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© 2020 Sociedade Portuguesa de Gastrenterologia Published by S. Karger AG, Basel. This article is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes as well as any distribution of modified material requires written permission, The burden of hepatitis C virus infection remains very high despite huge progress in the cure of the infection. The high prevalence of hepatitis C, especially in vulnerable groups and particularly drug users, may compromise the achievement of the 2030 WHO targets with a 90% reduction in new infections and a 65% reduction in mortality. Therapy with the latest pangenotypic direct-acting antivirals provides cure rates in the order of 97% with short-term oral treatment (8-12 weeks) and with an excellent safety and tolerability profile. Curing the infection causes significant health gains derived from preventing complications from cirrhosis, especially hepatocellular carcinoma, and from liver transplantation. Elimination of hepatitis seems feasible with the implementation of a massive therapy program, focusing particularly on vulnerable populations, through micro-elimination strategies, and in the general population with age-based screening. The reduction of the virus reservoir (humans are the only reservoir) is a determining factor in eradicating the virus., O peso relativo da infecção pelo vírus da hepatite C permanece muito elevado apesar dos enormes progressos verificados na cura da infecção. A elevada prevalência da hepatite C, sobretudo nos nos grupos vulneráveis e em particular nos utilizadores de drogas, pode comprometer o atingimento das metas da WHO para 2030 com redução de 90% de novas infecções e redução de mortalidade em 65%. A terapêutica com os antivíricos de acção directa mais recentes, pangenotípicos, proporciona taxas de cura da ordem dos 97% com tratamento oral de curta duração (8–12 semanas), e com excelente perfil de segurança e to­lerabilidade. A curada infecção ocasiona significativos ganhos em saúde derivados da prevenção das complicações da cirrose, sobretudo do carcinoma hepatocelular, e do transplante hepático. A eliminação da hepatite parece exequível com a aplicação de um programa de massificação da terapêutica, incidindo particularmente nas populações vulneráveis, através de estratégias de microeliminação, e na população geral com rastreio baseado na idade. A redução do reservatório do vírus (o homem é o único reservatório), é determinante para a sua eliminação.

Acute Kidney Injury (AKI) is a very frequent complication in the Acute Liver Failure (ALF) population associated with negative outcomes. We aim to evaluate the impact of AKI duration on the outcomes of an ALF population. A 20-year retrospective analysis of ALF patients admitted to an Intensive Care Unit (ICU) was performed. Chronic liver failure, chronic kidney disease on renal replacement therapy, dialysis requirement within the week prior or an ICU stay of less than 48 h after AKI diagnosis, were exclusion criteria. AKI was defined according to the KDIGO criteria and classified into transient (

Background Acute liver failure (ALF) induced by diffuse metastatic disease has rarely been reported. Case presentation We present a 51-years-old woman with relevant clinical history for breast cancer. The patient was admitted in the emergency department with jaundice, dark urine and pale stools. She was on the 10th day of hormonotherapy for recurrence of breast cancer, diagnosed 7 years previously. Usual causes of acute liver failure were excluded, all drugs were stopped and the imaging studies performed were positive only for steatosis. Nonetheless, ALF progressed and the patient died 4 days later. Autopsy demonstrated a massive intrasinusoidal infiltration of the liver by breast cancer cells. Conclusion We highlight a rare cause of ALF. Although uncommon, physicians should be alert for this situation as the diagnosis can be challenging and the imaging studies can remain normal.

Alcohol abuse can result in a spectrum of liver injury that ranges from mild fatty infiltration to alcoholic hepatitis (AH), cirrhosis, and hepatocellular carcinoma. The present study aimed to evaluate current scoring systems in predicting short-term and long-term mortality because of AH.Records of 170 consecutive patients with AH admitted to a tertiary center between January 2005 and October 2015 were reviewed. Clinical and biochemical parameters were retrieved for the assessment of AH scores for the day of admission (D1) and for the seventh day of hospitalization (D7). Endpoints included admission to the ICU, and 30-day, 90-day, and 1-year mortality.The Maddrey discriminant function and the Model of End-Stage Liver Disease (MELD) were modest predictors of the need for ICU admission. In-hospital, 30-day, 90-day, and 1-year mortality were 15.9, 18.2, 21.8, and 30.0%, respectively. There was a numerical, albeit nonsignificant, trend for higher accuracy using D7 scores, especially the MELD, in predicting 30-day and 1-year mortality. Overall, all scores showed high negative predictive values (30 day: 91.2-98.7% and 1 year: 78.8-93.7%), but modest positive predictive values (30 day: 30.6-70.8% and 1 year: 42.1-61.2%). Survival rates were the highest among patients showing a decrease in the MELD, Glasgow Alcoholic Hepatitis Score, and Age, serum Bilirubin, International normalized ratio, and serum Creatinine score over the first week of admission.AH scores were comparable in identifying patients at low risk of mortality up to 1 year following admission. Reassessment of the MELD, Glasgow Alcoholic Hepatitis Score, and Age, serum Bilirubin, International normalized ratio, and serum Creatinine score scores after 1 week further improved mortality prediction.

