Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin.

Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality.

Endovascular therapy for posterior cerebral artery occlusion: systematic review with meta-analysis.

Endovascular therapy (EVT) is a highly effective stroke treatment, but trials validating this intervention did not include patients with posterior cerebral artery (PCA) occlusion. The aim of this systematic review with meta-analysis was to assess the efficacy and safety of EVT for acute PCA occlusion. PubMed, Scopus, ISI, and CENTRAL were searched for studies assessing EVT in adult patients with PCA occlusion. Outcomes of interest were recanalization, symptomatic intracerebral haemorrhage (sICH), mortality, functional independence, and excellent functional outcome at 90 days. Frequencies and odds ratios (ORs) were pooled using random effect models and heterogeneity was measured using the I2 statistic and explored by means of meta-regression. Fifteen studies were included, all observational. Recanalization rates were high [81%, 95% CI (73-88%)] and sICH rates low [2%, 95% CI (1-4%)]. Heterogeneity was high for recanalization (I2 = 80%) but not for sICH, and not accounted for by any of the moderators tested. Compared to best medical treatment, EVT was associated with higher chances of sICH [OR = 2.04, 95% CI (1.12-3.71)] and no effect in functional independence [OR = 0.98, 95% CI (0.63-1.54)], with a tendency to higher chances of excellent functional outcome [OR = 1.29, 95% CI (0.90-1.86)] and mortality [OR = 1.56, 95% CI (0.84-2.90)]. EVT for acute PCA occlusion is technically feasible but associated with higher chance of sICH. There is no evidence to support this treatment to achieve higher rates of functional independence, but other gains that can impact patients' quality of life cannot be excluded. More studies are required with robust design, better patient selection, and comprehensive outcome evaluation.

Emerging therapeutic strategies in hypoxic-ischemic encephalopathy: a focus on cognitive outcomes.

Perinatal hypoxia-ischemia represents a significant risk to CNS development, leading to high mortality rates, diverse damages, and persistent neurological deficits. Despite advances in neonatal medicine in recent decades, the incidence of HIE remains substantial. Motor deficits can manifest early, while cognitive impairments may be diagnosed later, emphasizing the need for extended follow-up. This review aims to explore potential candidates for therapeutic interventions for hypoxic-ischemic encephalopathy (HIE), with a focus on cognitive deficits. We searched randomized clinical trials (RCT) that tested drug treatments for HIE and evaluated cognitive outcomes. The results included studies on erythropoietin, melatonin, magnesium sulfate, topiramate, and a combination of vitamin C and ibuprofen. Although there are several indications of the efficacy of these drugs among animal models, considering neuroprotective properties, the RCTs failed to provide complete effectiveness in the context of cognitive impairments derived from HIE. More robust RCTs are still needed to advance our knowledge and to establish standardized treatments for HIE.

Mycotoxins evaluation of total mixed ration (TMR) in bovine dairy farms: An update.

The total mixed ration (TMR) is currently a widespread method to feed dairy cows. It is a mixture of raw fodder and concentrate feed that can be contaminated by several mycotoxins. The main aim of this paper was to provide a critical review on TMR mycotoxin occurrence and its usefulness to monitor and control them on-farm. Aflatoxins, zearalenone, deoxynivalenol, T-2 toxin and fumonisins (regulated mycotoxins) are the most prevalent mycotoxins evaluated in TMR. Nonetheless, several emerging mycotoxins represent a health risk at the animal level regarding their prevalence and level in TMR. Even when measured at low levels, the co-occurrence of mycotoxins is frequent and synergistic effects on animal health are still underevaluated. Similar to the animal feed industry, on-farm plans monitoring mycotoxin feed contamination can be developed as a herd health management program. The estimated daily intake of mycotoxins should be implemented, but thresholds for each mycotoxin are not currently defined in dairy farms.

