Introduction: Segmental cholangiectasia is a rare cause of focal intrahepatic bile duct dilatation that can mimic congenital, inflammatory or neoplastic conditions, posing diagnostic challenges. Case Presentation: We describe a 58-year-old man with a longstanding cystic lesion in hepatic segment 8 who developed multiple cholangitic episodes. Despite exclusion of infectious, autoimmune and overt neoplastic disease, an elevated carbohydrate antigen 19-9 (CA 19-9) raised concern for intraductal papillary neoplasm of the bile duct (IPNB) and prompted anterior hepatic sectorectomy. Histopathology confirmed segmental cholangiectasia, showing ductal dilatation, bile stasis, cholesterol clefts, fibrosis, and mixed inflammation, without evidence of malignancy or IgG4-related disease. Discussion and Conclusion: Segmental cholangiectasia is a benign yet clinically significant entity that can mimic biliary tumors and prompt major surgery. This case, involving hepatic segment 8, expands the known anatomical spectrum and underscores the importance of multidisciplinary evaluation. Awareness of this condition may prevent misdiagnosis and guide appropriate management, while further studies are needed to better characterize its presentation in Western populations.

Introduction: Liver fibrosis is a key determinant of morbidity and mortality in chronic liver disease. Noninvasive scores such as Fibrosis-4 index (FIB-4) and AST to Platelet Ratio Index (APRI) are widely used, but their accuracy varies across populations. Fibrosis-6 score (FIB-6), a novel composite biomarker incorporating age, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, platelet count, and albumin improves fibrosis prediction. This study evaluated the diagnostic performance of FIB-6 in a Portuguese cohort with steatotic liver disease. Methods: This observational study analyzed 301 adult patients who underwent transient elastography. Demographic, clinical, and laboratory data were retrospectively collected. Noninvasive scores (FIB-4, APRI, and FIB-6) were calculated, and liver fibrosis (F0–F4) was assessed based on liver stiffness measure adjusted for liver disease etiology. Receiver operating characteristic (ROC) analyses, correlations with liver stiffness, Youden index–derived cutoffs, and rule-in/rule-out thresholds were subsequently performed. A subanalysis was also conducted for liver disease etiologies. Results: FIB-6 correlated moderately with liver stiffness (r=0.334; p<0.001), significantly outperforming FIB-4 and APRI, which showed weak or nonsignificant correlations. Area under the ROC Curve values for cirrhosis (F4) were 0.716 (FIB-6), 0.579 (APRI), and 0.513 (FIB-4); for advanced fibrosis (F3–F4), 0.709 (FIB-6), 0.568 (APRI), and 0.545 (FIB-4). Optimal Youden cutoffs for FIB-6 were >2.06 for advanced fibrosis and >1.69 for cirrhosis. A rule-in threshold of >2.30 achieved 90% specificity, while a rule-out threshold of <1.5 reached 90% sensitivity, for advanced fibrosis. FIB-6 outperformed FIB-4 and APRI across Metabolic Dysfunction–Associated Steatotic Liver Disease, Metabolic and Alcohol-Related Steatotic Liver Disease and Alcohol-Related Liver Disease. Discussion: FIB-6 demonstrated superior diagnostic performance compared to FIB-4 and APRI, showing greater accuracy in identifying advanced fibrosis and cirrhosis. Cut-off values varied across different etiologies, underscoring the importance of etiology-specific thresholds for optimal clinical application. Despite its robustness, laboratory-based scores may be influenced by physiological or clinical variations, requiring contextual interpretation. FIB-6 is a reliable and accessible noninvasive tool. Its application within a tripartite “rule-in/rule-out” framework can effectively guide clinical triage and prioritize patients for elastography, particularly in resource-limited settings.