Approximately one-third of patients infected with chronic HCV have cirrhosis, and this is likely to increase in the near future. The risk of complications, mainly the development of hepatocellular carcinoma, depends on the presence of cirrhosis, and a significant increase in the incidence of cirrhosis-related events, including mortality, is likely in the following years. All-oral therapy with direct-acting antivirals (DAAs) offers a safe and short treatment, with cure rates over 90% in compensated cirrhosis. Cirrhotic patients should be given high priority for treatment because viral clearance has a significant impact on the natural history of HCV infection, halting the progression of the disease and inducing the regression of fibrosis, as well as reducing the need for liver transplantation and improving survival. The benefit of DAAs is great in patients with decompensated cirrhosis, up until recently a population for whom no alternative therapy was available. The efficacy of all-oral therapy has been reported to improve liver function in about 50% of Child–Pugh class C patients. The regression of cirrhosis observed in more than half of patients achieving viral eradication on prior interferon-based regimens still has to be demonstrated in patients treated with DAAs, although there is reason to believe that this will happen. Advanced cirrhosis will eventually become the last boundary of antiviral therapy that will soon be conquered with new drugs currently pending approval.

© 2019 Wolters Kluwer Health, Inc. All rights reserved., We conducted an epidemiological analysis on pancreatic cancer mortality trends in Portugal over the past 25 years. Using the Portuguese National Statistical Institute public database, we identified deaths attributed to malignant pancreatic cancer (ICD10-C25; ICD9/8–157) on official death certificates issued between 1991 and 2015.

Background and Aims: Laparoscopic sleeve gastrectomy (LSG)-related fistulas are important and potentially fatal complications. We aimed at determining the incidence, predictive factors, and management of recurrence of post-LSG fistulas. Methods: This is a retrospective cohort study of 12 consecutive patients with LSG fistulas managed endoscopically between 2008 and 2013. We analyzed factors associated with recurrence of post-LSG fistulas and the efficacy of a primarily endoscopic approach to manage fistula recurrence. Results: The average age at fistula detection after LSG was 43.3 ± 10.9 years, and 10 (83%) patients were female. The median interval between surgery and initial fistula detection was 14 (4-145) days. Fistulas were located at the gastric cardia in 9/12 patients. A median of 4 (1-10) endoscopies were performed per patient until all fistulas were successfully closed. The median follow-up was 30.5 (15-72) months. Fistula recurrence was detected in 3 (25%) female patients with an average age of 31.7 ± 7.9 years after a median of 119 (50-205) days of the initial fistula closure. Fistulas in all 3 patients recurred at the gastric cardia and were successfully managed endoscopically. There was a second recurrence in 1 patient after 6 months, and she was re-operated with anastomosis of a jejunal loop at the site of the fistula orifice at the gastric cardia. We did not find any factors at initial fistula detection that were significantly associated with fistula recurrence. There were no deaths related to initial fistula after LSG and fistula recurrence. Conclusions: A primarily endoscopic approach is an effective and safe method for the management of fistulas after LSG. Fistula recurrence occurred in 25% of patients and was managed endoscopically. Key Messages: Although we could not define predictive factors of post-LSG fistula recurrence, it is a clinical reality and can be managed endoscopically. Objectivos: As fistulas pós-gastrectomia vertical (sleeve) laparoscópica (LSG) são complicações importantes e potencialmente fatais. O objectivo do estudo foi determinar a incidência, factores preditivos e manejo da recorrência de fistulas pós LSG. Métodos: Estudo retrospectivo de 12 doentes com fistulas pós LSG manejados endoscopicamente entre 2008 e 2013. Analisámos factores associados à recorrência de fistulas pós LSG e a eficácia da abordagem endoscópica. Resultados: Idade média na detecção das fistulas pós LSG foi de 43.3 ± 10.9 anos e 10 (83%) doentes eram mulheres. O intervalo mediano entre a cirurgia e a detecção da fistula inicial foi de 14 (4-145) dias. As fistulas localizaram-se no cárdia em 9/12 doentes. Foram realizadas em mediana 4 (1-10) endoscopias por doente até ao encerramento eficaz das fistulas. O tempo mediano de seguimento foi de 30.5 (15-72) meses. A recorrência das fistulas foi detectada em 3 (25%) doentes, todas mulheres, com idade média de 31.7 ± 7.9 anos, após um tempo mediano de 119 (50-205) dias após encerramento da fistula inicial. As recorrências das fistulas nas três doentes ocorreram no cárdia e foram manejados endoscopicamente. Houve uma segunda recorrência de fistula numa doente após 6 meses que foi reoperada com anastomose de ansa jejunal no local do orifício de fistula no cárdia. Não conseguimos determinar factores na altura da detecção da fistula inicial pós LSG significativamente associados com recorrência de fistulas. Não houve mortalidade associada às fistulas pós LSG (inicial ou recorrência). Conclusões: A abordagem primariamente endoscópica das fistulas pós LSG é um método eficaz e seguro. A recorrência de fistulas ocorreu em 25% dos doentes. As recorrências de fistulas pós LSG são manejáveis endoscopicamente. Mensagens chave: Embora não tenhamos conseguido definir factores preditivos de recorrência de fistulas pós LSG, a recorrência de fistulas é uma realidade clínica e é manejável endoscopicamente.

© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd., Background and aims: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. There are few prospective studies validating risk factors for development of PVT. We analysed the incidence, factors associated with PVT development and its influence on cirrhosis decompensations and orthotopic liver transplant (OLT)-free survival. Methods: In this prospective observational study between January 2014 and March 2019, 445 consecutive patients with chronic liver disease were screened and finally 241 with cirrhosis included. Factors associated with PVT development and its influence on cirrhosis decompensations and OLT-free survival by time dependent covariate coding were analysed. Results: Majority of patients belonged to Child-Pugh class A 184 (76.3%) and the average MELD score was 10 ± 5. Previous cirrhosis decompensations occurred in 125 (52.1%), 63 (26.1%) were on NSBB and 59 (27.2%) had undergone banding for bleeding prophylaxis. Median follow-up was 29 (1-58) months. Cumulative incidence of PVT was 3.7% and 7.6% at 1 and 3 years. Previous decompensation of cirrhosis and low platelet counts but not NSBB independently predicted the development of PVT. During follow-up, 82/236 (34.7%) patients developed cirrhosis decompensations. OLT-free survival was 100% and 82.8% at 3 years, with and without PVT respectively. MELD score, but not PVT, independently predicted cirrhosis decompensations (HR 1.14; 95%CI:1.09-1.19) and OLT-free survival (HR 1.16;95%CI:1.11-1.21). Conclusion: Previous decompensations of cirrhosis and thrombocytopenia predict PVT development in cirrhosis suggesting a pathophysiologic role for severity of portal hypertension. PVT development did not independently predict cirrhosis decompensations or lower OLT-free survival.

Background. Morbid obesity is an important cause of morbidity and mortality. Bariatric surgery is the best option to manage obesity. Vertical gastrectomy is safe and effective but sometimes complicate with hemorrhage, fistulas and stenosis. Fistulas can be solved by conventional surgery or interventional endoscopy. Case presentation. We describe a morbidly obese patient with vertical gastrectomy who developed complications after surgery. Immediately after surgery the patient developed sepsis. Upper gastrointestinal endoscopy excluded fistula. One month later a peri-gastric abscess developed due to a fistula orifice in the distal esophagus, treated with argon plasma and two double pigtail plastic stents placed endoscopically to drain the abscess. The stents were removed two weeks later and was placed a covered metallic stent in the distal esophagus. Six weeks later the metallic stent was removed and the orifice closed. Four months later the patient developed sepsis. Computer tomography revealed a subdiaphragmatic abscess and endoscopy revealed a 2 mm fistula orifice at the previous site, treated with argon plasma and two trough-the-scope clips that closed it. There were no further incidents after two years of follow-up. Conclusions. Early diagnosis and endoscopic approach can resolve these complications without the morbidity and increased mortality risk of surgical re-interventions.

Background Spontaneous bacterial peritonitis (SBP) is a known complication of advanced cirrhosis and presents a high mortality rate. A polymorphonuclear (PMN) cell count >250/μl in the ascitic fluid is the current gold standard for diagnosing SBP. Aim We evaluated the accuracy of a point-of-care test (POCT) for ascitic calprotectin in diagnosing patients with SBP. Methods Eighty-eight patients admitted with decompensation of liver cirrhosis were studied including 41 patients (46.6%) with SBP. Ascitic calprotectin was measured using a quantitative POCT developed by Buhlmann®. Results Calprotectin levels correlated with PMN cell count and other inflammatory markers and were significantly higher in patients with SBP. An optimal cutoff of calprotectin above 1.57 μg/ml presented high sensitivity (87.8%), specificity (97.9%), and positive (97.3%) and negative (90.2%) predictive values for diagnosing SBP. Using calprotectin selectively in patients with a serum albumin-ascites gradient above 11 g/l further increased the sensitivity and negative predictive values of the test. Conclusion Ascitic calprotectin appears to be a reliable method for diagnosing SBP in patients with liver cirrhosis. It may present an alternative to other conventional diagnostic methods.