Autoinflammatory diseases: a Latin American multicenter study according to age and sex / Doenças autoinflamatórias: um estudo multicêntrico latino-americano de acordo com o sexo e a idade

ABSTRACT Objective: To evaluate autoinflammatory diseases (AID) according to age at diagnosis and sex, and response to therapy in a large population. Methods: This is a cross-sectional observational study of a Latin American registry using a designed web system for data storage, collected between 2015 and 2018. Any altered findings during follow-up were recorded. The forms were translated into Portuguese and Spanish, including demographic, clinical, laboratory, genetic and treatment characteristics. Results: We included 152 patients, 51.3% male and 75% Caucasian. The median age at disease onset was 2.1 years (0-15.6 years) and median age at diagnosis 6.9 years (0-21.9 years); 111 (73%) were children (0-9 years old), and 41 (27%) were adolescents and young adults (AYA) (10-21 years old). Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) occurred in 46/152 (30%), chronic non-bacterial osteomyelitis (CNO) in 32/152 (21%), and familial Mediterranean fever (FMF) in 24/152 (15.7%). PFAPA was significantly higher in young children than in AYA (38.7% vs. 7.3%, p<0.001), while CNO were lower (13.5% vs. 41.5%, p<0.001). The frequency of females was significantly higher in CNO (28.4% vs. 14.1%, p=0.031) and lower in FMF (8.1% vs. 23.1%, p=0.011). The most used drugs were glucocorticoids, non-steroidal anti-inflammatory drugs (NSAID), and colchicine. Glucocorticoids and colchicine treatment were used in all AID with good to moderate response. However, cryopyrin-associated periodic syndromes (CAPS) seemed unresponsive to glucocorticoids. NSAIDs and methotrexate were the main medications used to treat CNO. Conclusions: Differences among AID patients were observed in the LA population regarding sex and age at disease diagnosis.

RESUMO Objetivo: Avaliar as doenças autoinflamatórias (DAI) de acordo com sexo e idade no momento do diagnóstico e a resposta terapêutica em uma grande população. Métodos: Este é um estudo observacional transversal de um registro latino-americano que usou um sistema de dados coletados entre 2015 e 2018. Quaisquer achados alterados ao longo do acompanhamento foram registrados. Os formulários foram traduzidos para os idiomas português e espanhol, incluindo características demográficas, clínicas, laboratoriais, genéticas e de tratamento. Resultados: Incluímos 152 pacientes, sendo 51,3% do sexo masculino e 75% da raça branca. A média de idade de início da doença foi de 2,1 anos (0-15,6 anos) e a média de idade de diagnóstico 6,9 anos (0-21,9 anos); 111 (73%) eram crianças (0-9 anos) e 41 (27%) adolescentes/adultos jovens (10-21 anos). A síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA) ocorreu em 46/152 (30%), osteomielite não bacteriana crônica (CNO) em 32/152 (21%) e febre familiar do Mediterrâneo (FMF) em 24/152 (15,7%). A PFAPA foi significativamente maior em crianças pequenas (38,7 vs. 7,3%, p<0,001), e a CNO, em adolescentes/adultos jovens (13,5 vs. 41,5%, p<0,001). A frequência do sexo feminino foi significativamente maior na CNO (28,4 vs. 14,1%, p=0,031) e menor na FMF (8,1 vs. 23,1%, p=0,011). Os medicamentos mais utilizados foram glicocorticoides, anti-inflamatórios não esteroidais (AINE) e colchicina. O tratamento com glicocorticoides e colchicina foi usado em todas as DAI com resposta boa a moderada. No entanto, as síndromes periódicas associadas à criopirina (CAPS) pareciam não responder aos glicocorticoides. AINE e metotrexato foram os principais medicamentos utilizados no tratamento da CNO. Conclusões: Diferenças de pacientes com DAI foram observadas na população latino-americana em pacientes agrupados por sexo e idade ao diagnóstico da doença.

Shorter Reperfusion Time in Stroke is Associated with Better Cognition.

BACKGROUND: Cognitive changes that result from cerebrovascular disease contribute to a poor functional outcome with reduced quality of life. Among patients undergoing endovascular therapy (EVT), we aim to assess cognitive function and evaluate the impact of reperfusion time in cognitive performance. METHODS: Patients with acute right anterior circulation strokes that underwent EVT between January 2018 and August 2020 at Centro Hospitalar de Vila Nova de Gaia/Espinho, participated in the study. Modified treatment in cerebral infarction (mTICI) assessed the level of recanalization. Cognitive evaluation was assessed with Addenbrooke's Cognitive Examination revised (ACE-R). Multiple linear regression analyses were used to determine the association between time for recanalization and ACE-R. The level of significance adopted was 0.05. RESULTS: The mean age of participants was 71.5 (interquartile range [IQR] 62.0-78.2) years, and 50% (22) were women. The median time after stroke was 28.6 months (IQR 18.94-31.55). All patients in our sample had a successful level of recanalization with EVT (mTICI ≥ 2b). Time for recanalization showed an inverse association with the ACE-R (b = -0.0207, P = 0.0203). Also the mRS at 3 months had an inverse association with cognition (b = -5.2803, p = 0.0095). Level of education had a strong and direct relationship with ACE-R results (b = 3.0869, p < 0.0001). CONCLUSIONS: Longer time between stroke symptoms and recanalization with EVT in patients with right hemisphere ischemic stroke lead to lower ACE-R scores. Measures to improve door-to-recanalization time are also important for cognitive performance after ischemic stroke.

