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OBJECTIVE: Primary biliary cholangitis is a chronic and progressive autoimmune liver disease, whose prognosis can be improved by normalizing alkaline phosphatase and bilirubin. While ursodeoxycholic acid (UDCA) is first line standard of care, approximately 40 % of patients exhibit incomplete response. We aimed to identify prognostic markers for deep response to UDCA therapy at presentation. PATIENT AND METHODS: Data from the Brazilian Cholestasis Study Group cohort were analyzed retrospectively. Patients were assessed for deep response, defined as normal alkaline phosphatase and bilirubin, after 1 year of UDCA treatment. Additionally, the performance of the UDCA response score in predicting deep response was evaluated. RESULTS: A total of 297 patients were analyzed, with 57.2 % achieving an adequate response according to the Toronto criteria, while 22.9 % reached deep response. Cirrhosis (OR 0.460; 95 % CI 0.225-0.942; p = 0.034) and elevated baseline alkaline phosphatase levels (OR 0.629; 95 % CI 0.513-0.770; p < 0.001) were associated with reduced odds of deep response. The UDCA response score exhibited moderate discrimination power (AUROC = 0.769) but lacked calibration. CONCLUSIONS: Baseline ALP and liver fibrosis emerge as the most important prognostic factors to predict normalization of alkaline phosphatase and bilirubin after UDCA. The UDCA response score was inadequate for predicting deep response in the Brazilian PBC population.

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BACKGROUND: Ursodeoxycholic acid (UDCA) is the standard treatment for primary biliary cholangitis (PBC), but a significant proportion of patients do not respond adequately, leading to increased risk of adverse outcomes. This study aims to develop a new and straightforward predictive score to identify PBC patients likely to achieve a complete response to UDCA. METHODS: A logistic regression analysis was conducted using a derivation cohort of PBC patients to identify pre-treatment variables associated with response to UDCA. This analysis led to the development of the ALP-A score, calculated as: Age at diagnosis divided by (alkaline phosphatase at diagnosis/upper limit of normal). ALP-A score accuracy was evaluated using the area under the ROC curve, validated with a large external cohort from Brazil. Additionally, the correlation between the ALP-A score and the previously validated UDCA response score (URS) was assessed. RESULTS: ALP-A score had good predictive power for adequate (AUC 0.794; 95% CI, 0.737-0.852) and deep (0.76; 95% CI, 0.69-0.83) UDCA response at 1 year of treatment. A cutoff score of 17 and 23 points was determined to be the optimal threshold for distinguishing adequate and deep responders, respectively, from non-responders. ALP-A score demonstrated a sensitivity of 73%, specificity of 71%, positive predictive value of 65%, negative predictive value of 78%, and overall accuracy of 72% for biochemical response. The URS displayed similar discriminative ability (AUC 0.798; 95% CI, 0.741-0.855). CONCLUSION: ALP-A score performs comparably to URS but offers the great advantage of simplicity for routine clinical use. It serves as a valuable tool to identify PBC patients less likely to respond to UDCA treatment, facilitating early consideration of alternative therapeutic approaches.

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INTRODUCTION AND OBJECTIVES: Autoimmune hepatitis (AIH) is a rare disease with a complex and not fully understood pathogenesis. Prognostic factors that might influence treatment response, relapse rates, and transplant-free survival are not well established. This study investigates clinical and biochemical markers associated with response to immunosuppression in patients with AIH. MATERIALS AND METHODS: This retrospective cohort study included 102 patients with AIH treated with immunosuppressants and followed at the Federal University of Minas Gerais, Brazil, from 1990 to 2018. Pretreatment data such as clinical profiles, laboratory, and histological exams were analyzed regarding biochemical response at one year, histological remission, relapse, and death/transplantation rates. RESULTS: Cirrhosis was present in 59 % of cases at diagnosis. One-year biochemical remission was observed in 55.7 % of the patients and was found to be a protective factor for liver transplant. Overall survival was 89 %. Patients with ascites at disease onset showed a higher aspartate aminotransferase (AST)/ alanine aminotransferase (ALT) ratio and elevated Model of end-stage liver disease (MELD) score. The presence of ascites was significantly associated with a 20-fold increase in mortality rate. CONCLUSIONS: AIH has a severe clinical phenotype in Brazilians, with high rates of cirrhosis and low remission rates. Early diagnosis and treatment are essential for achieving remission and reducing complications. The presence of ascites is significantly associated with mortality, emphasizing the importance of monitoring and prompt intervention. This study also stresses the need for further research on AIH in Latin America.

