ABSTRACT COVID-19 is commonly associated with high serum levels of pro-inflammatory cytokines, and the post-infection status can disturb self-tolerance and trigger autoimmune responses. We are reporting a 45-year-old male who was admitted with fatigue, jaundice, elevated liver enzymes (with cholestatic pattern), and acute kidney injury two weeks after recovering from a mild SARS-CoV-2 infection. Serologies for viral hepatitis and anti-mitochondrial antibody were negative, while anti-nuclear and anti-smooth muscle antibodies were positive. There were no signs of chronic liver disease, and a magnetic resonance cholangiography showed no dilatation of biliary ducts. Histologic evaluation of the liver evidenced numerous foci of lobular necrosis without ductopenia or portal biliary reaction. Considering the autoantibody profile and histologic changes, the medical team started oral prednisone, but there was a suboptimal biochemical response in the outpatient follow-up. Two months later, a second liver biopsy was performed and revealed non-suppurative destructive chronic cholangitis, extensive areas of confluent necrosis with hepatocytes regenerating into pseudorosettes, and numerous plasma cells. According to the Paris Criteria, the patient was then diagnosed with an autoimmune hepatitis-primary biliary cholangitis overlap syndrome (AIH-PBC-OS). After adding azathioprine and ursodeoxycholic acid to the treatment, there was a satisfactory response. This is the second worldwide report of an AIH-PBC-OS triggered by COVID-19, but the first case with a negative anti-mitochondrial antibody. In this setting, histologic evaluation of the liver by an experienced pathologist is a hallmark of achieving the diagnosis and correctly treat the patient.

Abstract Blast-induced vibration may be harmful to facilities in the vicinity of operating mines, mainly causing structural damage and human discomfort. This study presents an application of multivariate statistics to predict vibration levels regarding their potential to cause structural damage and human discomfort. An extensive seismic monitoring campaign was executed in a large open-pit iron ore mine, near a small village, to gather a dataset for a predictive multivariate analysis. Ten blasting events have produced a dataset of 158 valid measurements. Three classes of vibration peak velocity were adopted from legal standards, which later supported a cluster analysis. Then, it was possible to compare how much these two classification modalities respond to discriminant analysis. The next step was to carry out a principal component analysis (PCA) from the original database, and, comparatively, to plot both the scores concerning the classes derived from the vibration standard and those from the groups obtained from cluster analysis. PCA has considerably explained the data variability, while the three classes from cluster analysis resulted very similar to the corresponding ones from the vibration standards. The results have demonstrated that multivariate statistics may be applied to manage blasting-induced vibration and its deleterious effects with few adjustments and automation.

ABSTRACT Membranoproliferative glomerulonephritis describes a glomerular-injury pattern common to a heterogeneous group of diseases. Evaluation based on clinical and laboratory presentation and immunofluorescence staining on kidney biopsy allows identification of underlying pathophysiological processes and may facilitate proper diagnosis and treatment. Hypocomplementemic urticarial vasculitis syndrome is a rare autoimmune disease of multi-organ involvement. The diagnosis is based on well-defined clinical and laboratory criteria. The pathophysiology is not completely understood but the presence of anti-C1q antibody seems to be involved. Renal involvement occurs in up to 50% of cases. It can be heterogeneous and can be indistinguishable from lupus nephritis. Serological findings and skin involvement distinguish these two entities. We report the case of a 40-year-old female who presented with urticarial skin lesions, hypocomplementemia and nephrotic syndrome. Kidney biopsy showed membranoproliferative glomerulonephritis with full house immune complex deposits. The diagnosis of hypocomplementemic urticarial vasculitis syndrome was made and the patient was successfully treated with prednisolone and mycophenolate mofetil.