Metabolic and Molecular Rearrangements of Sauvignon Blanc (Vitis vinifera L.) Berries in Response to Foliar Applications of Specific Dry Yeast.

Dry yeast extracts (DYE) are applied to vineyards to improve aromatic and secondary metabolic compound content and wine quality; however, systematic information on the underpinning molecular mechanisms is lacking. This work aimed to unravel, through a systematic approach, the metabolic and molecular responses of Sauvignon Blanc berries to DYE treatments. To accomplish this, DYE spraying was performed in a commercial vineyard for two consecutive years. Berries were sampled at several time points after the treatment, and grapes were analyzed for sugars, acidity, free and bound aroma precursors, amino acids, and targeted and untargeted RNA-Seq transcriptional profiles. The results obtained indicated that the DYE treatment did not interfere with the technological ripening parameters of sugars and acidity. Some aroma precursors, including cys-3MH and GSH-3MH, responsible for the typical aromatic nuances of Sauvignon Blanc, were stimulated by the treatment during both vintages. The levels of amino acids and the global RNA-seq transcriptional profiles indicated that DYE spraying upregulated ROS homeostatic and thermotolerance genes, as well as ethylene and jasmonic acid biosynthetic genes, and activated abiotic and biotic stress responses. Overall, the data suggested that the DYE reduced berry oxidative stress through the regulation of specific subsets of metabolic and hormonal pathways.

Identification of eight novel proteasome variants in five unrelated cases of proteasome-associated autoinflammatory syndromes (PRAAS).

Mutations in genes coding for proteasome subunits and/or proteasome assembly helpers typically cause recurring autoinflammation referred to as chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures (CANDLE) or proteasome-associated autoinflammatory syndrome (PRAAS). Patients with CANDLE/PRAAS present with mostly chronically elevated type I interferon scores that emerge as a consequence of increased proteotoxic stress by mechanisms that are not fully understood. Here, we report on five unrelated patients with CANDLE/PRAAS carrying novel inherited proteasome missense and/or nonsense variants. Four patients were compound heterozygous for novel pathogenic variants in the known CANDLE/PRAAS associated genes, PSMB8 and PSMB10, whereas one patient showed additive loss-of-function mutations in PSMB8. Variants in two previously not associated proteasome genes, PSMA5 and PSMC5, were found in a patient who also carried the PSMB8 founder mutation, p.T75M. All newly identified mutations substantially impact the steady-state expression of the affected proteasome subunits and/or their incorporation into mature 26S proteasomes. Our observations expand the spectrum of PRAAS-associated genetic variants and improve a molecular diagnosis and genetic counseling of patients with sterile autoinflammation.

Depressive-like behavior and impaired synaptic plasticity in the prefrontal cortex as later consequences of prenatal hypoxic-ischemic insult in rats.

Perinatal hypoxia-ischemia (HI) is a leading cause of morbidity and mortality among newborns. Infants with HI encephalopathy may experience lasting consequences, such as depression, in adulthood. In this study, we examined depressive-like behavior, neuronal population, and markers of monoaminergic and synaptic plasticity in the prefrontal cortex (PFC) of adolescent rats subjected to a prenatal HI model. Pregnant rats underwent a surgery in which uterine and ovarian blood flow was blocked for 45 min at E18 (HI procedure). Sham-operated subjects were also generated (SH procedure). Behavioral tests were conducted on male and female pups from P41 to P43, and animals were histologically processed or dissected for western blotting at P45. We found that the HI groups consumed less sucrose in the sucrose preference test and remained immobile for longer periods in the forced swim test. Additionally, we observed a significant reduction in neuronal density and PSD95 levels in the HI group, as well as a smaller number of synaptophysin-positive cells. Our results underscore the importance of this model in investigating the effects of HI-induced injuries, as it reproduces an increase in depressive-like behavior and suggests that the HI insult affects circuits involved in mood modulation.

