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- A complex case of low-phospholipid-associated cholelithiasis syndrome.Publication . Figueiredo, LM; Lourenço, L; Horta, D; Martins, AThe low-phospholipid-associated cholelithiasis (LPAC) syndrome is a form of symptomatic and recurring cholelithiasis occurring in young adults, associated with mutations in the ABCB4 gene. It is a clinical syndrome characterized by at least two of the following criteria: age at onset of biliary symptoms below 40 years, intrahepatic echogenic foci or microlithiasis and recurrence of biliary symptoms after cholecystectomy. In the rare cases progressing to end-stage liver disease, a liver transplant may be indicated. We report a case of a 40-year-old female patient with clinical criteria for LPAC syndrome and with ABCB4 gene mutation. She had a complex history of choledocholithiasis recurrence despite treatment with ursodeoxycholic acid and multiple therapeutic endoscopic retrograde cholangiopancreatography, and she developed portal vein thrombosis.
- Endoscopic therapy in disconnected duct syndrome: re-connecting the duct.Publication . Figueiredo, LM; Lourenço, L; Horta, D; Martins, AWe report the case of a 61-year-old male patient with a history of acute necrotizing biliary pancreatitis and a disconnected duct syndrome. He underwent transgastric drainage using a luminal apposing metal stent and transgastric necrosectomy with complete resolution of the necrosis. A pancreatic fistula was identified during pancreatography and a pancreatic plastic stent was placed in order to manage the disconnected duct syndrome. The tip of the pancreatic stent could be seen inside the pancreatic collection, which is an unusual finding. There was a resolution of the collection and the pancreatic stent was removed.
- A Rare Cause of Diarrhea in the Occident: A Case of Colonic SchistosomiasisPublication . Branco, J; Santos, L; Manso, RT; Reis, J
- Should We Use Papilla Morphology to Estimate the Size of the Terminal Common Bile Duct During Endoscopic Retrograde Cholangiopancreatography?Publication . Lopes, L; Canena, J; Fernandes, J; Moreira, M; Costa, I; Gomes-Fonseca, J, et al.Objective: A small common bile duct (CBD) diameter has been associated with complications and with a difficult biliary cannulation. Previous studies suggested that this diameter can be predicted during the endoscopic retrograde cholangiopancreatography (ERCP) simply by observing the papillary morphology. Despite this published suggestion there is no study addressing this topic. This study evaluated a possible association between the morphology of the major papilla and the diameter of the terminal CBD (t-CBD). Methods: Observational cross-sectional study including consecutive patients with naïve papillae was referred for ERCP in two affiliated university hospitals. The transverse (p-transv) and longitudinal measures (p-long) of the papilla were obtained using a visual method. Papillae were classified into nonprominent, prominent, bulging or other. The t-CBD was measured 1 cm from the papilla using fluoroscopic images in prone/supine. Measurements were performed by two senior endoscopists and outcomes were evaluated using correlation and linear regression model. Results: We included 245 patients with a median age of 76 years. The median p-transv for each type of papillae was as follows: nonprominent = 6 mm, prominent = 9 mm, bulging = 15 mm and other = 6 mm; P < 0.001. The median t-CBD for nonprominent = 7.62 mm, prominent = 8.34 mm, bulging = 8.60 mm and other = 8.52 mm; P = 0.40. The correlation between the transverse and longitudinal measures of papilla and the t-CBD were 0.0092 and 0.0614, respectively. In the regression model, the t-CBD diameter was not explained by papilla's size or morphology (R = 1.70%; P = 0.80). Conclusion: The morphology of the papilla must not be used as a predictor of the diameter of the CBD as there is no correlation between these two items.