Resumen Introducción La aspiración de cuerpo extraño (ACE) es una emergencia pediátrica potencialmente fatal. La intención del presente artículo es resaltar la importancia de un abordaje multidisciplinario en caso de un diagnóstico difícil/dudoso. Reporte de caso Se trata de una paciente de 4 meses de edad remitida para broncoscopia rígida de urgencia, luego de la sospecha de ingestión de una hojilla metálica (se encontró a la bebé mordiéndola). La paciente tenía antecedentes de sibilancia. Al examen físico se encontraron heridas en la cara y los labios, rastros de polvo en los dientes, pero no había dificultad respiratoria. El examen de rayos-x mostraba imágenes radio opacas en el cuadrante superior derecho del pulmón y en la cámara gástrica. En virtud de la ausencia de signos clínicos de ACE en una paciente con antecedentes de hiperresponsividad bronquial, se realizó un estudio radiográfico digital. No hubo imágenes compatibles con cuerpos extraños: los resultados se interpretaron como artefactos y no se realizó la broncoscopia. Conclusiones Un cuidadoso examen pre-anestesia, el alto grado de sospecha y una excelente comunicación multidisciplinaria, permitieron el reconocimiento de hallazgos radiológicos falsos. Se siguió un abordaje conservador, evitando así procedimientos invasivos en lugares remotos con alto grado de riesgo para la población pediátrica.

Abstract Introduction Foreign body aspiration (FBA) is a potentially fatal paediatric emergency. Our objective was to highlight the importance of a multidisciplinary approach to difficult/doubtful diagnosis. Case report 34-month-old girl referred for urgent rigid bronchoscopy after suspected metallic blade ingestion (found chewing on it). She had a previous recurrent history of wheezing. The physical examination revealed face/lip wounds, traces of powder on her teeth but no breathing difficulty. The plain X-Ray revealed radiopaque images of the upper pulmonary field and gastric chamber. In the absence of FBA clinical signs but considering a previous history of bronchial hyperresponsiveness, a direct digital radiographic study was performed. There were no images compatible with foreign bodies: the results were interpreted as artefacts and no bronchoscopy was performed. Conclusions A careful pre-anaesthetic evaluation, a high level of suspicion and excellent multidisciplinary communication led to the recognition of false radiologic findings. A conservative approach was followed and invasive procedures in a remote location, with high anaesthetic risk for the paediatric population were avoided.

ABSTRACT BACKGROUND: Turner syndrome (TS) is a rare genetic disease. Understanding its clinical findings contributes to better management of clinical conditions. OBJECTIVE: To investigate the clinical and karyotypic characteristics of patients diagnosed with TS at two reference services for clinical genetics in southern Brazil. DESIGN AND SETTING: Retrospective cross-sectional study conducted in two clinical genetics services in Porto Alegre (RS), Brazil. METHODS: The sample consisted of 59 patients with TS diagnosed from 1993 to 2019. A review of their medical records was performed and a standard protocol was filled out. RESULTS: The average age of the patients at diagnosis was 15.9 years, and 40.7% were over 13 years old. The largest proportion of them (42.4%) had been referred from an endocrinology department and their constitution was 45,X (40.7%). The most common clinical findings were short stature (85.7%), hypoplastic/ hyperconvex nails (61.2%), low posterior hairline (52.1%) and cubitus valgus (45.8%). There was no difference regarding the presence of short stature (P = 0.5943), number of dysmorphia (P = 0.143), anatomical regions affected and malformations identified through imaging examinations (P = 1.0000), regarding the presence or absence of 45,X constitution. Only 6% of the patients had used growth hormone and 43%, estrogen. CONCLUSION: We found that, in general, patients with TS were being diagnosed late. This has important implications for their treatment. In addition, only a small proportion of the patients were undergoing further examination or evaluation, which appeared to be leading to underdiagnosis of many abnormalities.

OBJECTIVES: Despite higher rates of sustained virologic response (SVR), important concerns remain when patients with decompensated cirrhosis due to hepatitis C virus (HCV) are treated with direct-acting antiviral agents (DAA). Questions include efficacy, safety, and the magnitude of liver function improvement. Here, we aimed to evaluate HCV treatment data in this specific population in Brazil. METHODS: We included 85 patients with decompensated cirrhosis submitted to HCV therapy with DAA followed at two academic tertiary centers in the southeastern region of Brazil. RESULTS: Seventy-nine patients (92.9%) were Child-Pugh (CP) score B, and six (7.1%) were CP score C. The mean MELD score was 12.86. The most common treatment was sofosbuvir plus daclatasvir±ribavirin for 24 weeks. The overall intention-to-treat (ITT) SVR rate was 87.4% (74/85) and modified-ITT 96.1% (74/77). ITT SVR was associated with lower baseline INR values (p=0.029). Adverse events (AE) occurred in 57.9% (44/76) of patients. Serious AE were reported in 12.8% (10/78), and were related to the presence of hepatic encephalopathy (p=0.027). SVR was associated with improvement in CP (p<0.0001) and MELD scores (p=0.021). Among baseline CP score B patients with SVR, 46% (29/63) regressed to CP score A. Ascites was independently associated with no improvement in liver function in patients who achieved SVR (p=0.001; OR:39.285; 95% CI:4.301-258.832). CONCLUSIONS: Patients with decompensated HCV cirrhosis showed a high SVR rate with interferon-free therapy. Early liver function improvement occurred after successful HCV eradication. However, long-term follow-up of these patients after SVR remains strongly advised.

