Abstract Introduction: Autoimmune hepatitis (AIH) has a spectrum of symptoms ranging from asymptomatic disease to acute severe hepatitis, chronic hepatitis, and decompensated cirrhosis. The acute presentation is not rare and could represent genuine acute AIH (GAAIH) or acute exacerbation of chronic autoimmune hepatitis. We aimed to identify the prevalence, clinical features, and prognostic factors associated with GAAIH and compare these cases with acute exacerbation of chronic AIH. Methods: This cross-sectional observational study evaluated patients with acute AIH presentation, defined as total bilirubin >5 times the upper limit of normality (xULN) and/or alanine aminotransferase >10 xULN, and no prior history of liver disease. Histology findings of acute disease defined GAAIH. Bivariate analyses were performed to identify factors associated with the GAAIH, when compared with acute exacerbation of chronic AIH. Results: Seventy-two patients with acute presentation of AIH were included and six (8.3%) of them presented GAAIH. Comparative analysis between patients with GAAIH and patients with acute exacerbation of chronic AIH revealed that prothrombin activity (96% [74-100] vs. 61% [10-100]; p = 0.003) and albumin levels (3.9 ± 0.2 g/dL vs. 3.4 ± 0.5 g/dL; p < 0.001) were higher in patients with GAAIH. The International Autoimmune Hepatitis Group score was higher in patients with acute exacerbation of chronic AIH (18.5 [8-23] vs. 16.5 [15-17]; p = 0.010). Compared to 15.2% of acute exacerbation of chronic AIH, complete therapeutic response to treatment was achieved in 67.7% of cases with GAAIH (p = 0.018). Conclusions: GAAIH was rare (8.3%), and patients with this presentation exhibited more preserved liver function tests, suggesting that most cases presenting with loss of function are acute exacerbation of chronic AIH. Additionally, patients with GAAIH had a better complete therapeutic response, suggesting a more preserved liver function at presentation, and early diagnosis has a positive therapeutic implication.

Resumo Introdução: A hepatite autoimune (HAI) apresenta um espectro de sintomas que varia de doença assintomática a hepatite aguda grave, hepatite crónica e cirrose descompensada. A apresentação aguda não é rara e pode representar hepatite autoimune aguda genuína (HAIAG) ou exacerbação aguda de hepatite autoimune crónica (EAHAIC). O nosso objetivo foi identificar a prevalência, caraterísticas clínicas e fatores prognósticos associados à HAIAG, e comparar esses casos com EAHAIC. Métodos: Estudo observacional, transversal, incluindo doentes com apresentação aguda de HAI, definida como bilirrubina total > 5 vezes o limite superior da normalidade (xLSN) e/ou ALT > 10 xLSN, e sem história prévia de doença hepática. HAIAG foi definida pela presença de achados histológicos de doença aguda. Análises bivariadas foram realizadas para identificar fatores associados à HAIAG, quando comparado com o EAHAIC. Resultados: Foram incluídos setenta e dois doentes com apresentação agu-da de HAI, dos quais seis (8.3%) com HAIAG. A análise comparativa entre doentes com HAIAG e doentes com EAHAIC mostrou que a atividade de protrombina (96%(74-100) versus 61% (10-100; p=0.003) e os níveis de albumina (3,9 ± 0,2 g/dL vs. 3,4 ± 0,5 g/dL; p < 0,001) foram significativamente mais elevados em pacientes com HAIAG. O score do Grupo Internacional de Hepatite Autoimune foi mais elevado em doentes com EAHAIC (18.5 (8-23) versus 16.5 (15-17); p=0.010). A resposta terapêutica completa ao tratamento foi alcançada em 66.7% dos casos de HAIAG (vs. 15,2% na EAHAIC, p=0,018). Conclusões: A HAIAG é rara (8.3%), e os doentes com esta apresentação mostraram testes de função hepática mais preservados, sugerindo que a maioria dos casos com perda de função são EAHAIC. Além disso, os doentes com HAIAG tiveram maior taxa de resposta terapêutica completa, sugerindo que uma função hepática mais preservada na apresentação e o diagnóstico precoce tem uma implicação terapêutica positiva.