Clinical features, treatment and outcome of pediatric patients with severe cutaneous manifestations in IgA vasculitis: Multicenter international study.

OBJECTIVE: IgA vasculitis (IgAV) (formerly Henoch-Schönlein Purpura, HSP) rarely causes severe skin lesions in children. The purpose of the research was to determine whether severe skin manifestations were associated with a more severe disease course. METHODS: Severe cutaneous manifestations were defined as presence of hemorrhagic vesicles, bullae, ulcerations and/or necroses. Data were collected retrospectively from 12 international tertiary university medical centers. RESULTS: A total of 64 patients with the most severe skin changes in IgAV/HSP and median (Q1, Q3) age of 8.08 (5.08, 11.92) years at the disease onset were compared with 596 IgAV/HSP patients without these manfiestations and median (Q1, Q3) age of 6.33 (4.50, 8.92) years. The patients with severe cutaneous manifestations were older in comparison to other patients with IgAV/HSP (p<0.001), they developed nephritis more frequently (40.6% vs. 20.6%, p = 0.001) with worse outcome of renal disease (p = 0.001). This group of patients also had higher frequencies of severe gastrointestinal complications like hematochezia, massive bleeding and/or intussusception (29.3% vs. 14.8%, p<0.001). d-dimer concentrations were significantly higher in these patients (4.60 mg/L vs. 2.72 mg/L, p = 0.003) and they had more frequent need for treatment with systemic glucocorticoids (84.4% vs. 37.2%, p<0.001) in comparison with the control group. Further multivariate analysis showed that severe cutaneous changes were associated with higher risk of developing nephritis [OR=3.1 (95%CI 1.04-9.21), p = 0.042] and severe gastrointestinal complications [OR=3.65 (95%CI 1.08-12.37), p = 0.038]. CONCLUSION: Patients with IgAV/HSP and severe skin manifestations had a more severe clinical course and more frequently required glucocorticoids compared to classic IgAV/HSP patients.

Safety and Outcome of Revascularization Treatment in Patients With Acute Ischemic Stroke and COVID-19: The Global COVID-19 Stroke Registry.

BACKGROUND AND OBJECTIVES: COVID-19-related inflammation, endothelial dysfunction, and coagulopathy may increase the bleeding risk and lower the efficacy of revascularization treatments in patients with acute ischemic stroke (AIS). We aimed to evaluate the safety and outcomes of revascularization treatments in patients with AIS and COVID-19. METHODS: This was a retrospective multicenter cohort study of consecutive patients with AIS receiving intravenous thrombolysis (IVT) and/or endovascular treatment (EVT) between March 2020 and June 2021 tested for severe acute respiratory syndrome coronavirus 2 infection. With a doubly robust model combining propensity score weighting and multivariate regression, we studied the association of COVID-19 with intracranial bleeding complications and clinical outcomes. Subgroup analyses were performed according to treatment groups (IVT-only and EVT). RESULTS: Of a total of 15,128 included patients from 105 centers, 853 (5.6%) were diagnosed with COVID-19; of those, 5,848 (38.7%) patients received IVT-only and 9,280 (61.3%) EVT (with or without IVT). Patients with COVID-19 had a higher rate of symptomatic intracerebral hemorrhage (SICH) (adjusted OR 1.53; 95% CI 1.16-2.01), symptomatic subarachnoid hemorrhage (SSAH) (OR 1.80; 95% CI 1.20-2.69), SICH and/or SSAH combined (OR 1.56; 95% CI 1.23-1.99), 24-hour mortality (OR 2.47; 95% CI 1.58-3.86), and 3-month mortality (OR 1.88; 95% CI 1.52-2.33). Patients with COVID-19 also had an unfavorable shift in the distribution of the modified Rankin score at 3 months (OR 1.42; 95% CI 1.26-1.60). DISCUSSION: Patients with AIS and COVID-19 showed higher rates of intracranial bleeding complications and worse clinical outcomes after revascularization treatments than contemporaneous non-COVID-19 patients receiving treatment. Current available data do not allow direct conclusions to be drawn on the effectiveness of revascularization treatments in patients with COVID-19 or to establish different treatment recommendations in this subgroup of patients with ischemic stroke. Our findings can be taken into consideration for treatment decisions, patient monitoring, and establishing prognosis. TRIAL REGISTRATION INFORMATION: The study was registered under ClinicalTrials.gov identifier NCT04895462.