Obesity remains a major health concern for which surgery has proven to be the most effective treatment in the long term. Routine upper gastrointestinal endoscopy (UGE) is recommended before surgery, but few studies have evaluated its impact on postoperative complications. We studied a cohort of 613 patients submitted to UGE before being listed for bariatric surgery between May 2004 and May 2015. A logistic regression analysis was performed to evaluate potential predictors of postoperative complications. Three hundred forty-five patients (56.3 %) presented abnormal endoscopic findings. Helicobacter pylori (Hp) was the strongest predictor of an abnormal endoscopy (OR 10.343, 95 % CI [3.970–26.943], p

Background Acute pancreatitis (AP) represents a complex and potentially fatal disease with a highly variable clinical course. Three classification systems for assessing the severity in AP have been validated for clinical use. Aims The aim of the present study was to evaluate the performance of the Atlanta and Determinant-based classifications in predicting severe clinical outcomes in patients with AP. Methods In this retrospective study we reviewed the treatment and follow-up records of 525 patients with AP admitted to our unit between the years of 2003 and 2014. Outcomes included mortality, admission to the ICU, need for interventional procedures or nutritional support, and duration of hospital and ICU stay. Results The prevalence of organ failure and persistent organ failure in our cohort was 23.0 and 10.7%, respectively, and the mortality rate was 5.9%. Higher grades of severity were associated with worse outcomes in all classification systems. The revised Atlanta and Determinant-based classifications performed similarly in predicting outcomes, and both proved to be superior to the former classic Atlanta classification. Conclusion Recent classifications proved to be more accurate in predicting important clinical outcomes in patients with AP.

The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. We analyzed the safety and effect of anticoagulant therapy (AT) on PVT recanalization and orthotopic liver transplant (OLT)-free survival. Eighty consecutive patients from a prospective registry of cirrhosis and non-tumoral PVT at a tertiary center were analyzed. AT effect on PVT recanalization and OLT-free survival was determined by time-dependent Cox regression analysis. Average MELD score was 15 ± 7. Portal hypertension-related complications at PVT diagnosis were present in 65 (81.3%) patients. Isolated portal vein trunk/branch thrombosis was present in 53 (66.3%) patients. AT was started in 37 patients. AT was stopped in 17 (45.9%) patients, in 4 (10.8%) due to bleeding events. No variceal bleeding occurred while on AT. Anticoagulation was restarted in 6/17 (35.2%) patients due to rethrombosis. In 67 patients with adequate follow-up imaging, AT significantly increased the rate of PVT recanalization compared with those who did not receive anticoagulation [51.4% (18/35) vs 6/32 (18.8%), p = 0.005]. OLT-free survival after a median follow-up of 25 (1–146) months was 32 (40%). Although there was no significant effect of AT on overall OLT-free survival, OLT-free survival was higher among patients with MELD ≥ 15 receiving AT compared to those who did not (p = 0.011). Baseline MELD at PVT detection independently predicted PVT recanalization (HR 1.11, 95% CI 1.01–1.21, p = 0.027) and mortality/OLT (HR 1.12, 95% CI 1.05–1.19, p

Joana Gameiro,1 José Agapito Fonseca,1 Joana Monteiro Dias,1 Maria João Melo,1 Sofia Jorge,1 José Velosa,2 José António Lopes1 1Division of Nephrology and Renal Transplantation, Department of Medicine, Centro Hospitalar Lisboa Norte, Lisboa, Portugal; 2Department of Gastroenterology and Hepatology, Centro Hospitalar Lisboa Norte, Lisboa, Portugal Introduction: Acute kidney injury (AKI) is common in hospitalized patients with cirrhosis and is associated with poor prognosis. A risk prediction score combining values easily measured at admission could be valuable to stratify patients for prevention, monitoring and early intervention, ultimately improving patient care and outcomes. The aim of this study was to develop a risk score for AKI in a cohort of cirrhotic patients. Patients and methods: We cross-examined the data from a retrospective analysis of 186 patients with cirrhosis admitted to the Gastroenterology and Hepatology Service of Centro Hospitalar Lisboa Norte from January 2003 to December 2005. AKI was defined as an increase in serum creatinine (SCr)≥0.3mg/dL within 48hours or a percentage increase in SCr≥50% from baseline. Neutrophil-to-lymphocyte ratio (NLR) was used as a marker for inflammation. A receiver operating characteristic (ROC) curve was produced to assess the discriminative ability of the variables. Cutoff values were defined as those with highest validity. The final AKI risk score model was assessed using the ROC curve. Results: A total of 52 patients (28%) developed AKI. Higher baseline SCr (p