The role of uric acid as an independent risk factor for chronic kidney disease development and progression is still a matter of discussion. Several observational studies showed a positive association between hyperuricemia and the progression of kidney dysfunction, but others did not, which probably derived from different biases and studies insufficiencies. Moreover, the results from studies on patients in hemodialysis and peritoneal dialysis are even more controversial, with some evidence pointing towards a protective role of uric acid in hemodialysis patients, but not in peritoneal dialysis. In addition to most evidence suggesting a role of uric acid in chronic kidney disease pathogenesis and progression, pharmacological treatment of asymptomatic hyperuricemia is still not indicated, with no consensus on either the uric acid level at which treatment would be beneficial, or the target-level to achieve. There are several studies on the renal benefits of xanthine oxidase inhibitors allopurinol and febuxostat, with heterogeneous results. Most of them showed a renoprotective effect of both drugs, delaying renal disease progression. However, the different results found in other studies makes it difficult to draw definitive conclusions. Despite recent evidence pointing toward an important role of uric acid in the pathogenesis and progression of kidney disorders, and the benefits of its treatment, there are still several unanswered questions, and well-conducted studies are needed to make valid conclusions.

Abstract RNA interference (RNAi) is a powerful gene silencing technology, widely used in analyses of reverse genetics, development of therapeutic strategies and generation of biotechnological products. Here we present a free software tool for the rational design of RNAi effectors, named siRNA and shRNA designer (SSD). SSD incorporates our previously developed software Strand Analysis to construct template DNAs amenable for the large scale production of mono-, bi- and trivalent multimeric shRNAs, via in vitro rolling circle transcription. We tested SSD by creating a trivalent multimeric shRNA against the vitellogenin gene of Apis mellifera. RT-qPCR analysis revealed that our molecule promoted a decrease in more than 50% of the target mRNA, in a dose-dependent manner, when compared to the control group. Thus, SSD software allows the easy design of multimeric shRNAs, for single or multiple simultaneous knockdowns, which is especially interesting for studies involving large amounts of double-stranded molecules.

Abstract Introduction: this study aimed to verify the ability of speech therapists to identify, sort and name the different consistencies used in neurogenic oropharyngeal dysphagia (NOD) management, and to compare the results with the terms proposed by the International Dysphagia Diet Standardization Initiative (IDDSI). Methods: this research was approved by the ethics committee. Sixty speech therapists who work with NOD patients sorted 5 commercial foods from thinnest to thickest to match IDDSI levels 0 to 4, and then used a term to designate each consistency. Results: most subjects (76.66%) sorted the foods properly. Terminologies were divergent at all levels. For level 0, practitioners assigned 3 different terms. For level 1, 24 different terms were reported; for level 2 there were 25 terms, 23 terms for level 3, and 18 terms for level 4. Level 0 (IDDSI - thin) was designated by most participants as liquid; level 1 (IDDSI - slightly thick) was referred to as semi-thickened liquid; level 2 (IDDSI - mildly thick) as thickened liquid; level 3 (IDDSI - moderately thick) as honey; and level 4 (IDDSI - extremely thick) as pasty by most subjects. A reduced number of participants used terms in accordance with IDDSI. Level 0 was appropriately named by 5 subjects (8.33%); levels 1, 2 and 4 by 2 practitioners each (3.33%); and level 3 by 1 professional (1.66%). None of the subjects named all 5 IDDSI levels correctly. Conclusion: most practitioners progressed consistencies properly. There was a diversity of terminologies used for the same consistency at all levels, with no standardization.