Meniscus Sign in Patients with Anterior Circulation Large Vessel Occlusion Stroke does not Predict Outcome.

PURPOSE: The angiographic appearance of the occlusion site was suggested to influence outcomes of stroke patients with large vessel occlusion (LVO) who undergo endovascular treatment (EVT). We aimed to study the impact of the meniscus sign (MS) on outcomes of stroke patients with anterior circulation LVO. METHODS: Based on two prospective registries of acute ischemic stroke, we selected patients with carotid­T, M1 or M2 occlusion who underwent EVT. Clinical characteristics and outcomes were collected from the registries or from individual records. Two independent observers blinded to outcomes assessed the presence of MS in digital subtraction angiography before thrombectomy. Angiographic and clinical outcomes of patients with and without MS were compared. RESULTS: We included 903 patients, with median age of 78 years, 59.8% were male, median baseline NIHSS was 14 and 39.5% received intravenous thrombolysis. Patients with MS (n = 170, 18.8%) were more frequently female, presented with higher NIHSS scores and more frequently underwent intravenous thrombolysis. Presence of MS was significantly associated with cardioembolic etiology. Successful reperfusion, number of passes, first pass effect, procedural time, symptomatic intracerebral hemorrhage, in-hospital mortality and favorable 3­month functional outcome were similar in the groups of patients with and without MS. In the multivariable analyses, MS was not associated with successful reperfusion (odds ratio, OR = 1.08, 95% confidence interval, CI = 0.76-1.55), first pass effect (OR = 0.96, 95%CI = 0.48-1.92) or favorable 3­month outcome (OR = 1.40, 95%CI = 0.88-2.24). CONCLUSION: The presence of MS in acute ischemic stroke patients with anterior circulation large vessel occlusion who undergo EVT does not appear to influence angiographic or clinical outcomes.

Remote Training of Neurointerventions by Audiovisual Streaming : Experiences from the European ESMINT-EYMINT E-Fellowship Program.

BACKGROUND: Remote access of trainees to training centers via video streaming (tele-observership, e­fellowship) emerges as an alternative to acquire knowledge in endovascular interventions. Situational awareness is a summary term that is also used in surgical procedures for perceiving and understanding the situation and projecting what will happen next. A high situational awareness would serve as prerequisite for meaningful learning success during tele-observerships. We hypothesized that live perception of the angiographical procedures using streaming technology is feasible and sufficient to gain useful situational awareness of the procedure. METHODS: During a European tele-observership organized by the European Society of Minimally Invasive Neurological Therapy (ESMINT) and its trainee association (EYMINT), a total of six neurointerventional fellows in five countries observed live cases performed by experienced neurointerventionalists (mentors) in six different high-volume neurovascular centers across Europe equipped with live-streaming technology (Tegus Medical, Hamburg, Germany). Cases were prospectively evaluated during a 12-month period, followed by a final questionnaire after completion of the course. RESULTS: A total of 102/161 (63%) cases with a 1:1 allocation of fellow and mentor were evaluated during a 12-month period. Most frequent conditions were ischemic stroke (27.5%), followed by embolization of unruptured aneurysms (25.5%) and arteriovenous malformations (AVMs) (15.7%). A high level of situational awareness was reported by fellows in 75.5% of all cases. After finishing the program, the general improvement of neurointerventional knowledge was evaluated to be extensive (1/6 fellows), substantial (3/6), and moderate (2/6). The specific fields of improvement were procedural knowledge (6/6 fellows), technical knowledge (3/6) and complication management (2/6). CONCLUSION: Online streaming technology facilitates location-independent training of complex neurointerventional procedures through high levels of situational awareness and can therefore supplement live hands-on-training. In addition, it leads to a training effect for fellows with a perceived improvement of their neurointerventional knowledge.

Dissecting postharvest chilling injuries in pome and stone fruit through integrated omics.