Summary Background & aims Vitamin D is known to modulate immune function and proliferation. Higher vitamin D [25(OH)D3] serum levels have been reported to have protective effects on adenoma detection and colorectal cancer (CRC) development and survival. Methods This retrospective cohort study included 315 peri and post-menopausal women submitted to opportunistic colorectal and osteoporosis screening at the gynaecology outpatient clinic of a tertiary medical centre between 2004 and 2015. Colonoscopy findings were correlated with 25(OH)D3 and PTH serum levels, and subsequently adjusted in a multivariate logistic regression model. Confounding factors included demographic and colorectal risk factors, pharmacological therapies and bone densitometry metrics. Results A total of 77 lesions were identified in 66 patients. Vitamin D insufficiency ( Conclusion The association of 25(OH)D3 serum levels with colorectal lesions seems to be restricted to adenomatous lesions and is influenced by histological grading. Vitamin D may be a valuable biomarker for optimization of risk stratification in group-specific CRC screening protocols.

Introduction Acute diverticulitis represents an inflammatory process originating in a colonic diverticulum. Although acute diverticulitis usually follows a benign course - uncomplicated diverticulitis (UCD), up to 25% may develop complicated diverticulitis (CD) including abscesses, fistulas, strictures, and perforation. Current guidelines recommend performing colonoscopy after an episode of acute diverticulitis to rule out colorectal cancer (CRC). However, the literature supporting this recommendation is still scarce. Aim and methods Our aim was to assess the relevance of endoscopy following an acute diverticulitis. Using a large cohort from a tertiary center, we retrospectively evaluated endoscopic and histologic findings in patients with acute diverticulitis confirmed by abdominal ultrasound or computed tomography. Findings were characterized as (a) normal endoscopy (highlighting only the presence of diverticula), (b) abnormal endoscopy with low-grade lesions, and (c) abnormal endoscopy with high-grade lesions. Results A total of 427 patients were evaluated, including 347 cases with UCD and 80 cases with CD. The prevalence of normal findings at endoscopy was 67.3%. An advanced adenoma or neoplasm was found in 23.7%. The estimated number of endoscopies required for diagnosing a CRC was 29 in UCD and 10 in CD. Increasing age and male sex were associated significantly with the presence of polyps at endoscopy. Increasing age was associated with CD and cancer. Conclusion In our series, the prevalence of high-grade lesions and CRC was higher than that in other studies. In our opinion, the findings justify endoscopic evaluation, especially in older patients with CD.

BACKGROUND & AIMS Chronic liver disease is a major worldwide cause of morbidity and mortality. Palliative care policies are not clearly established in chronic liver disease. The NECPAL CCOMS-ICO© (NECesidades PALiativas/Palliative Needs) is a tool to identify palliative care needs, including a section for liver disease. AIM The aim of this study was to identify palliative care needs in liver patients hospitalised in a tertiary referral Liver Unit. METHODS Single-centre prospective observational study. One hundred and twenty patients with cirrhosis were included and NECPAL questionnaire was applied to all patients in a 7-month period. RESULTS 84.2% of patients were considered as requiring palliative intervention; however, clinicians identified those needs only in 65.8% of the cases and caregivers in 6.7% of the cases; less than 8% of the patients were referred for palliative care consultation. An excessive use of healthcare resources (positive answer to question 3) was strongly associated with a positive need for palliative care (positive NECPAL): OR 7.305, CI 95% 2.54-20.995, P

BACKGROUND Acute pancreatitis (AP) is an aggressive and potentially fatal clinical condition. Although all age groups are at risk, the elderly may be a group of special concern. We aimed at evaluating clinical outcomes of patients with elderly-onset AP. MATERIALS AND METHODS Using a single-center retrospective database, treatment and follow-up records of 550 patients admitted with AP were reviewed. Outcomes included mortality, admission to the ICU, need for interventional procedures, nutritional support, and length of hospital and ICU stay. Elderly-onset AP was defined as an episode of AP occurring in patients older than 65 years. RESULTS A total of 263 patients were classified as having elderly-onset AP. There was an association between older age and higher Ranson and the bedside index of severity in AP scores, translating into longer lengths of hospital stay, higher requirements for ICU admission, interventional procedures, organ failure, persistent organ failure, and overall mortality. In multivariate analysis, age was an independent predictor of mortality in AP. CONCLUSIONS Age was strongly associated with a more severe course of AP. Early recognition and prompt action are essential to improve outcomes in this population.