Abstract Objective: To determine the frequency and interobserver reproducibility of the magnetic resonance imaging (MRI) features considered diagnostic for autoimmune hepatitis. Materials and Methods: Two abdominal radiologists, blinded to pathology data, reviewed the MRI examinations of 20 patients with autoimmune hepatitis, looking for liver enhancement, lymphadenopathy, portal hypertension, and chronic liver disease. The pattern of liver fibrosis was categorized as reticular, confluent, or mixed. Interobserver agreement was assessed by calculating intraclass correlation coefficients and kappa statistics. Results: The most common abnormal finding on MRI was surface nodularity (in 85%), followed by liver fibrosis with a reticular pattern (in 80%)—categorized as mild (in 25.0%), moderate (in 43.8%), or severe (in 31.2%)—; heterogeneous liver enhancement (in 65%); splenomegaly (in 60%); caudate lobe enlargement (in 50%); and lymphadenopathy (in 40%). The interobserver agreement was almost perfect for surface nodularity (0.83), ascites (0.89), and liver volume (0.95), whereas it was just slight and fair for the degree of fibrosis and for heterogeneous liver enhancement (0.12 and 0.25, respectively). It was also slight and fair for expanded gallbladder fossa and enlarged preportal space (0.14 and 0.36, respectively), both of which are indicative of chronic liver disease. Conclusion: The interobserver agreement was satisfactory for surface nodularity (the most prevalent abnormal MRI finding), ascites, liver volume, and splenomegaly. Conversely, it was only slight or fair for common but less objective criteria. Objective (MRI hepatitis Methods radiologists data 2 hypertension disease confluent mixed statistics Results in 85%, 85 85% , 85%) 80%categorized 80categorized 80% 80 25.0%, 250 25.0% 25 0 25.0%) 43.8%, 438 43.8% 43 8 43.8%) 31.2% 312 31 31.2%)— 65% 65 65%) 60% 60 60%) 50% 50 50%) 40%. 40 40% . 40%) 0.83, 083 0.83 83 (0.83) 0.89, 089 0.89 89 (0.89) 0.95, 095 0.95 95 (0.95) 0.12 012 12 (0.1 025 0.25 respectively. respectively respectively) 0.14 014 14 036 36 0.36 respectively, Conclusion finding, finding) Conversely criteria 25.0 43.8 4 31.2 3 31.2%) 6 5 08 0.8 (0.83 (0.89 09 0.9 9 (0.95 0.1 01 1 (0. 02 0.2 03 0.3 25. 43. 31. 0. (0.8 (0.9 (0 (

Resumo Objetivo: Determinar a frequência e reprodutibilidade interobservador das características de imagem por ressonância magnética na hepatite autoimune. Materiais e Métodos: Dois radiologistas abdominais, cegos para dados patológicos, revisaram ressonâncias magnéticas de 20 pacientes com hepatite autoimune quanto ao realce hepático, linfadenopatia, hipertensão portal e doença hepática crônica. A fibrose foi classificada como reticular, confluente ou ambas. A concordância interobservador foi avaliada por coeficientes de correlação intraclasse e estatística kappa. Resultados: O achado anormal mais comum foi nodularidade superficial (85%), seguido de fibrose reticular hepática (80%) — leve (25%), moderada (43,8%), grave (31,2%) —, realce heterogêneo (65%), esplenomegalia (60%), aumento do lobo caudado (50%) e linfadenopatia (40%). A concordância interobservador foi quase perfeita para nodularidade superficial (0,83), ascite (0,89) e volume hepático (0,95); entretanto, foi apenas leve (0,12) e razoável (0,25) para grau de fibrose e realce heterogêneo, respectivamente. Também foi leve (0,14) ou regular (0,36) para achados de doença hepática crônica, como fossa da vesícula biliar expandida e espaço pré-portal alargado, respectivamente. Conclusão: A concordância geral foi satisfatória para nodularidade superficial (achado anormal mais prevalente), ascite, volume hepático e esplenomegalia. Critérios frequentes, porém menos objetivos, tiveram apenas concordância leve a razoável. Objetivo Métodos abdominais patológicos 2 crônica ambas kappa Resultados 85%, 85 85% , (85%) 80% 80 (80% 25%, 25 25% (25%) 43,8%, 438 43,8% 43 8 (43,8%) 31,2% 312 31 (31,2% 65%, 65 65% (65%) 60%, 60 60% (60%) 50% 50 (50% 40%. 40 40% . (40%) 0,83, 083 0,83 0 83 (0,83) 0,89 089 89 (0,89 0,95 095 95 (0,95) entretanto 0,12 012 12 (0,12 0,25 025 (0,25 respectivamente 0,14 014 14 (0,14 0,36 036 36 (0,36 préportal pré alargado Conclusão prevalente, prevalente prevalente) frequentes objetivos (85% (80 (25% 43,8 4 (43,8% 31,2 3 (31,2 6 (65% (60% 5 (50 (40% 08 0,8 (0,83 (0,8 0,9 09 9 (0,95 0,1 01 1 (0,1 0,2 02 (0,2 0,3 03 (0,3 (85 (8 (25 43, (43,8 31, (31, (65 (60 (5 (40 0, (0, (0,9 ( (2 (43, (31 (6 (4 (0 (43 (3