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INTRODUCTION: Women with Polycystic Ovary Syndrome (PCOS) have a higher prevalence of Nonalcoholic Fatty Liver Disease (NAFLD) than the general population. PCOS and NAFLD have common metabolic risk factors, however, the role of diet in NAFLD development is still uncertain in PCOS women. OBJECTIVE: To evaluate and compare the dietary patterns and nutritional intake in patients with PCOS with and without NAFLD. METHOD: Cross-sectional study that included patients with PCOS diagnosed according to Rotterdam criteria. All participants were submitted to abdominal ultrasound to investigate liver steatosis. Dietary profile was assessed by 24-hour food recall (24hR), and Food Frequency Questionnaire (FFQ). Diet quality was assessed by the Healthy Eating Index (HEI) adapted for the Brazilian population. Physical activity practice was also assessed. RESULTS: 87 participants were included (average age 35.2 ± 5.7 years), among whom, 67 (77%) had NAFLD. The group with PCOS and NAFLD presented higher body mass index (BMI) (34.9 ± 4.5 vs. 30.4 ± 4.9 kg/m2; p = 0.001), Waist Circumference (WC) (103 [97‒113] vs. 95 [87.5‒100] cm; p < 0.001) and were considered physically active less frequently than those without NAFLD (34.3% vs. 60%; p = 0.04). Food intake and dietary patterns assessed by 24hR, FFQ and HEI presented no difference between the groups. CONCLUSIONS: PCOS women with coexistent NAFLD had higher BMI, WC and were less physically active than those without NAFLD. Dietary evaluation showed that PCOS women with NAFLD had no significant difference in macro and micronutrients or food group intake and diet quality in comparison to those without NAFLD.

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Corticosteroid-induced myopathy is the most common drug-induced myopathy and could appear during the treatment of diseases where corticosteroids are the mainstay of treatment. We present a clinical case of a patient treated with corticosteroids who presented with proximal muscle weakness, myalgias, marked elevation of muscle enzymes, and acute kidney injury due to rhabdomyolysis. The definitive diagnosis was only possible through a muscle biopsy.

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Increasing epidemiological evidence suggests a bidirectional relationship between non-alcoholic fatty liver disease (NAFLD) and type 2 diabetes, and that NAFLD may precede and/or promote the development of diabetes. This study aimed to investigate whether liver steatosis is associated with the incidence of diabetes in the Brazilian Longitudinal Study of Adult Health (ELSA-Brasil). The ELSA-Brasil is an occupational cohort study of active or retired civil servants, aged 35-74 years, in six capital cities in Brazil. We excluded participants with diabetes at baseline, those who reported excessive alcohol consumption or with missing information on relevant covariates, and those with self-referred hepatitis or cirrhosis. In total, 8,166 individuals participated, and the mean duration of follow-up was 3.8 years. The Cox proportional regression model was used to estimate the adjusted hazard ratio (HR) for the associations. Abdominal ultrasonography was used to detect liver steatosis. In the follow-up period, the cumulative incidence of diabetes was 5.25% in the whole sample, 7.83% and 3.88% in the groups with and without hepatic steatosis, respectively (p < 0.001). Compared to those without steatosis, individuals with hepatic steatosis had an increased risk of developing diabetes (HR = 1.31; 95%CI: 1.09-1.56) after adjustment for potential confounders, including body mass index (BMI). Hepatic steatosis was an independent predictor of incident diabetes in the ELSA-Brasil cohort study. Physicians should encourage changes in lifestyle and screen for diabetes in patients with fatty liver.