Signet ring cell carcinoma is a rare form of adenocarcinoma that predominantly affects the stomach. Signet ring cell carcinoma originated from the ileum is extremely rare and the prognosis is poor. We present a case of small bowel obstruction with features suggesting Crohn disease of the ileum. The symptoms were chronic diarrhea and abdominal pain with a family history of inflammatory bowel disease. The patient underwent surgery and histopathology revealed both aspects of signet ring cell carcinoma and Crohn disease of the ileum. An association between long-standing inflammation and the development of this subtype of tumor has been proposed but there are no established surveillance guidelines for small bowel neoplasm in inflammatory bowel disease.O carcinoma de células em anel de sinete é uma forma rara de adenocarcinoma que afeta predominantemente o estômago. A forma primária do íleon é muito rara e apresenta mau prognóstico. Apresentamos um caso de doença de Crohn do íleon cuja forma de apresentação cursou com quadro oclusivo associado a adenocarcinoma de células em anel de sinete do íleon. A doente apresentava história prévia de diarreia crónica e tinha história familiar de doença inflamatória. Foi submetida a ressecção cirúrgica e os achados histopatológicos revelaram a presença de aspetos compatíveis com doença de Crohn do íleon e adenocarcinoma de células em anel de sinete. Foi proposta uma associação com esta forma rara de tumor e inflamação de longa duração, contudo, não existem recomendações estabelecidas de vigilância de neoplasia na doença inflamatória intestinal com atingimento ileal.

BACKGROUND Mucosal healing (MH) is currently accepted as one of the best treatment targets in Crohn's disease. However, even in patients with sustained MH, residual bowel wall inflammation can still be detected by cross-sectional imaging. The long-term benefits of obtaining transmural healing (TH) have not been previously assessed. METHODS We performed an observational study including 214 patients with Crohn's disease with a magnetic resonance enterography (MRE) and colonoscopy performed within a 6-month interval. Patients were classified as having TH (inactive MRE and colonoscopy), MH (active MRE with inactive colonoscopy), or no healing (active colonoscopy). Need for surgery, hospital admission, and therapy escalation were evaluated at 12 months of follow-up. RESULTS Patients with TH presented lower rates of hospital admission, therapy escalation, and surgery than patients with MH or no healing. In logistic regression analysis, endoscopic remission (odds ratio 0.331 95% confidence interval [0.178-0.614], P < 0.001) and MRE remission (odds ratio 0.270 95% confidence interval [0.130-0.564], P < 0.001) were independently associated with a lower likelihood of reaching any unfavorable outcome. CONCLUSIONS TH is associated with improved long-term outcomes in Crohn's disease and may be a more suitable target than MH.

Aim - To identify clinical and/or genetic predictors of response to several therapies in Crohn’s disease (CD) patients. Methods - We included 242 patients with CD (133 females) aged (mean ± standard deviation) 39 ± 12 years and a disease duration of 12 ± 8 years. The single-nucleotide polymorphisms (SNPs) studied were ABCB1 C3435T and G2677T/A, IL23R G1142A, C2370A, and G9T, CASP9 C93T, Fas G670A and LgC844T, and ATG16L1 A898G. Genotyping was performed with real-time PCR with Taqman probes. Results - Older patients responded better to 5-aminosalicylic acid (5-ASA) and to azathioprine (OR 1.07, p = 0.003 and OR 1.03, p = 0.01, respectively) while younger ones responded better to biologicals (OR 0.95, p = 0.06). Previous surgery negatively influenced response to 5-ASA compounds (OR 0.25, p = 0.05), but favoured response to azathioprine (OR 2.1, p = 0.04). In respect to genetic predictors, we observed that heterozygotes for ATGL16L1 SNP had a significantly higher chance of responding to corticosteroids (OR 2.51, p = 0.04), while homozygotes for Casp9 C93T SNP had a lower chance of responding both to corticosteroids and to azathioprine (OR 0.23, p = 0.03 and OR 0.08, p = 0.02,). TT carriers of ABCB1 C3435T SNP had a higher chance of responding to azathioprine (OR 2.38, p = 0.01), while carriers of ABCB1 G2677T/A SNP, as well as responding better to azathioprine (OR 1.89, p = 0.07), had a lower chance of responding to biologicals (OR 0.31, p = 0.07), which became significant after adjusting for gender (OR 0.75, p = 0.005). Conclusions - In the present study, we were able to identify a number of clinical and genetic predictors of response to several therapies which may become of potential utility in clinical practice. These are preliminary results that need to be replicated in future pharmacogenomic studies.

Background Crigler-Najjar syndrome (CN) is a very rare genetic disorder characterized by an inability to conjugate bilirubin. Contrary to CN type I, patients with CN II exhibit residual capacity to conjugate bilirubin and may present a normal life expectancy. Case presentation We report an unusual late diagnosis of CN type II in an 80-year-old female admitted with severe acute cholangitis. While the patient present typical clinical and radiologic signs of bile duct obstruction and cholangitis, her blood analysis showed severe unconjugated hyperbilirubinemia. Endoscopic retrograde cholangiopancreatography confirmed the diagnosis and allowed therapeutic intervention. The anatomopathologic examination of her gallbladder following cholecystectomy showed signs of chronic cholecystitis. Conclusion The risk of gallstone disease may be increased in patients with CN syndrome. While unusual, we alert to this curious and potential life-threatening presentation.