Lowering the storage temperature is an effective method to extend the postharvest and shelf life of fruits. Nevertheless, this technique often leads to physiological disorders, commonly known as chilling injuries. Apples and pears are susceptible to chilling injuries, among which superficial scald is the most economically relevant. Superficial scald is due to necrotic lesions of the first layers of hypodermis manifested through skin browning. In peaches and nectarines, chilling injuries are characterized by internal symptoms, such as mealiness. Fruits with these aesthetic or compositional/structural defects are not suitable for fresh consumption. Genetic variation is a key factor in determining fruit susceptibility to chilling injuries; however, physiological, or technical aspects such as harvest maturity and storage conditions also play a role. Multi-omics approaches have been used to provide an integrated explanation of chilling injury development. Metabolomics in pome fruits specifically targets the identification of ethylene, phenols, lipids, and oxidation products. Genomics and transcriptomics have revealed interesting connections with metabolomic datasets, pinpointing specific genes linked to cold stress, wax synthesis, farnesene metabolism, and the metabolic pathways of ascorbate and glutathione. When applied to Prunus species, these cutting-edge approaches have uncovered that the development of mealiness symptoms is linked to ethylene signaling, cell wall synthesis, lipid metabolism, cold stress genes, and increased DNA methylation levels. Emphasizing the findings from multi-omics studies, this review reports how the integration of omics datasets can provide new insights into understanding of chilling injury development. This new information is essential for successfully creating more resilient fruit varieties and developing novel postharvest strategies.

Host genetic susceptibility underlying SARS-CoV-2-associated Multisystem Inflammatory Syndrome in Brazilian Children.

BACKGROUND: Multisystem Inflammatory Syndrome in Children (MIS-C) is a life-threatening complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, which manifests as a hyper inflammatory process with multiorgan involvement in predominantly healthy children in the weeks following mild or asymptomatic coronavirus disease 2019 (COVID-19). However, host monogenic predisposing factors to MIS-C remain elusive. METHODS: Herein, we used whole exome sequencing (WES) on 16 MIS-C Brazilian patients to identify single nucleotide/InDels variants as predisposition factors associated with MIS-C. RESULTS: We identified ten very rare variants in eight genes (FREM1, MPO, POLG, C6, C9, ABCA4, ABCC6, and BSCL2) as the most promising candidates to be related to a higher risk of MIS-C development. These variants may propitiate a less effective immune response to infection or trigger the inflammatory response or yet a delayed hyperimmune response to SARS-CoV-2. Protein-Protein Interactions (PPIs) among the products of the mutated genes revealed an integrated network, enriched for immune and inflammatory response mechanisms with some of the direct partners representing gene products previously associated with MIS-C and Kawasaki disease (KD). In addition, the PPIs direct partners are also enriched for COVID-19-related gene sets. HLA alleles prediction from WES data allowed the identification of at least one risk allele in 100% of the MIS-C patients. CONCLUSIONS: This study is the first to explore host MIS-C-associated variants in a Latin American admixed population. Besides expanding the spectrum of MIS-C-associated variants, our findings highlight the relevance of using WES for characterising the genetic interindividual variability associated with COVID-19 complications and ratify the presence of overlapping/convergent mechanisms among MIS-C, KD and COVID-19, crucial for future therapeutic management.

Concomitant ischemic stroke and myocarditis as the initial clinical presentation of Takayasu arteritis.

Tayakasu arteritis (TA) is a chronic large-vessel systemic vasculitis more frequent in women. The pathogenesis of TA is not fully understood. Due to its silent and heterogenous nature, it is a challenging diagnosis, with no specific diagnostic tests. We report the case of a 45-year-old woman presenting with a concomitant ischemic stroke and myocarditis as an atypical presentation of Takayasu arteritis, revealing its heterogeneous nature. The pre-existent myocardial scar and dilated cardiomyopathy are is a reminder of how silent yet aggressive this vasculitis can be and the need of for high level of suspicion to detect and treat Takayasu arteritis early, avoiding late consequences. Corticosteroids (0.5-1.0mg/kg/day) are the mainstay treatment associated with steroid-sparing drugs, such as methotrexate or azathioprine.

Sobre as pestes, o desamparo e o desgoverno. Psicanálise e política, 1920 e 2020 / On plagues, helplessness and misgovernment. Psychoanalysis and politics, 1920 and 2020 / Sur les pestes, l'impuissance et la mauvaise administration. Psychanalyse et politique, 1920 et 2020 / Sobre las pestes, el desamparo y el desgobierno. Psicoanálisis y política, 1920 y 2020

Para compreender melhor a percepção de desgoverno e o sentimento de desamparo que se alastraram no Brasil sob a Covid-19, este artigo examina dois momentos históricos relativos à interface peste-desamparo, das perspectivas psicanalítica e política. O primeiro momento trata do entrelaçamento entre vida e obra de Freud e culmina com sua experiência com a pandemia da gripe espanhola, em 1920; o segundo, trabalha a forma pela qual estamos lidando com o advento da pandemia do coronavírus, desde março de 2020, que — até o momento em que escrevo este artigo (janeiro de 2022) — matou mais de 600 mil brasileiros e trouxe o luto para mais de cinco milhões de famílias no mundo. A hipótese deste trabalho assevera que o sentimento de desamparo que estamos vivenciando desvela o negacionismo e o desgoverno que, inclusive, põem em xeque o estado democrático de direito no país.