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INTRODUCTION: Non-alcoholic fatty liver disease (NAFLD) is related to cardiovascular disease. Cardiorespiratory fitness (CRF) is an important indicator of cardiovascular health. Therefore, we aimed to evaluate the CRF of NAFLD patients. METHODS: Cross-sectional study, including 32 patients with biopsy-proved NAFLD. The patients underwent ergometric test (ET) and six-minute walk test (6MWT) to determine CRF. The test results were compared to disease parameters and with each other. RESULTS: Considering the ET, 20 (62.5%) patients had very poor or poor CRF, and in 12 (37.5%), it was regular or good. In the 6MWT, 13 (40.6%) individuals had poor CRF, in 12 (37.5%), it was very poor, and in seven (21.9%), regular. NAFLD activity score (NAS) ≥5 was observed in 12 (37.5%) individuals. Twelve (37.5%) patients were sedentary, 11 (34.4%), insufficiently active, and nine (28.1%), active. Obesity and liver inflammation on biopsy were associated with very poor/poor CRF. NAS ≥5 and sedentary lifestyle were independently associated with very poor/poor CRF by ET. Although mean VO2max values determined by both tests were similar, no correlation of VO2max determined by ET and 6MWT was observed, as occurred for the distance walked in 6MWT and values of metabolic equivalent (MET) determined by ET. There was no reproducibility between CRF determined by ET and 6MWT. CONCLUSION: Most NAFLD patients had very poor or poor CRF. Severe liver injury (NAS ≥5) and sedentary lifestyle were independently associated with very poor/poor fitness, according to ET. No reproducibility was observed between the CRF defined by ET and 6MWT.

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INTRODUCTION AND OBJECTIVES: Primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) and PBC overlap syndrome (AIH/PBC) have been associated with a higher risk of hepatocellular carcinoma (HCC) and extra-hepatic malignancy (EHM). This study aims to assess potential risk factors associated with cancer development in PBC and AIH/PBC. MATERIALS AND METHODS: The Brazilian Cholestasis Study Group database was reviewed to compare clinical and laboratory features of PBC patients with HCC and EHM with those without cancer. RESULTS: Among the 752 PBC patients enrolled, 64 of them with AIH/PBC, 87 cancers were identified in 72 patients, including 20 cases of HCC and 67 of EHM. Patients with HCC had a higher prevalence of cirrhosis (95% vs. 32.5% of those subjects without cancer, p≤0.001), smoking (55% vs. 12.3%, p≤0.001), CREST syndrome (30% vs 7.6%, p=0.003) and prior azathioprine (30% vs 8%, p= 0.005) and prednisone (35% vs 14%, p= 0.018) use, whereas patients with EHM had a higher prevalence of smoking (42.3% vs 12.4% of those subjects without cancer, p= <0.001), AMA positivity (96.6% vs 80.1%, p≤0.001), azathioprine therapy (21% vs 7.9%, p= 0.01) and concurrent other autoimmune diseases. In multivariate analysis, cirrhosis, obesity and prior azathioprine therapy were independent risk factors for HCC, while Sjogren syndrome and psoriasis were associated with EHM. Fibrates reduced EHM risk. CONCLUSIONS: The prevalence of EHM is higher when compared to HCC in PBC patients. Cirrhosis, obesity, prior azathioprine use, and concurrent autoimmune diseases were significantly associated with cancer in PBC.

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BACKGROUND: Response to ursodeoxycholic acid (UDCA) in primary biliary cholangitis (PBC) has been traditionally assessed 1 to 2 years after treatment initiation. With the development of new drugs, some patients may benefit from an earlier introduction of second-line therapies. AIMS: This study aims to identify whether well-validated response criteria could correctly identify individuals likely to benefit from add-on second-line therapy at 6 months. METHODS: Analysis of a multicenter retrospective cohort which included only patients with clear-cut PBC. RESULTS: 206 patients with PBC (96.6% women; mean age 54 ± 12 years) were included. Kappa concordance was substantial for Toronto (0.67), Rotterdam (0.65), Paris 1 (0.63) and 2 (0.63) criteria at 6 and 12 months, whereas Barcelona (0.47) and POISE trial (0.59) criteria exhibited moderate agreement. Non-response rates to UDCA was not statistically different when assessed either at 6 or 12 months using Toronto, Rotterdam or Paris 2 criteria. Those differences were even smaller or absent in those subjects with advanced PBC. Mean baseline alkaline phosphatase was 2.73 ± 1.95 times the upper limit of normal (× ULN) among responders versus 5.05 ± 3.08 × ULN in non-responders (p < 0.001). CONCLUSIONS: After 6 months of treatment with UDCA, the absence of response by different criteria could properly identify patients who could benefit from early addition of second-line therapies, especially in patients with advanced disease or high baseline liver enzymes levels.