Resumen Introducción: este estudio tuvo como objetivo verificar la capacidad de los logopedas para identificar, clasificar y nombrar las diferentes consistencias utilizadas en el manejo de la disfagia orofaríngea neurogénica (NOD) y comparar los resultados con los términos propuestos por la Iniciativa Internacional de Estandarización de la Dieta para la Disfagia (IDDSI). Métodos: esta investigación fue aprobada por el comité de ética. Sesenta terapeutas del habla que trabajan con pacientes de NOD clasificaron 5 alimentos comerciales, desde los más finos hasta los más gruesos, coincidiendo con los niveles 0 a 4 de la IDDSI; luego, debían nombrar cada consistencia. Resultados: la mayoría de los sujetos (76,66%) clasificaron correctamente las consistencias. Las terminologías fueron divergentes en todos los niveles. Para el nivel 0, los participantes asignaron 3 términos diferentes. Para el nivel 1 usaron 24 términos diferentes, 25 términos para el nivel 2, 23 para el nivel 3 y 18 para el nivel 4. La consistencia del nivel 0 (IDDSI fina) fue nombrada por la mayoría de los participantes como líquida; la del nivel 1 (IDDSI ligeramente espesa) la denominaron líquida semi-espesada; la del nivel 2 (IDDSI poco espesa) como líquida espesada; la del nivel 3 (IDDSI moderadamente espesa) como miel, y la del nivel 4 (IDDSI extremadamente espesa) como pastosa. Un número reducido de participantes emplearon términos coincidentes con los de la IDDSI. El nivel 0 fue nombrado correctamente por 5 sujetos (8,33%), los niveles 1, 2 y 4 por 2 participantes cada uno (3,33%), y el nivel 3 por 1 profesional (1,66%). Ninguno de los sujetos nombró correctamente los 5 niveles de la IDDSI. Conclusión: la mayoría de los participantes ordenaron correctamente la progresión de las consistencias. Hubo diversidad en las terminologías utilizadas para una misma consistencia en todos los niveles, sin estandarización alguna.

RESUMO Desde a publicação em 2015 das recomendações da Sociedade Brasileira de Hepatologia sobre a prevenção e tratamento de doenças hepáticas autoimunes, novos dados baseados em evidências científicas foram publicados na literatura, mudando o diagnóstico e tratamento da hepatite autoimune (HAI), colangite biliar primária (CBP), colangite esclerosante primária (CEP), síndromes de sobreposição de HAI, CBP e CEP e o manejo de complicações específicas além de outros tópicos relativos à HAI e doenças hepáticas colestáticas. Este manuscrito atualiza as recomendações anteriores de acordo com as melhores evidências disponíveis na literatura até o momento. O mesmo painel de experts que participou da primeira diretriz revisou todas as recomendações de acordo com os dados publicados na literatura e elaborou um manuscrito submetido subsequentemente à apreciação e revisão de todos os membros da Sociedade Brasileira de Hepatologia via homepage da sociedade. As recomendações finais atualizadas foram condensadas no presente documento.

ABSTRACT New data concerning the management of autoimmune liver diseases have emerged since the last single-topic meeting sponsored by the Brazilian Society of Hepatology to draw recommendations about the diagnosis and treatment of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), overlap syndromes of AIH, PBC and PSC and specific complications and topics concerning AIH and cholestatic liver diseases. This manuscript updates those previous recommendations according to the best evidence available in the literature up to now. The same panel of experts that took part in the first consensus document reviewed all recommendations, which were subsequently scrutinized by all members of the Brazilian Society of Hepatology using a web-based approach. The new recommendations are presented herein.

