamental à privacidade do trabalhador. Concluiu-se que deve-se buscar a utilização de métodos de controle menos invasivos, assim como a utilização de qualquer método de controle não poderá ocorrer de maneira oculta e sem o conhecimento prévio do trabalhador. Que todo e qualquer recolhimento de dados e informações pelo empregador deve-se ater à relação de emprego, não podendo invadir a intimidade e vida privada do trabalhador e que os dados e informações armazenados ou recolhidos não podem ocorrer por prazo indefinido. Além disso, que o empregador jamais poderá ceder ou dispor para terceiros dados ou informações colhidos dos trabalhadores e que o empregador deverá obedecer às diretrizes dos direitos fundamentais sociais trabalhistas elencados na Constituição Federal. O método utilizado neste trabalho é o dedutivo, cuja técnica de pesquisa é documental e bibliográfica, materializada na pesquisa de doutrina, jurisprudência e legislação

The HBP1 tumor suppressor is a negative epigenetic regulator of MYCN driven neuroblastoma through interaction with the PRC2 complex

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