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ABSTRACT BACKGROUND: Occult hepatitis B virus infection (OBI) is defined as the presence of hepatitis B virus (HBV) deoxyribonucleic acid (DNA) in the liver of individuals with undetectable hepatitis B virus surface antigen (HBsAg) in the serum. The actual prevalence of OBI and its clinical relevance are not yet fully understood. OBJECTIVE: To evaluate the prevalence of HBV DNA in liver biopsies of HBsAg-negative patients with chronic liver disease of different etiologies in a referral center in Brazil and compare two different HBV DNA amplification protocols to detect HBV. DESIGN AND SETTING: This cross-sectional observational study was conducted at the Liver Outpatient Clinic, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil, between January 2016 and December 2019. METHODS: HBV DNA was investigated in 104 liver biopsy samples from individuals with chronic liver disease of different etiologies, in whom HBsAg was undetectable in serum by nested-polymerase chain reaction (nested-PCR), using two different protocols. RESULTS: OBI, diagnosed by detecting HBV DNA using both protocols, was detected in 6.7% of the 104 individuals investigated. Both protocols showed a good reliability. CONCLUSION: In addition to the differences in the prevalence of HBV infection in different regions, variations in the polymerase chain reaction technique used for HBV DNA amplification may be responsible for the large variations in the prevalence of OBI identified in different studies. There is a need for better standardization of the diagnostic methods used to diagnose this entity.

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Abstract Introduction Women with Polycystic Ovary Syndrome (PCOS) have a higher prevalence of Nonalcoholic Fatty Liver Disease (NAFLD) than the general population. PCOS and NAFLD have common metabolic risk factors, however, the role of diet in NAFLD development is still uncertain in PCOS women. Objective To evaluate and compare the dietary patterns and nutritional intake in patients with PCOS with and without NAFLD. Method Cross-sectional study that included patients with PCOS diagnosed according to Rotterdam criteria. All participants were submitted to abdominal ultrasound to investigate liver steatosis. Dietary profile was assessed by 24-hour food recall (24hR), and Food Frequency Questionnaire (FFQ). Diet quality was assessed by the Healthy Eating Index (HEI) adapted for the Brazilian population. Physical activity practice was also assessed. Results 87 participants were included (average age 35.2 ± 5.7 years), among whom, 67 (77%) had NAFLD. The group with PCOS and NAFLD presented higher body mass index (BMI) (34.9 ± 4.5 vs. 30.4 ± 4.9 kg/m2; p = 0.001), Waist Circumference (WC) (103 [97‒113] vs. 95 [87.5‒100] cm; p < 0.001) and were considered physically active less frequently than those without NAFLD (34.3% vs. 60%; p = 0.04). Food intake and dietary patterns assessed by 24hR, FFQ and HEI presented no difference between the groups. Conclusions PCOS women with coexistent NAFLD had higher BMI, WC and were less physically active than those without NAFLD. Dietary evaluation showed that PCOS women with NAFLD had no significant difference in macro and micronutrients or food group intake and diet quality in comparison to those without NAFLD.