ABSTRACT BACKGROUND: Although autonomic dysfunction has been shown to be associated with liver cirrhosis, the prevalence and prognostic implications are unclear. Abnormal heart rate variability (HRV), a measure of autonomic function, has not been well investigated in cirrhosis. OBJECTIVE: To evaluate the prevalence of high-risk HRV parameters in a cohort of cirrhotic patients and their association with cardiac dysfunction and mortality. DESIGN AND SETTING: Prospective observational study conducted in the Federal University of São Paulo. METHOD: A cohort of 120 patients, comprising 17 healthy controls and 103 cirrhotic outpatients, was evaluated and followed for 10 months. HRV analysis was based on 24-hour Holter monitoring and defined using time-domain and frequency-domain parameters. RESULTS: The HRV parameters were statistically lower in cirrhotic patients than in healthy subjects. High-risk HRV parameters were prevalent, such that 64% had at least one high-risk parameter. Time-domain parameters correlated with Child scores (P < 0.0001). In regression models, HRV parameters were independent predictors of diastolic dysfunction and mortality. During 10 months of follow-up, there were 11 deaths, all of patients with at least one high-risk HRV parameter. Kaplan-Meier analysis estimated low survival rates among patients with standard deviation of normal-to-normal RR intervals (SDNN) < 100. CONCLUSION: Reduced HRV is prevalent in liver cirrhosis and is related to cardiac dysfunction, severity of liver disease and mortality. Abnormal high-risk HRV parameters are prevalent among cirrhotic patients and are also predictors of mortality. Our findings highlight the need for a more careful cardiac evaluation of cirrhotic patients.

ABSTRACT Objective: To evaluate the degree of satisfaction and the socioeconomic profile of patients attending Gastroenterology Outpatient Clinics at a University institution linked to the Brazilian Unified Health System. Method: A researcher-administered questionnaire was applied during a structured interview in outpatient clinics. Results: Two hundred and forty (240) patients were included in the study (mean age of 53 years, 55% women). About 30% of the patients had incomplete elementary education, 25% had complete secondary education, and 53% were active workers. Approximately 87% attending the outpatient clinics were from the B2, C1 and C2 socioeconomic classes with an estimated family income of USD$275.00 to USD$825.00/month. Ninety-two percent (92%) of patients were satisfied with the care received; the items associated with a lower degree of satisfaction were facilities/comfort, cleanliness, and waiting time for consultation. No relationship was observed between socioeconomic profile and degree of satisfaction. Conclusion: Satisfaction surveys are important to identify opportunities for improving healthcare services, and it is incumbent upon managers, health professionals and even users to promote compliance with laws and decrees that seek to improve healthcare.

RESUMEN Objetivo: Evaluar el grado de satisfacción y el perfil socioeconómico de pacientes atendidos en Clínicas Externas de Gastroenterología de un centro universitario vinculado al Sistema Único de Salud brasileño. Método: Un cuestionario administrado por investigador fue aplicado durante entrevista estructura en clínicas externas. Resultados: Doscientos cuarenta (240) pacientes fueron incluidos en el estudio (edad media de 53 años, el 55% mujeres). Un 30% de los pacientes tenían educación básica incompleta, el 25% tenían el bachillerato/secundaria incompleto/a y el 53% eran trabajadores activos. Cerca del 87% de los que eran atendidos en la clínicas externas se hallaban en las clases económicas B2, C1 y C2 con ingresos familiares estimados entre 275,00 y 825,00 dólares estadounidenses por mes. El noventa y dos por ciento (92%) de los pacientes estaban satisfechos con la atención recibida; los puntos asociados con bajo grado de satisfacción fueron las instalaciones/comodidad, limpieza y tiempo de espera para consulta. Ninguna relación fue observada entre perfil socioeconómico y grado de satisfacción. Conclusión: Encuestas de satisfacción son importantes para identificar oportunidades de mejorar los servicios sanitarios y para que sus gerentes responsables, profesionales sanitarios e incluso los mismos usuarios puedan promocionar el cumplimiento a la leyes y decretos a fin de mejorar la atención sanitaria.