Resumos To better understand the widespread perception of misgovernment and helplessness in Brazil during the COVID-19 pandemic, this article examines two historic moments referring to the interface between plague and helplessness, from the psychoanalytic and political perspectives. Firstly, it addresses the intertwining between Freud's life and work, culminating with his experience during the Spanish flu pandemic, in 1920. Secondly, it analyses the measures put in place to combat the coronavirus pandemic, since March 2020, which — until the moment I am writing this article (January 2022) — killed more than 600,000 Brazilians and left more than five million families around the world in mourning. This paper asserts that the feeling of helplessness unveils a state of denialism and misgovernment, which calls into question the Brazilian rule of law.

Pour mieux comprendre la perception répandue de la mauvaise administartion et de l'impuissance au Brésil pendant la pandémie de Covid-19, cet article examine deux moments historiques liés à l'interface peste-impuissance, du point de vue psychanalytique et politique. Premièrement, il traite de l'imbrication entre la vie et l'œuvre de Freud, qui culmine avec son expérience pendant la pandémie de grippe espagnole en 1920. Deuxièmement, il analyse les mesures misen en place pour combattre la pandémie du coronavirus, depuis mars 2020, qui — jusqu'au moment de la rédaction de cet article (janvier 2022) — a fait plus de 600 000 victimes brésiliennes et endeuillé plus de cinq millions de familles dans le monde. Cet essai affirme que le sentiment d'impuissance dévoile un état de négationnisme et de mauvaise gouvernance, qui remet en question l'État de droit et la démocratie au Brésil.

Para comprender mejor la percepción del desgobierno y del sentimiento de desamparo en Brasil durante la crisis del Covid-19, este artículo examina dos momentos históricos relacionados con la interfaz peste-desamparo, desde un punto de vista psicoanalítico y político. El primer momento trata del entrelazamiento entre la vida y la obra de Freud y culmina con su experiencia con la pandemia de gripe española en 1920. El segundo momento se refiere a la forma en que estamos lidiando con el advenimiento de la pandemia de Coronavirus desde marzo de 2020 que, al momento de redactar este artículo (enero de 2022), ha causado más de 600.000 víctimas brasileñas y dejado más de cinco millones de familias en todo el mundo. Este ensayo se basa en el supuesto de que el sentimiento de desamparo que experimentamos se debe a la negación del Covid y al desgobierno. Además, estas dos razones han representado una amenaza para el estado de derecho y la democracia en Brasil.

Valuing tonsillitis manifestations in schoolchildren in Rio de Janeiro.

OBJECTIVE: To evaluate the importance attributed to tonsillitis by guardians and their level of knowledge about the disease, correlating their management with their schooling and socioeconomic profiles. METHODS: A quantitative, descriptive and observational cross-sectional study involving students aged 5 to 17 years from state-owned and private schools. A questionnaire was applied on management of tonsillitis and knowledge about rheumatic fever, addressing demographic and socioeconomic data. RESULTS: A total of 323 students were included, predominantly females (61.3%), from state-owned schools (77.1%), with a mean age of 9.7±0.3 years. Among the guardians, 48.6% completed high school. Among the students 75.2% had at least one episode of odynophagia in the previous 12 months. Considering the previous 2 years, 89.8% reported this symptom. There was no collection of bacteriological specimens in 67.8%, and in 83% if taking into account only the last episode, despite the search for medical care in most cases (92.6%). The use of anti-inflammatory drugs was very frequent (43.0% "always" and 42.4% "sometimes"). Among the guardians, 81.7% denied being aware of the relation between rheumatic fever and inappropriate treatment of odynophagia; 85.8% said they wished they had received more information. CONCLUSION: Although it is quite common in schoolchildren, tonsillitis is neglected by many guardians and health professionals, and the ignorance of its relation with rheumatic fever is practically universal, which may contribute to its high prevalence.