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Increasing epidemiological evidence suggests a bidirectional relationship between non-alcoholic fatty liver disease (NAFLD) and type 2 diabetes, and that NAFLD may precede and/or promote the development of diabetes. This study aimed to investigate whether liver steatosis is associated with the incidence of diabetes in the Brazilian Longitudinal Study of Adult Health (ELSA-Brasil). The ELSA-Brasil is an occupational cohort study of active or retired civil servants, aged 35-74 years, in six capital cities in Brazil. We excluded participants with diabetes at baseline, those who reported excessive alcohol consumption or with missing information on relevant covariates, and those with self-referred hepatitis or cirrhosis. In total, 8,166 individuals participated, and the mean duration of follow-up was 3.8 years. The Cox proportional regression model was used to estimate the adjusted hazard ratio (HR) for the associations. Abdominal ultrasonography was used to detect liver steatosis. In the follow-up period, the cumulative incidence of diabetes was 5.25% in the whole sample, 7.83% and 3.88% in the groups with and without hepatic steatosis, respectively (p < 0.001). Compared to those without steatosis, individuals with hepatic steatosis had an increased risk of developing diabetes (HR = 1.31; 95%CI: 1.09-1.56) after adjustment for potential confounders, including body mass index (BMI). Hepatic steatosis was an independent predictor of incident diabetes in the ELSA-Brasil cohort study. Physicians should encourage changes in lifestyle and screen for diabetes in patients with fatty liver.

Evidências epidemiológicas crescentes sugerem uma relação bidirecional entre a doença hepática gordurosa não alcoólica (DHGNA) e o diabetes tipo 2 e que a DHGNA pode preceder e/ou promover o desenvolvimento de diabetes. O objetivo deste estudo foi investigar se a esteatose hepática está associada à incidência de diabetes no Estudo Longitudinal de Saúde do Adulto (ELSA-Brasil). O ELSA-Brasil é um estudo de coorte ocupacional com servidores públicos ativos ou aposentados, com idades entre 35 e 74 anos, de seis capitais do Brasil. Foram excluídos os participantes com diabetes no início do estudo, aqueles que relataram consumo excessivo de álcool ou com falta de informações sobre covariáveis relevantes e indivíduos com hepatite ou cirrose autorreferida. No total, 8.166 indivíduos participaram e o tempo médio de seguimento foi de 3,8 anos. O modelo de regressão proporcional de Cox foi utilizado para estimar a razão de risco (HR) ajustada para as associações. A ultrassonografia abdominal foi utilizada para detectar esteatose hepática. No período de seguimento, a incidência cumulativa de diabetes foi de 5,25% em todo o grupo de participantes e de 7,83% e 3,88% nos grupos com e sem esteatose hepática, respectivamente (p < 0,001). Em comparação com aqueles sem esteatose, os indivíduos com esteatose hepática apresentaram um risco elevado de desenvolver diabetes (HR = 1,31; IC95%: 1,09-1,56) após o ajuste para potenciais fatores de confusão, incluindo o índice de massa corporal (IMC). A esteatose hepática foi um preditor independente de diabetes incidente no ELSA-Brasil. Os médicos devem incentivar mudanças no estilo de vida e a triagem para diabetes para pacientes com fígado gorduroso.

La creciente evidencia epidemiológica sugiere una relación bidireccional entre la enfermedad del hígado graso no alcohólica (EHGNA) y la diabetes tipo 2 y que la EHGNA puede preceder y/o desarrollar la diabetes. El objetivo de este estudio fue investigar si la esteatosis hepática está asociada con la incidencia de diabetes en el Estudio Longitudinal de Salud del Adulto (ELSA-Brasil). ELSA-Brasil es un estudio de cohorte ocupacional, realizado con funcionarios públicos activos o jubilados, con edades entre 35 y 74 años, de seis capitales en Brasil. Se excluyeron a los participantes con diabetes al inicio del estudio, aquellos que informaron consumir excesivamente alcohol o que carecían de información sobre las covariables relevantes, y los individuos con hepatitis o cirrosis autorreportada. En total participaron 8.166 sujetos, y el tiempo medio de seguimiento fue de 3,8 años. Se utilizó el modelo de regresión proporcional de Cox para estimar la razón de riesgo ajustada (HR) en las asociaciones. Se realizó ecografía abdominal para detectar esteatosis hepática. En el periodo de seguimiento, el grupo de participantes tuvo incidencia acumulada de diabetes del 5,25%, y en los grupos con y sin esteatosis hepática fueron del 7,83% y el 3,88%, respectivamente (p < 0,001). Los individuos con enfermedad de hígado graso tuvieron mayor riesgo de desarrollar diabetes (HR = 1,31; IC95%: 1,09-1,56) después de ajustar los posibles factores de confusión, incluido el índice de masa corporal (IMC), en comparación con aquellos sin esteatosis. La esteatosis hepática fue un predictor independiente de diabetes incidente en ELSA-Brasil. Los médicos deben alentar cambios en el estilo de vida y la detección de diabetes a los pacientes con hígado graso.