RESUMO Objetivo: Avaliar o grau de satisfação e o perfil socioeconômico de pacientes que são atendidos em Clínicas Externas de Gastrenterologia de uma instituição universitária vinculada ao Sistema Único de Saúde brasileiro. Método: Um questionário administrado por pesquisador foi aplicado durante uma entrevista estruturada em clínicas externas. Resultados: Duzentos e quarenta (240) pacientes foram incluídos no estudo (idade média de 53 anos, 55% mulheres). Aproximadamente 30% dos pacientes tinham ensino fundamental incompleto, 25% tinham ensino médio incompleto e 53% eram trabalhadores na ativa. Cerca de 87% dos que eram atendidos nas clínicas externas se encontravam nas classes econômicas B2, C1 e C2 com renda familiar estimada entre 275,00 e 825,00 dólares americanos por mês. Noventa e dois por cento (92%) dos pacientes estavam satisfeitos com o atendimento recebido; os itens associados a baixo grau de satisfação foram as instalações/conforto, limpeza e tempo de espera para consulta. Nenhuma relação foi observada entre perfil socioeconômico e grau de satisfação. Conclusão: Pesquisas de satisfação são importantes para identificar oportunidades para melhorar os serviços de saúde e para que seus gerentes responsáveis, profissionais da saúde e até mesmo usuários possam promover o cumprimento às leis e decretos que visam melhorar o atendimento em saúde.

RESUMO Objetivo: Avaliar o grau de satisfação e o perfil socioeconômico de pacientes que são atendidos em Clínicas Externas de Gastrenterologia de uma instituição universitária vinculada ao Sistema Único de Saúde brasileiro. Método: Um questionário administrado por pesquisador foi aplicado durante uma entrevista estruturada em clínicas externas. Resultados: Duzentos e quarenta (240) pacientes foram incluídos no estudo (idade média de 53 anos, 55% mulheres). Aproximadamente 30% dos pacientes tinham ensino fundamental incompleto, 25% tinham ensino médio incompleto e 53% eram trabalhadores na ativa. Cerca de 87% dos que eram atendidos nas clínicas externas se encontravam nas classes econômicas B2, C1 e C2 com renda familiar estimada entre 275,00 e 825,00 dólares americanos por mês. Noventa e dois por cento (92%) dos pacientes estavam satisfeitos com o atendimento recebido; os itens associados a baixo grau de satisfação foram as instalações/conforto, limpeza e tempo de espera para consulta. Nenhuma relação foi observada entre perfil socioeconômico e grau de satisfação. Conclusão: Pesquisas de satisfação são importantes para identificar oportunidades para melhorar os serviços de saúde e para que seus gerentes responsáveis, profissionais da saúde e até mesmo usuários possam promover o cumprimento às leis e decretos que visam melhorar o atendimento em saúde.

ABSTRACT Objective: To evaluate the degree of satisfaction and the socioeconomic profile of patients attending Gastroenterology Outpatient Clinics at a University institution linked to the Brazilian Unified Health System. Method: A researcher-administered questionnaire was applied during a structured interview in outpatient clinics. Results: Two hundred and forty (240) patients were included in the study (mean age of 53 years, 55% women). About 30% of the patients had incomplete elementary education, 25% had complete secondary education, and 53% were active workers. Approximately 87% attending the outpatient clinics were from the B2, C1 and C2 socioeconomic classes with an estimated family income of USD$275.00 to USD$825.00/month. Ninety-two percent (92%) of patients were satisfied with the care received; the items associated with a lower degree of satisfaction were facilities/comfort, cleanliness, and waiting time for consultation. No relationship was observed between socioeconomic profile and degree of satisfaction. Conclusion: Satisfaction surveys are important to identify opportunities for improving healthcare services, and it is incumbent upon managers, health professionals and even users to promote compliance with laws and decrees that seek to improve healthcare.