- Diagnosis and outcome of oesophageal Crohn's diseasePublication . Rodrigues, R; Sladek, M; Katsanos, K; van der Woude, J; Wei, J; Vavricka, R; Teich, N; Ellul, P; Savarino, E; Chaparro, M; Beaton, D; Oliveira, AM, et al.BACKGROUND AND AIMS: Crohn's disease (CD) can involve any part of the gastrointestinal tract. We aimed to characterize clinical, endoscopic, histologic features and treatment outcomes of CD patients with oesophageal involvement. METHODS: We collected cases through a retrospective multicentre European Crohn's and Colitis Organisation CONFER [COllaborative Network For Exceptionally Rare case reports] project. Clinical data were recorded in a standardized case report form. RESULTS: A total of 40 patients were reported [22 males, mean (±SD, range) age at oesophageal CD diagnosis: 25 (±13.3, 10-71) years and mean time of follow-up: 67 (±68.1, 3-240) months]. Oesophageal involvement was established at CD diagnosis in 26 patients (65%) and during follow-up in 14. CD was exclusively located in the oesophagus in 2 patients. Thirteen patients (32.2%) were asymptomatic at oesophageal disease diagnosis. Oesophageal strictures were present in 5 patients and fistulizing oesophageal disease in one. Eight patients exhibited granulomas on biopsies. Proton-pump inhibitors (PPIs) were administered in 37 patients (92.5%). Three patients underwent endoscopic dilation for symptomatic strictures and none oesophageal-related surgery. Diagnosis in pre-established CD resulted in treatment modifications in 9/14 patients. Clinical remission of oesophageal disease was seen in 33/40 patients (82.5%) after a mean time of 7 (±5.6, 1-18) months. Follow-up endoscopy was performed in 29/40 patients and 26/29 (89.7%) achieved mucosal healing. CONCLUSION: In this case series the endoscopic and histologic characteristics of isolated oesophageal CD were similar to those reported in other sites of involvement. Treatment was primarily conservative, with PPIs administered in the majority of patients and modifications in pre-existing IBD-related therapy occurring in two thirds of them. Clinical and endoscopic remission was achieved in more than 80% of the patients.
- The impact and evolution of acute-on-chronic liver failure in decompensated cirrhosis: A Portuguese single-center studyPublication . Cardoso, M; Alexandrino, G; Carvalho E Branco J2, Anapaz V2, Carvalho R2, Horta D2, Martins A2.; Branco, J; Anapaz, V; Carvalho, R; Horta, D; Martins, AAcute-on-chronic liver failure (ACLF) is a dynamic syndrome that should be assessed repeatedly. An algorithm for risk stratification in decompensated cirrhosis was recently proposed by the EASL-CLIF (European Association for the Study of the Liver-Chronic Liver Failure) Consortium. AIM: To validate the EASL-CLIF Consortium scores in patients with and without ACLF. MATERIALS AND METHODS: Retrospective single-center cohort study including patients admitted for acute decompensation of cirrhosis between January 2014 and December 2015, and followed-up until December 2016. We separated patients with and without ACLF and compared the various EASL-CLIF Consortium scores to Child-Pugh and MELD for predicting 28-day (M28), 90-day and 12-month mortality. These scores were recalculated at different time points over 28 days. RESULTS: 106 patients were included (age 60.3±10.7 years; 87.7% male), 35.8% of whom met ACLF criteria on admission (50%) or during hospitalization. A CLIF-C AD Score ≥60 on admission was associated with a higher risk of developing ACLF. The onset of ACLF during hospitalization portended a poor prognosis. The prognostic performance of the CLIF-C ACLF Score (AUROC for M28: 0.856±0.071) was globally comparable to that of Child-Pugh and MELD. Overall, ACLF resolved in 54.1% patients, resulting in increased survival. Almost 40% of the patients reached their final ACLF grade after ≥8 days, with 13.9% of ACLF patients experiencing resolution by then. DISCUSSION: We confirmed the accuracy and clinical value of the several proposed scores in our population. Prognosis was better defined by the early clinical course than by the initial evaluation, emphasizing the importance of repeated assessments.
- Comparing the Continuous Geboes Score With the Robarts Histopathology Index: Definitions of Histological Remission and Response and Their Relation to Faecal Calprotectin LevelsPublication . Magro, F; Lopes, J; Borralho, P; Lopes, S; Coelho, R; Cotter, J; Oliveira, A, et al.Background and aims: The histological status of ulcerative colitis [UC] patients in clinical and endoscopic remission has gained space as an important prognostic marker and a key component of disease monitoring. Our main aims were to compare two histological indexes-the continuous Geboes score [GS] and the Robarts Histopathology index [RHI]-regarding their definitions of histological remission and response, and the ability of faecal calprotectin [FC] levels to discriminate between these statuses. Methods: This was an analysis of three prospective cohorts including 422 patients previously enrolled in other studies. Results: The two continuous scores [GS and RHI] were shown to be significantly correlated [correlation coefficient of 0.806, p < 0.001] and particularly close regarding their definition of histological response: 95% and 88% of all patients classified as having/not having [respectively] histological response according to RHI also did so according to GS. Moreover, median FC levels in patients with histological response were lower than those in patients without histological response [GS: 73.00 vs 525.00, p < 0.001; RHI: 73.50 vs 510.00, p < 0.001]; a similar trend was observed when FC levels of patients in histological remission were compared to those of patients with histological activity [GS: 76.00 vs 228.00, p < 0.001; RHI: 73.50 vs 467.00, p < 0.001]. FC levels allowed us to exclude the absence of histological remission [according to RHI] and absence of histological response [according to RHI and GS], with negative predictive values varying from 82% to 96%. However, optimization of the FC cut-off to exclude the absence of histological remission, as for the continuous GS, falls within values that resemble those of the healthy population. Conclusion: The continuous GS and RHI histological scores are strongly correlated in their definitions of histological response. An absence of histological remission could only be excluded at physiological levels of FC.
