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- Encased by the Pancreas: An Unusual Cause of Gastric Outlet Obstruction.Publication . Prata, Rita; Martins, Pedro; Ramos, Gonçalo; Coimbra, João
- Prediction of Hepatocellular Carcinoma in a Portuguese Population after Hepatitis C Cure: Comparative Accuracy of Noninvasive Tests (Transient Elastography, FIB-4, and aMAP).Publication . Mascarenhas, André; Serrazina, Juliana; Bronze, Sérgio; Cortez-Pinto, Helena; Presa, José; Barreira, Ana; Carrola, Paulo; Vara-Luiz, Francisco; Rosu-Pires, Alexandra; Martins, Pedro Lages; Prata, Rita; Revés, Joana; Bravo, Catarina; Nascimento, Catarina; Gouveia, Catarina; Franco, Ana Rita; Lima, Pedro; O'Neill, Catarina; Mendes, Raquel R; Simão, Inês Rodrigues; Santos, Inês Costa; Gonçalves, André Ruge; Barreiro, Pedro; Mendo, Rui; Barosa, Rita; Figueiredo, Pedro; Chagas, CristinaIntroduction: Chronic infection with hepatitis C virus (HCV) causes 25% of hepatocellular carcinoma (HCC) cases worldwide, a major cause of morbimortality even after sustained virologic response (SVR). Universal screening to all patients with advanced liver fibrosis is currently recommended. A risk-based strategy could improve the detection rate of early HCC and diminish the surveillance burden. Although several risk prediction models exist, exclusion of a subgroup of patients from surveillance has not yet been recommended. The objective of this study was the comparison of the predictive accuracy of transient elastography, FIB-4, and aMAP for HCC in HCV patients after SVR in Portugal. Methods: This was a multicentric retrospective study including patients with HCV after SVR. Comparative, univariate, multivariate, area under the ROC (receiver-operating characteristic) curve (AUC), and Youden's J-statistic analysis were performed. Results: HCC incidence was 4.2% (1.3/100 patient-years) after a median follow-up of 31 months with inclusion of 337 patients. All patients had a liver stiffness measurement (LSM) before SVR (considered the baseline), but only 148 (43.9%) had a transient elastography after SVR. FIB-4 and aMAP post-SVR were calculated in all patients. Multiple parameters positively correlated with HCC, but only age and baseline transient elastography remained as independent predictors in the multivariate analysis. The optimal cutoffs for prediction of HCC were baseline transient elastography 13.7 kPa, post-SVR transient elastography 16.5 and 15.8 kPa (first and last measurements, respectively), FIB-4 1.6, and aMAP 58. Baseline transient elastography revealed a fair accuracy in predicting HCC (AUC 0.776, p < 0.001), with the cutoff of 13.7 kPa presenting a sensitivity of 85% and a specificity of 69%. Regarding patients who were F3-4 at baseline (n = 162), almost one-third had a baseline LSM ≤13.7 kPa (n = 51, 31.5%), an FIB-4 ≤1.6 (n = 50, 30.9%), and an aMAP score ≤58 (n = 48, 29.6%), and these cutoffs presented an NPV of 98%, 94%, and 96%, respectively, when considering HCC development. Conclusion: Transient elastography (FibroScan) before SVR was a fair predictor of HCC, being more accurate than FIB-4 and aMAP. Transient elastography values ≤13.7 kPa at baseline, FIB-4 ≤1.6 and aMAP ≤58 were the cutoffs considered of low risk for HCC in a Portuguese cohort of HCV patients after SVR with advanced fibrosis. aMAP score is a risk-based surveillance tool that could improve the current HCC screening strategy, but further validation is needed.
- Practical and Multidisciplinary Review on Wilson Disease: The Portuguese Perspective.Publication . Calinas, Filipe; Cardoso, Hélder; Carvalhana, Sofia; Ferreira, José; Gonçalves, Cristina; Magalhães, Marina; Miranda, Helena Pessegueiro; Presa, José; Rolanda, Carla; Santos, Arsénio; Santos, Rui MWilson disease (WD) is a genetic disorder of copper metabolism caused by mutations in the ATP7B gene resulting in toxic copper accumulation in several organs. WD can manifest as liver disease, a progressive neurological disorder, a psychiatric illness, or a combination of these. Other clinical manifestations can also occur. Diagnosis is challenging and typically requires a range of biochemical tests, imaging, genetic testing for ATP7B, and/or liver biopsy. WD is treatable with chelating agents, such as d-penicillamine and trientine, and/or zinc salts alongside with dietary copper restriction. Liver transplantation may be indicated in WD patients with severe hepatic disease, and cautiously considered in patients with neurological WD. Treatment success highly depends on patient adherence and treatment persistence. Therefore, effective interventions for improving patient adherence and close monitoring are key for preventing WD progression. In Portugal, there are no reference centers for WD, and patients are dispersed across numerous medical specialists. This review aimed to summarize the most recent and relevant information for the diagnosis, treatment, and monitoring of WD in Portugal, as well as possible interventions for stimulating adherence to treatment.