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Objetivo: Avaliar as dimensões de estrutura, processo e resultado do acolhimento com classificação de risco realizado nas Unidades de Pronto Atendimento. Método: Trata-se de pesquisa exploratória, com abordagem quantitativa, coletados via formulário eletrônico, dividido em duas partes: caracterização do profissional e aplicação do "Instrumento para Avaliação do Acolhimento com Classificação de Risco". Resultados: Com base na avaliação por pontuação média em cada dimensão envolvendo os serviços, apenas um deles, alcançou a pontuação de titulação satisfatória na dimensão Processo, com 26,83 e 26,52 para dimensão Resultado. Já no ranking médio total, todas as dimensões receberam titulação precária, de acordo com o escore aplicado. Conclusões: Embora os resultados tenham apontado a dimensão Processo e Resultado como satisfatórios para um dos serviços, ainda não é capaz de sugerir qualidade, o que requer melhorias em todas as dimensões, com destaque para treinamentos periódicos aos enfermeiros que desempenham o acolhimento com classificação de risco (AU).

Objective: Evaluate the dimensions of structure, process and result of the reception with risk classification carried out in the Emergency Care Units. Method: This is exploratory research, with a quantitative approach, collected via electronic form, by: characterization of the professional and application of the "Instrument for Assessment of Welcoming with Risk Classification". Results: Based on the average score evaluation in each dimension involving the services, only one of them achieved a satisfactory degree score in the Process dimension, with 26.83 and 26.52 for the Outcome dimension. In the total average ranking, all dimensions received a precarious degree, according to the applied score. Conclusions: Although the results indicated the Process and Result dimension as satisfactory for one of the services, it is still not able to suggest quality, which requires improvements in all dimensions, with emphasis on periodic training for nurses who perform the reception with a classification of risk (AU).

Objetivo: Evaluar las dimensiones de la estructura, proceso y resultado de la recepción con clasificación de riesgo realizada en las Unidades de Atención de Urgencias. Método: se trata de una investigación exploratoria, con enfoque cuantitativo, compilada electrónicamente, mediante: caracterización del profesional y aplicación del "Instrumento de Evaluación de Bienvenida con Clasificación de Riesgo". Resultados: Con base en la evaluación del puntaje promedio de las dimensiones de los servicios, solo uno de ellos obtuvo puntajes satisfactorios en la dimensión Procesos, con 26.83 y 26.52 en la dimensión Resultados. En la clasificación general media, todas las dimensiones recibieron una puntuación precaria. Conclusiones: Si bien los resultados indicaron la dimensión Proceso y Resultado como satisfactoria para uno de los servicios, aún no es capaz de sugerir calidad, lo que requiere mejoras en todas las dimensiones, con énfasis en la formación periódica de los enfermeros que realizan la recepción con calificación de riesgo (AU).

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BACKGROUND: Occult hepatitis B virus infection (OBI) is defined as the presence of hepatitis B virus (HBV) deoxyribonucleic acid (DNA) in the liver of individuals with undetectable hepatitis B virus surface antigen (HBsAg) in the serum. The actual prevalence of OBI and its clinical relevance are not yet fully understood. OBJECTIVE: To evaluate the prevalence of HBV DNA in liver biopsies of HBsAg-negative patients with chronic liver disease of different etiologies in a referral center in Brazil and compare two different HBV DNA amplification protocols to detect HBV. DESIGN AND SETTING: This cross-sectional observational study was conducted at the Liver Outpatient Clinic, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil, between January 2016 and December 2019. METHODS: HBV DNA was investigated in 104 liver biopsy samples from individuals with chronic liver disease of different etiologies, in whom HBsAg was undetectable in serum by nested-polymerase chain reaction (nested-PCR), using two different protocols. RESULTS: OBI, diagnosed by detecting HBV DNA using both protocols, was detected in 6.7% of the 104 individuals investigated. Both protocols showed a good reliability. CONCLUSION: In addition to the differences in the prevalence of HBV infection in different regions, variations in the polymerase chain reaction technique used for HBV DNA amplification may be responsible for the large variations in the prevalence of OBI identified in different studies. There is a need for better standardization of the diagnostic methods used to diagnose this entity.