Abstract Aim: In this study, we sought to analyze the influence of the quality of opposition on players’ performance of Club Atlético de Madrid (ATM) 2016/2017. For that, the Golden Index (GI) formula was applied to identify and rank the Golden Players of ATM when playing against opponents of different quality levels. Methods: GI formula variables were collected through notational analysis and three global adjacency matrices were constructed to record all successful ball-passing actions performed. Next, the matrices were imported to SocNetV to collect the network centrality metrics. To uniformize each variable, the statistical standardization technique was applied to all variables. ATM opponents were classified into three groups: high-level (n=4), medium-level (n=2) and low-level (n=2), according to their classification in La Liga and participation in the final stage of UEFA Champions League 2016/2017. Results: Koke was considered the Golden Player playing against high-level teams, while opposing low-level opponents he was ranked as second. Against medium- and low-level teams, Antoine Griezmann was considered the Golden Player, but was not classified in the three first positions against high-level opponents. Yannick Carrasco and Filipe Luís were ranked in the second and third positions, respectively, when playing against high- and medium-level opponents. Also, Saúl Ñíguez obtained the third higher index against low-level teams. Conclusions: This study evidenced that players’ individual performance is influenced by the quality of the opposition. Additionally, the GI formula proved to be a potent tool in analyzing player’s performance in attacking plays in Football.

RESUMO Objetivo Verificar a existência de relação entre pressão máxima da língua e a etiologia da respiração oral em crianças respiradoras orais atendidas em um Ambulatório do Respirador Oral. Método Foi conduzido um estudo transversal observacional descritivo e analítico com 59 crianças respiradoras orais com idades entre três e 12 anos (média de 6,5 anos e DP=2,4). Para a coleta da pressão de língua, foi utilizado o Iowa Oral Performance Instrument – (IOPI) e dados sobre a etiologia da respiração oral e oclusão dentária foram coletados nos prontuários desses pacientes para análise. As associações entre a pressão máxima da língua e a etiologia da respiração oral, idade, gênero e oclusão dentária foram verificadas pelo teste T, ANOVA, coeficiente de Spearman e Teste de Tuckey, utilizando-se nível de significância de 5%. Resultados Houve correlação moderada e positiva entre idade e pressão máxima, verificou-se que houve diferença estatisticamente significativa entre a pressão máxima da língua e as variáveis hipertrofia da tonsila faríngea e hipertrofia das tonsilas palatinas. Não foram verificadas diferenças estatísticas entre as outras variáveis. Conclusão Conclui-se que as obstruções mecânicas, dentre elas a hipertrofia das tonsilas faríngea e palatinas alteram a pressão máxima de língua em crianças respiradoras orais.

ABSTRACT Purpose To verify the relationship between maximum tongue pressure and the etiology of oral breathing in oral breathing children attended at the Oral Respiratory Outpatient Clinic. Methods A descriptive and analytical cross-sectional study was accomplished with 59 mouth breathing children aged 3 to 12 years (mean age 6.5 years and SD: standard deviation= 2.4). To collect tongue pressure, the Iowa Oral Performance Instrument (IOPI) was used and data regarding the etiology of oral breathing and dental occlusion were collected in the records of these patients for analysis. The associations between the maximum tongue pressure and the etiology of oral breathing, age, gender and dental occlusion were verified by the T test, ANOVA, Spearman's coefficient and Tuckey's test, using a significance level of 5%. Results There was a moderate and positive correlation between age and maximum pressure, it was verified that there was a statistically significant difference between the maximum tongue pressure and the variables pharyngeal tonsil hypertrophy and palatine tonsil hypertrophy. There were no statistical differences between the other variables. Conclusion It was concluded that mechanical obstructions, among them the pharyngeal and palatine tonsil hypertrophy alter the maximum tongue pressure in oral breathing children.

RESUMO A lesão renal aguda é uma complicação comum da cirrose, acometendo até 20% dos pacientes hospitalizados. Este tema está em rápida evolução devido aos importantes avanços em novas classificações, biomarcadores e tratamentos que ocorreram nos últimos anos. Em nome da Sociedade Brasileira de Hepatologia, um painel de especialistas em Hepatologia e Nefrologia revisou as evidência publicadas na literatura, integrando os diferentes resultados de estudos, para desenvolver a presente recomendação.

ABSTRACT Acute kidney injury is a common complication of cirrhosis, occurring in up to 20% of patients hospitalized with cirrhosis. This field is rapidly changing, with significant advances in classification, biomarkers and therapy over the last few years. On the behalf of the Brazilian Society of Hepatology, a panel of experts in Hepatology and Nephrology reviewed published evidence to integrate findings and develop the recommendations presented in this manuscript.