RESUMEN Objetivo: Evaluar el grado de satisfacción y el perfil socioeconómico de pacientes atendidos en Clínicas Externas de Gastroenterología de un centro universitario vinculado al Sistema Único de Salud brasileño. Método: Un cuestionario administrado por investigador fue aplicado durante entrevista estructura en clínicas externas. Resultados: Doscientos cuarenta (240) pacientes fueron incluidos en el estudio (edad media de 53 años, el 55% mujeres). Un 30% de los pacientes tenían educación básica incompleta, el 25% tenían el bachillerato/secundaria incompleto/a y el 53% eran trabajadores activos. Cerca del 87% de los que eran atendidos en la clínicas externas se hallaban en las clases económicas B2, C1 y C2 con ingresos familiares estimados entre 275,00 y 825,00 dólares estadounidenses por mes. El noventa y dos por ciento (92%) de los pacientes estaban satisfechos con la atención recibida; los puntos asociados con bajo grado de satisfacción fueron las instalaciones/comodidad, limpieza y tiempo de espera para consulta. Ninguna relación fue observada entre perfil socioeconómico y grado de satisfacción. Conclusión: Encuestas de satisfacción son importantes para identificar oportunidades de mejorar los servicios sanitarios y para que sus gerentes responsables, profesionales sanitarios e incluso los mismos usuarios puedan promocionar el cumplimiento a la leyes y decretos a fin de mejorar la atención sanitaria.

OBJECTIVE: To evaluate the effectiveness and safety of first-generation protease inhibitors for the treatment of genotype 1 hepatitis C virus-infected patients at Brazilian reference centers. METHODS: This multicenter cross-sectional study included hepatitis C virus genotype 1 monoinfected patients treated with Peg-interferon, ribavirin, and either boceprevir (n=158) or telaprevir (n=557) between July 2013 and April 2014 at 15 reference centers in Brazil. Demographic, clinical, virological, and adverse events data were collected during treatment and follow-up. RESULTS: Of the 715 patients, 59% had cirrhosis and 67.1% were treatment-experienced. Based on intention-to-treat analysis, the overall sustained viral response was 56.6%, with similar effectiveness in both groups (51.9% for boceprevir and 58% for telaprevir, p=0.190). Serious adverse events occurred in 44.2% of patients, and six deaths (0.8%) were recorded. Cirrhotic patients had lower sustained viral response rates than non-cirrhotic patients (46.9% vs. 70.6%, p<0.001) and a higher incidence of serious adverse events (50.7% vs. 34.8%, p<0.001). Multivariate analysis revealed that sustained viral response was associated with the absence of cirrhosis, viral recurrence after previous treatment, pretreatment platelet count greater than 100,000/mm3, and achievement of a rapid viral response. Female gender, age>65 years, diagnosis of cirrhosis, and abnormal hemoglobin levels/platelet counts prior to treatment were associated with serious adverse events. CONCLUSION: Although serious adverse events rates were higher in this infected population, sustained viral response rates were similar to those reported for other patient cohorts.

Abstract Brazil is one of the 22 countries that concentrates 80% of global tuberculosis cases concomitantly to a large number of hepatitis C carriers and some epidemiological risk scenarios are coincident for both diseases. We analyzed tuberculosis cases that occurred during α-interferon-based therapy for hepatitis C in reference centers in Brazil between 2001 and 2012 and reviewed their medical records. Eighteen tuberculosis cases were observed in patients submitted to hepatitis C α-interferon-based therapy. All patients were human immunodeficiency virus-negative. Nine patients (50%) had extra-pulmonary tuberculosis; 15 (83%) showed significant liver fibrosis. Hepatitis C treatment was discontinued in 12 patients (67%) due to tuberculosis reactivation and six (33%) had sustained virological response. The majority of patients had a favorable outcome but one died. Considering the evidences of α-IFN interference over the containment of Mycobacterium tuberculosis, the immune impairment of cirrhotic patients, the increase of tuberculosis case reports during hepatitis C treatment with atypical and severe presentations and the negative impact on sustained virological response, we think these are strong arguments for latent tuberculosis infection screening before starting α-interferon-based therapy for any indication and even to consider IFN-free regimens against hepatitis C when a patient tests positive for latent tuberculosis infection.

Approximately 170 million people are infected with hepatitis C, and the sustained virological response rate to treatment with pegylated interferon and ribavirin is 30-50%. In an attempt to improve the chances of cure, boceprevir is being added to therapy, but it is associated with an increased incidence of adverse events. We herein report a case of acute pancreatitis developed during treatment with pegylated interferon, ribavirin and boceprevir. Boceprevir was the most likely cause of drug-associated pancreatitis after the most common causes were ruled out, since this adverse event had not occurred when the patient had previously been exposed to pegylated interferon and ribavirin and there was no recurrence of the episode of pancreatitis when these two drugs were reintroduced. Acute pancreatitis is a rare adverse event associated with boceprevir therapy, but a potentially fatal event. Sequential determination of pancreatic enzymes should be considered during hepatitis C treatment with boceprevir.