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BACKGROUND: Sarcopenia is a common complication of cirrhosis and an important predictor of morbimortality. We aimed to determine the prevalence of sarcopenia and its associated factors in hepatosplenic schistosomiasis (HSS) as well as to evaluate whether muscle mass and function are associated with variceal upper gastrointestinal bleeding (VUGIB) and previous splenectomy in subjects without other liver diseases. METHODS: We conducted a cross-sectional study including adults with HSS who underwent clinical, biochemical, anthropometric, muscle strength and physical performance evaluations and were submitted to bioelectrical impedance analysis and abdominal ultrasound. Sarcopenia was diagnosed according to the 2019 European consensus criteria. RESULTS: A total of 66 patients with HSS (62.1% male; mean age 48.8±8.6 y) were included. Overall, six subjects (9.1%) were diagnosed with probable sarcopenia and none had confirmed sarcopenia. Fat-free body mass index (BMI) was independently associated with VUGIB (odds ratio 0.701 [95% confidence interval 0.51 to 0.96]; p=0.025). Compared with patients who did not undergo surgery, individuals who underwent esophagogastric devascularization combined with splenectomy (EGDS) had higher serum lipid levels, fat percentage and frequency of metabolic syndrome, with lower skeletal muscle mass index and hand grip strength. CONCLUSIONS: HSS mansoni seems not to cause sarcopenia. However, a lower fat-free BMI was associated with previous VUGIB and the subgroup of patients who underwent EGDS presented higher lipid levels, fat percentage and frequency of metabolic syndrome and lower muscle mass and function.

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BACKGROUND: Deposition of Schistosoma mansoni eggs in the brain of patients with hepatosplenic schistosomiasis (HS-SM) is frequent and usually asymptomatic. However, it is questioned whether it could cause seizures. Thus, we investigated the occurrence of seizures in these patients and also searched for parameters associated with this disorder. METHODS: In a cross-sectional survey, we compared 128 patients with HS-SM with 102 patients with portal hypertension due to compensated chronic hepatic disease of other etiologies. A standardized questionnaire, emphasizing epilepsy-related parameters, was applied to all participants. RESULTS: Eight (6.3%) patients with HS-SM had a history of seizures, whereas this condition was reported by three (2.9%) individuals from the comparison group (p=0.354). None of the variables were associated with the occurrence of seizures, either in univariate or in multivariate analysis. CONCLUSIONS: The frequency of seizures was similar in both study groups. However, it was higher than that described in population-based studies. Thus, we hypothesize that HS-SM individuals may have a higher frequency of seizure. The lack of difference between the two study groups may be explained by the inclusion of cases of HS-MS overlapping other chronic liver diseases in the comparison group, because this finding is relatively common in schistosome-endemic areas.

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BACKGROUND: Upper variceal bleeding (UVB) is a possible complication of portal hypertension secondary to hepatosplenic schistosomiasis (HSS). Propranolol is a non-selective beta-blocker used as secondary prophylaxis for UVB, but no previous studies have addressed carvedilol effects in rebleeding prevention. METHODS: A retrospective exploratory study of 57 patients with chronic HSS and index UVB treated with endoscopic variceal ligation and propranolol or carvedilol was conducted. The primary outcome was UVB-free time in the first 12 mo after the initial bleeding episode. RESULTS: Propranolol was used for secondary UVB prophylaxis in 43 (75.4%) participants (median dose 80 [interquartile range - IQR 60-80] mg/d) and carvedilol in 14 (24.6%) participants (median dose 12.5 [IQR 7.9-25.0] mg/d). During a 12-mo follow-up, rebleeding was observed in 13 (22.8%) patients, 9 (20.9%) of those treated with propranolol and 4 (28.6%) treated with carvedilol (p=0.715). Mean time from the beginning of drug prophylaxis to rebleeding was 6±3 mo and there was no difference between that for propranolol vs carvedilol subgroups. Portal vein thrombosis did not influence the bleeding recurrence in either subgroup. CONCLUSION: Carvedilol may be equally effective as propranolol in preventing secondary UVB in HSS at 12-mo follow-up.