- Health-Related Quality of Life in Patients with Moderate to Severe Ulcerative Colitis: Surgical Intervention versus Immunomodulatory Therapy.Publication . Portela, F; Ministro, P; Deus, JR; Cravo, M; Cotter, J; Duque, G, e outrosPURPOSE: Ulcerative colitis (UC) can be managed with immunomodulation or surgery. We aimed to understand whether these strategies had a different impact on patients' health-related quality of life (HRQoL). METHODS: This was a retrospective, cross-sectional study: patients who had a moderate to severe UC episode that prompted the utilization of immunomodulatory drugs or surgery were invited to complete a generic (short form [36] health survey [SF-36]) and a disease-specific (inflammatory bowel disease questionnaire [IBDQ]) survey. RESULTS: We included 157 patients, 65 (41.4%) surgically treated. The therapeutic procedure had a minimal impact on HRQoL: only the social dimension of the IBDQ and the physical function component of the SF-36 were significantly different between the study arms - lower for the surgically treated patients. The type of surgery had no impact, but the occurrence of pouchitis, namely, in a chronic form, was associated with a lower HRQoL. Regression analysis confirmed surgery as an independent predictor of lower scores in the social dimension of the IBDQ (-4.646, 95% CI -6.953 to -2.339) and in the physical functioning (-9.622, 95% CI -17.061 to -2.183) and physical role functioning (-3.669, 95% CI -7.339 to 0.001) dimensions of the SF36. CONCLUSIONS: Although usually feared by patients, surgery has a limited impact on UC patients HRQoL when compared to medical management with immunomodulatory drugs.
- Metastatic cutaneous Crohn's disease of the face - a successful case treated with ustekinumabPublication . Figueiredo, L; Oliveira, AM; Martins, A
- C-reactive protein prognostic accuracy in acute pancreatitis: timing of measurement and cutoff points.Publication . Cardoso, F; Ricardo, L; Oliveira, AM; Canena, J; Horta, D; Papoila, A; Deus, JRC-reactive protein (CRP) has been used widely in the early risk assessment of patients with acute pancreatitis. This study evaluated the prognostic accuracy of CRP for severe acute pancreatitis (SAP), pancreatic necrosis (PNec), and in-hospital mortality (IM) in terms of the best timing for CRP measurement and the optimal CRP cutoff points. MATERIALS AND METHODS: This was a single-center retrospective cohort study including 379 patients consecutively admitted with acute pancreatitis. CRP determinations at hospital admission, 24, 48, and 72 h after hospital admission were collected. Discriminative and predictive abilities of CRP for SAP, PNec, and IM were assessed by the area under the receiver-operating characteristic curve and the Hosmer-Lemeshow test, respectively. To determine the optimal CRP cutoff points for SAP, PNec, and IM, the minimum P-value approach was used. RESULTS: In total, 11% of patients had SAP, 20% developed PNec, and 4.2% died. The area under the receiver-operating characteristic curves of CRP at 48 h after hospital admission for SAP, PNec, and IM were 0.81 [95% confidence interval (CI) 0.72-0.90], 0.77 (95% CI 0.68-0.87), and 0.79 (95% CI 0.67-0.91), respectively. The Hosmer-Lemeshow test P-values of CRP at 48 h after hospital admission for SAP, PNec, and IM were 0.82, 0.47, and 0.24, respectively. The optimal CRP at 48 h after hospital admission cutoff points for SAP, PNec, and IM derived were 190, 190, and 170 mg/l, respectively. CONCLUSION: CRP at 48 h after hospital admission showed a good prognostic accuracy for SAP, PNec, and IM, better than CRP measured at any other timing. The optimal CRP at 48 h after hospital admission cutoff points for SAP, PNec, and IM varied from 170 to 190 mg/l.