- COVID-19 Vaccination in Liver Cirrhosis: Safety and Immune and Clinical Responses.Publication . Canha, Inês; Silva, Mário Jorge; Silva, Maria Azevedo; Sarmento Costa, Mara; Saraiva, Rita Ornelas; Ruge, André; Machado, Mariana Verdelho; Félix, Catarina Sousa; Morão, Bárbara; Figueiredo, Pedro Narra; Mendes, Milena; Leal, Carina; Calinas, FilipeIntroduction: Three years after the beginning of the SARS-CoV-2 pandemic, the safety and efficacy of COVID-19 vaccination in liver cirrhosis (LC) patients remain controversial. We aimed to study the safety, immunological, and clinical responses of LC patients to COVID-19 vaccination. Methods: Prospective multicentric study in adults with LC eligible for COVID-19 vaccination, without prior known infection. Patients were followed up until the timing of a booster dose, SARS-CoV-2 infection, or death. Spike-protein immunoglobulin G antibody titers for SARS-CoV-2 at 2 weeks, 3 months, and 6 months postvaccination were assessed. Antibody titers <33.8 binding antibody units (BAU)/mL were considered seronegative and <200 BAU/mL suboptimal. Postvaccination infection and its severity were registered. Results: We included 124 LC patients, 81% males, mean aged 61 ± 10 years, with a mean follow-up of 221 ± 26 days. Alcohol was the most common (61%) cause of cirrhosis, and 7% were under immunosuppressants for autoimmune hepatitis; 69% had portal hypertension, 42% had a previous decompensation, and 21% had a Child-Pugh-Turcotte score of B/C. The type of vaccine administrated was BNT162b2 (n = 59, 48%), ChAdOx1nCoV-19 (n = 45, 36%), mRNA-1273 (n = 14, 11%), and Ad26.COV2.S (n = 6, 5%). Eighteen percent of the patients reported adverse events after vaccination, none serious. Median [Q1; Q3] antibody titers were 1,185 [280; 2,080] BAU/mL at 2 weeks, 301 [72; 1,175] BAU/mL at 3 months, and 192 [49; 656] BAU/mL at 6 months. There were seronegative and suboptimal antibody responses in 8% and 23% of the patients at 2 weeks, 16% and 38% at 3 months, and 22% and 48% at 6 months. Older age and adenovirus vector vaccines were the only factors associated with seronegative and suboptimal responses at 2 weeks and 3 months (p < 0.05) in a multivariable logistic regression analysis. Eleven patients (9%) were infected with SARS-CoV-2 during follow-up (3.8-6.6 months postvaccination), all with mild disease. There were no differences regarding the type of vaccine, and 73% had antibody titers >200 BAU/mL at 3 months. Conclusion: COVID-19 vaccines in patients with LC were safe, without serious adverse events. The humoral and clinical responses were similar to the reported for the general population. Humoral response was adversely impacted by older age and adenovirus vector vaccines and unrelated to the liver disease severity.
- Endoscopic Retrograde Cholangiopancreatography on Pediatric Patients: Experience of a Portuguese Adult Gastroenterology Department.Publication . Saraiva, Rita Ornelas; Borges, Verónica Pavão; Silva, Mário Jorge; Loureiro, Rafaela; Capela, Tiago; Ramos, Gonçalo; Canena, Jorge; Mateus Dias, António; Alves, Rui; Coimbra, JoãoIntroduction: Experience with endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric population is limited. Few medical centers have experts specifically trained in pediatric therapeutic endoscopy. As a result, patients are generally referred to adult endoscopists with high experience in the procedure. The aim of this study was to characterize the experience of an adult endoscopy unit with ERCP on pediatric patients, with a special focus on very young patients. Methods: We retrospectively analyzed indications, technical success rate, final clinical diagnosis, and complications of ERCPs in children <18 years at our tertiary referral hospital center between January 1994 and June 2022. Results: Sixty-five ERCPs were performed on 57 children with a median age of 13 years (range 1-17 years). Eleven ERCPs were performed on 9 patients up to 5 years old. Indications for ERCP were as follows: biliary obstruction (n = 40), mainly due to choledocholithiasis, lithiasic acute pancreatitis (n = 19), recurrent pancreatitis (n = 3), stent extraction (n = 2), and post-operative biliary fistula (n = 1). The cannulation success rate was 95.1%. Therapeutic interventions were performed in 79% of ERCP. All patients were followed up as inpatients. Complications were recorded in two procedures (3.1%), and no procedure-related mortality occurred. Conclusion: In our experience, ERCP in children can be safely performed with high success rates by advanced adult-trained expert endoscopists at a high-volume center.