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Since the first description of the new Coronavirus disease 19 (COVID 19), the number of associated manifestations described in literature haveincrease exponentially. The spread of virus to extrapulmonary tissues, especially to central and peripheral nervous system, concerns the physiciansand suspicious of disease even in the absence of respiratory symptoms had a major impact preventing it’s spread. The authors report two cases ofBell’s palsy in patients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), describing the clinical manifestations, evolutionand treatments. Both patients had a mild course of COVID 19, without respiratory symptoms reported and complete resolution of facial palsy. Theauthors postulate an association between isolated facial palsy and SARS-CoV-2 infection reviewing the reported cases in literature and the mechanism of neuroinvasion. The authors highlights the importance of suspecting SARS-CoV-2 infection when a patient presents with isolated facial palsy.(AU)

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OBJECTIVES: To evaluate and compare pregnancy outcomes in women with Wilson's disease (WD) undergoing different therapies during pregnancy. MATERIAL AND METHODS: Retrospective review of medication in WD patients during pregnancy and the outcomes. RESULTS: Of 26 pregnancies, zinc was used in 14 (53.8%), D-penicillamine in 4 (15.4%) patients, and 8 (30.8%) were untreated. Spontaneous abortion was observed in 8 (30.8%) pregnancies - untreated patients (4/8 pregnancies), zinc (2/14 pregnancies) and D-penicillamine (2/4 pregnancies) -, healthy outcome in 12 (46.1%) and birth defects in 6 (23.1%). All cases of birth defects occurred in patients using zinc therapy (6/14 pregnancies). CONCLUSIONS: A remarkably high frequency of fetal complications shed lights on the potentially harmful effect of WD drugs during childbearing age. Zinc's safety profile may have to be better evaluated during pregnancy, as all of birth defects occurred with zinc therapy.

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INTRODUCTION AND OBJECTIVES: Little is known about primary biliary cholangitis (PBC) in non-whites. The purpose of this study was to evaluate clinical features and outcomes of PBC in a highly admixed population. MATERIAL AND METHODS: The Brazilian Cholestasis Study Group multicentre database was reviewed to assess demographics, clinical features and treatment outcomes of Brazilian patients with PBC. RESULTS: 562 patients (95% females, mean age 51 ± 11 years) with PBC were included. Concurrent autoimmune diseases and overlap with autoimmune hepatitis (AIH) occurred, respectively, in 18.9% and 14%. After a mean follow-up was 6.2 ± 5.3 years, 32% had cirrhosis, 7% underwent liver transplantation and 3% died of liver-related causes. 96% were treated with ursodeoxycholic acid (UDCA) and 12% required add-on therapy with fibrates, either bezafibrate, fenofibrate or ciprofibrate. Response to UDCA and to UDCA/fibrates therapy varied from 39%-67% and 42-61%, respectively, according to different validated criteria. Advanced histological stages and non-adherence to treatment were associated with primary non-response to UDCA, while lower baseline alkaline phosphatase (ALP) and aspartate aminotransferase (AST) levels correlated with better responses to both UDCA and UDCA/fibrates. CONCLUSIONS: Clinical features of PBC in highly admixed Brazilians were similar to those reported in Caucasians and Asians, but with inferior rates of overlap syndrome with AIH. Response to UDCA was lower than expected and inversely associated with histological stage and baseline AST and ALP levels. Most of patients benefited from add-on fibrates, including ciprofibrate. A huge heterogeneity in response to UDCA therapy according to available international criteria was observed and reinforces the need of global standardization.

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