Background Liver disease, one of the most common causes of hospitalization worldwide, is particularly prevalent in Europe. This study aimed to determine the number of hospital discharges and admissions, mortality, premature death and costs associated with liver disease from the perspective of the National Health Service in Portugal. Methods A descriptive, retrospective analysis of data from 97 hospitals between 2000 and 2008, and mortality data for 2011 collected from the Portuguese National Institute of Statistics. The 9th and 10th revisions of the international classification of diseases were used to establish diagnoses. National data on demographics, average length of stay, in-patient mortality and direct costs associated with hospital admissions and liver transplantation were compared for the most common liver diseases. Mortality and premature death were compared using the potential years of life lost (PYLL) index. Results The annual mean number of discharges for liver disease was 11,503 between 2000 and 2008. Most cases of liver disease were diagnosed in men (70.4 %) and the prevalence of liver disease peaked in patients aged from 20 to 64 years (60.7 %). Alcoholic cirrhosis was the most frequent liver-disease diagnosis leading to discharge (38.6 %). In addition, alcoholic cirrhosis emerged as the main cost-driver, accounting for €26,818,930 (42.6 %) of the total cost imposed by liver disease. Overall, chronic hepatic disease was the 10th most common cause of mortality in Portugal in 2011, causing 21.8 deaths per 100,000. Chronic hepatic disease and hepatocellular carcinoma are even more important causes of premature death, ranking third based on PYLL. Conclusion In 2011, liver disease was the 10th most common cause of death and the third most important cause of premature death in Portugal. Alcohol cirrhosis was the leading cause of liver-related hospital admissions between 2001 and 2008. It appears that liver disease imposes a considerable social and economic burden on Portugal. Our results suggest that educational, legislative and therapeutic interventions to prevent morbidity, mortality and premature death from liver disease are urgently required to minimise the economic and clinical burdens.

Liposarcomas are malignant soft tissue neoplasms usually located in the retroperitoneum, head, neck and extremities. Although secondary invasion of the gastrointestinal tract by retroperitoneal sarcomas is frequent, primary involvement is uncommon. We report a young patient with Crohn's disease in remission presenting with intussusception of the colon due to a primary liposarcoma of the sigmoid. Emergency resection confirmed the diagnosis. The patient remains in remission after five years. This represents the youngest diagnosis of liposarcoma to date and the first occurring in the sigmoid. We also highlight the concomitant diagnosis of Crohn's disease.

Chronic liver disease and cirrhosis are known risk factors for hepatocellular carcinoma (HCC). We present a 68- year old male patient with chronic hepatitis B infection and cirrhosis admitted for investigation of a 10-cm solid liver nodule detected during surveillance ultrasound. Abdominal computed tomography (CT) revealed an additional 3 cm lesion in the right liver lobe and multiple lesions in the spleen without typical features of HCC. Histopathologic examination of the liver nodule biopsy was compatible with metastasis from a neuroendocrine tumor. The patient was referred for chemotherapy but unfortunately died 2 months later from infectious complications. The present case seemed unusual considering that the background chronic liver disease would make HCC a more likely diagnosis. The multiple comorbidities presented by our patient provided an additional challenge by broadening the differential diagnosis.

Background: Acute severe colitis (ASC) remains a challenging complication of ulcerative colitis. The early identification of patients who will not respond to optimal therapy is warranted. Increasing evidence suggests that endoscopy may play a role in predicting important outcomes in acute severe colitis. Methods: The endoscopic activity of consecutive patients with acute severe colitis was evaluated using the Mayo endoscopic sub-score (Mayo) and the ulcerative colitis endoscopic index of severity (UCEIS). Two segmental indexes were also produced by summing the scores of the rectum and sigmoid (seg-Mayo and seg-UCEIS, respectively). Endpoints included the need for salvage therapy with infliximab or cyclosporine, refractoriness to corticosteroids, and colectomy. Results: Of one hundred and eight patients enrolled in the study, 60 (55.6%) were male; with a median age of 34.5 years (range 15-80). All patients received intravenous steroids. Fifty-nine patients (55.6%) showed an incomplete or absent response to steroids, 35 patients (34.3%) received salvage therapy with infliximab or cyclosporine and 38 patients (33.3%) were colectomized during the index hospitalization or within the first year of follow-up. All scores were able to predict the need for surgery, but only the seg-UCEIS significantly predicted refractoriness to steroids. Conclusions: There was a strong correlation between endoscopic severity and unfavorable outcomes. The UCEIS outperformed the Mayo endoscopic sub-score in all important outcomes. Segmental scoring further improved the performance of the UCEIS.