- Low Golimumab Trough Levels at Week 6 Are Associated With Poor Clinical, Endoscopic and Histological Outcomes in Ulcerative Colitis Patients: Pharmacokinetic and Pharmacodynamic Sub-analysis of the Evolution Study.Publication . Magro, F; Lopes, S; Silva, M; Coelho, R; Portela, F; Branquinho, D; Correia, L; Fernandes, S; Cravo, M; Caldeira, P; Sousa, H T; Patita, M; Lago, P; Ramos, J; Afonso, J; Redondo, I; Machado, P; Cornillie, F; Lopes, J; Carneiro, FBackground and aims: Golimumab has an established exposure-response relationship in patients with ulcerative colitis [UC]. However, the association of serum golimumab trough levels [TL] with objective markers of disease activity, such as endoscopic and histological activity scores and concentrations of biomarkers, remains less understood. This report describes the relationship of serum golimumab TL at the end of the induction period [Week 6] with clinical, endoscopic, histological, and biomarker parameters. Methods: This was an open-label, uncontrolled, prospective and interventional study. Moderate to severely active UC patients naïve to biologic therapy were treated with golimumab. Serum golimumab TL and faecal calprotectin levels were measured at baseline [Week 0 of induction] and Week 6. Results: A total of 34 patients completed the induction phase [Week 6] and were included in this analysis. Overall, 47.1% and 14.7% of patients achieved clinical response and remission with significantly higher serum golimumab TL in patients with early response or remission [3.7 μg/mL vs 1.3 μg/mL, p = 0.0013; and 3.1 μg/mL vs 1.7 μg/mL, p = 0.0164, respectively]. In addition, golimumab TL were significantly higher in patients achieving histological remission [4.2 μg/mL vs 1.7 μg/mL, p = 0.0049]. Week 6 golimumab TL were inversely correlated with the total Mayo score [rs = -0.546; p = 0.0008], the Mayo endoscopic subscore [rs = -0.381; p = 0.0262], the Geboes histological activity score [rs = -0.464; p = 0.0057], and faecal calprotectin levels [rs = -0.497; p = 0.0044]. Conclusions: A higher early exposure to golimumab is associated with a better objective response in active UC patients and appears to drive the outcome at Week 6.
- Hepatobiliary and Pancreatic: an Unusual Cause of Biliary ObstructionPublication . Saraiva, R; Gamelas, V; Canha, MI; Carvalho, D; Loureiro, R; Coimbra, J
- Burden of Liver Cirrhosis in Portugal Between 2010 and 2017Publication . Silva, JM; Silva, MJ; Calinas, F; Nogueira, PJIntroduction: Liver cirrhosis is a prevalent disease in Portugal. Recent changes in alcohol consumption, as well as the wide use of direct-acting antivirals for hepatitis C since 2015, may be contributing to changes in the national burden of liver cirrhosis in the last few years. Objectives: We aim to characterize the burden of cirrhosis in Portugal between 2010 and 2017. Patients and methods: We analyzed all hospital admission episodes due to cirrhosis in Portugal Mainland between 2010 and 2017, registered in the national Diagnosis-Related Group database, according to etiology of cirrhosis. We also analyzed data on mortality and potential years of life lost from liver cirrhosis and chronic liver disease, retrieved from Statistics Portugal (National Institute for Statistics). Results: Between 2010 and 2017, a total of 51,438 admissions for liver cirrhosis occurred in Portugal. The annual number of admissions decreased (p = 0.044) during the analyzed period. The most frequent cause of cirrhosis was alcoholic liver disease, present in 78.9% of all admissions (n = 40,595), followed by chronic hepatitis C virus infection, present in 11.3% (n = 5,823). A male predominance was identified in the admissions for every analyzed cause of cirrhosis. Annual admissions for alcoholic cirrhosis remained stable (p = 0.075) during the 8-year period. The same stable tendency was observed in the number of admissions for cirrhosis caused by hepatitis C virus (p = 0.861) and alcohol plus hepatitis C virus infection (p = 0.082), although these admissions for hepatitis C-related cirrhosis increased until 2014-2015 and steadily decreased thereafter. Annual deaths due to liver cirrhosis and chronic liver disease decreased from 1,357 in 2010 to 1,038 in 2017 (p = 0.002). The number of potential years of life lost decreased as well in the period (p = 0.001). Conclusion: The burden of cirrhosis, evaluated by hospital admissions, mortality, and potential years of life lost, decreased in Portugal between 2010 and 2017.
- A Challenging Abdominal PainPublication . Rocha, M; Mateus Dias, A; Coimbra, J
- Tofacitinib: an Option for Acute Severe Ulcerative Colitis?Publication . Santos, S; Gamelas, V; Saraiva, R; Simões, G; Saiote, J; Ramos, JTofacitinib has emerged as a new option for ulcerative colitis. Its rapid absorption, metabolism, and clinical improvement make it an interesting option for rescue therapy in acute severe ulcerative colitis (ASUC), a situation with limited therapeutic options in patients with a long-term disease course and multiple drug failure. The management of ASUC in this setting becomes challenging, underlying the need for new drugs and data on their efficacy and safety. We describe 2 cases of acute episodes in which tofacitinib was used as a rescue therapy.