Ischemic colitis is the most common subtype of intestinal ischemia usually resulting from vasospasm, vessel occlusion or mesenteric hypoperfusion. Neuroleptics have seldom been linked to ischemic colitis by blocking peripheral anticholinergic and antiserotonergic receptors inducing severe gastrointestinal paresis. We report a young patient with severe ischemic colitis requiring surgery due to necrosis of the bowel. After exclusion of other potential causes, olanzapine was admitted as the cause of ischemia. Clinicians should be aware of how to recognize and treat the potentially life-threatening effects of neuroleptics.

Background and Aims: Deploying a longitudinal perspective, we observe how cirrhosis caused mortality rates in Portugal are converging with the levels reported in the European Union (15 countries). However, we still lack analysis of the burden of alcoholic cirrhosis in terms of hospital admissions and associated mortality. As Portugal may be considered a paradigmatic case in Europe, our aim was to characterize the evolution of hospital admissions for alcoholic cirrhosis between 1993 and 2008 and draw conclusions for other countries. Methods: Retrospective analysis of the hepatic cirrhosis admissions in 97 Portuguese state hospitals was carried out based on the National Registry. Results: We report a convergence in terms of mortality rates resulting from cirrhosis between Portugal and European Union (a differential of 6.7 deaths per 100 000 habitants in 1994 to 0.4 in 2008). We accounted for 81 543 hospital admissions for cirrhosis: 84% for alcoholic cirrhosis and 16% for non-alcoholic cirrhosis. Hospital admissions have increased 29% in men and with no increase in women. In the male, alcoholic cirrhosis patient group aged between 40 and 54, the rise in hospital admissions was more pronounced with an increase of around 45%. These patients underwent longer lengths of stay and reported higher mortality rates and passing away 20 years earlier than the average national expectancy of life. Conclusions: These data draw attention to the burden of alcohol consumption not only in Portugal but also in other countries and its impacts on hospital systems and on policy making. info:eu-repo/semantics/publishedVersion

The hepatitis C virus (HCV) infection is one of the most important chronic viral infections worldwide and affects 3% of the world population, approximately 170-200 million people. The consequences of chronic infection are liver cirrhosis and hepatocellular carcinoma, which develop in 20-30% of the patients, leading to hepatic failure, need for liver transplantation and death. The hepatitis C virus is a RNA virus that is prevalent worldwide and is classified by the World Health Organization (International Agency for Research on Cancer) as one of the six oncogenic viruses. Hepatocellular carcinoma is one of the most important cancers and is fifth worldwide, but third in men in terms of mortality. Hepatitis C kills approximately 350,000 people every year, surpassing HIV infection in many countries as a cause of death.Hepatitis C virus can kill in different ways: it can cause cirrhosis, cancer or severe liver disease in people co-infected with HIV. Hepatitis C treatment started in the mid 1980s with a 6% efficacy rate among patients taking thrice-weekly injections of human interferon. This therapy had numerous side effects. The efficacy of hepatitis C treatment has increased, and currently, the efficacy of the so-called direct antiviral agents (DAAs) is 80-90%. The benefits of a cure are enormous and include a lifetime negative serum HCV RNA, disappearance of HCV in the liver, regression of cirrhosis, decreased risk of developing hepatocellular carcinoma, disappearance of oesophageal varices, no more risk of HCV transmission to sexual partners or children, and increased survival.At present, hepatitis C can be considered a curable disease.

OBJECTIVE: We aimed to identify the clinical and genetic [IL23 receptor (IL23R) single nucleotide polymorphisms (SNPs)] predictors of response to therapy in patients with ulcerative colitis. PATIENTS AND METHODS: A total of 174 patients with ulcerative colitis, 99 women and 75 men, were included. The mean age of the patients was 47±15 years and the mean disease duration was 11±9 years. The number of patients classified as responders (R) or nonresponders (NR) to several therapies was as follows: 110 R and 53 NR to mesalazine (5-ASA), 28 R and 20 NR to azathioprine (AZT), 18 R and 7 NR to infliximab. Clinical and demographic variables were recorded. A total of four SNPs were studied: IL23R G1142A, C2370A, G43045A, and G9T. Genotyping was performed by real-time PCR using Taqman probes. RESULTS: Older patients were more prone to respond to 5-ASA (P=0.004), whereas those with pancolitis were less likely to respond to such therapies (P=0.002). Patients with extraintestinal manifestations (EIMs) were less likely to respond to 5-ASA (P=0.001), AZT (P=0.03), and corticosteroids (P=0.06). Carriers of the mutant allele for IL23R SNPs had a significantly higher probability of developing EIMs (P