Introduction: Malignant small bowel neoplasms are rare entities, and knowledge about them remains limited due to their histological diversity and the challenges associated with their investigation. However, their rising incidence has generated increasing clinical and research interest. This study aimed to describe the demographic and clinical characteristics of patients with malignant neoplasms of small bowel and the evolution in their diagnosis over 20 years in a tertiary center. Methods: Single-center retrospective study of data of patients with malignant small bowel neoplasms diagnosed between 2001 and 2020 in a tertiary hospital was performed. Statistical analysis was performed with SPSS version 29.0 (significance level ≤0.05). Results: Out of 135 patients included, 57% were male. Eighty-nine neoplasms (65.9%) were found in the jejunum/ileum. Adenocarcinomas were the most frequently diagnosed neoplasms (31.1%), followed by neuroendocrine tumors (28.1%). At the time of diagnosis, the majority of patients (80.7%) were symptomatic, with severe complications – including obstruction, hemorrhage, or perforation – occurring in 55.4% of cases. Diagnosis typically involved CT scan (40.7%) or upper digestive endoscopy (22.2%); notably, 22.2% patients still required surgery for diagnosis. Diagnoses were mainly made between 2011 and 2020 (65.9%). Between 2001 and 2010, the most common diagnosed tumors were adenocarcinomas (37.0%), whereas between 2011 and 2020, the most frequently diagnosed malignant neoplasms were neuroendocrine tumors (37.1%). The distribution of histological types differed significantly over the years (p = 0.016). Adenocarcinoma had a higher mortality rate (54.8%) compared to neuroendocrine tumors (7.9%). Discussion/Conclusion: Given the rarity of these tumors, the cohort of malignant small bowel neoplasms collected at this tertiary center over a 20-year period represents a substantial sample. More than half of patients were symptomatic at diagnosis, despite diagnostic advances over the years. In the last 10 years of the study, there has been an increase in incidence as well as in 5-year survival rates, possibly due to the higher incidence of neuroendocrine tumors and the lower incidence of adenocarcinomas. Notably, the diagnosis of each histological neoplasm type differed significantly statistically over the years, with neuroendocrine tumors being the most diagnosed in recent years.

Introduction: Ascites, the abnormal accumulation of fluid within the peritoneal cavity, is commonly associated with decompensated liver cirrhosis, primary or secondary malignancy, right-sided heart failure, and peritoneal tuberculosis. The underlying pathophysiology is usually related to one of portal hypertension, hypoalbuminaemia, or peritoneal inflammation. When these common causes are excluded, autoimmune aetiologies such as systemic lupus erythematosus (SLE) should be considered, albeit being rare. SLE is a chronic, multisystem autoimmune disease with diverse manifestations, and serositis is a recognized but variably expressed feature. Lupus peritonitis, a form of serosal inflammation leading to ascites, occurs infrequently and is often overshadowed by more typical pulmonary or pericardial involvement. We report an unusual case of massive painless ascites as the presenting feature of SLE in a middle-aged man, underscoring the diagnostic challenges of this rare presentation. Case Presentation: A 43-year-old man presented to the Emergency Department with a 2-week history of painless, progressive abdominal distension. He denied any associated symptoms and a thorough physical examination was unremarkable except for ascites. An extensive diagnostic panel followed to identify the cause of ascites, as the hepatobiliary system appeared normal on imaging and serum ascites albumin gradient was less than 1.1, making cirrhosis and portal hypertension unlikely. Echocardiogram and thyroid function tests were normal. His autoimmune serological markers revealed the presence of anti-Smith and anti-RNP antibodies, and the patient fulfilled the 2019 EULAR/ACR classification for SLE. Discussion: Ascites as the initial presentation of SLE is uncommon, especially massive ascites. Lupus peritonitis, the underlying mechanism, is thought to result from immune complex deposition and complement activation leading to peritoneal inflammation and exudative effusion. In this patient, the absence of typical features such as rash, arthritis, or haematological abnormalities initially obscured the diagnosis. This case illustrates how SLE can present insidiously and emphasizes the need for thorough autoimmune evaluation in unexplained exudative ascites once hepatic, cardiac, malignant, and infectious causes have been excluded. The rapid response to corticosteroid therapy underscores the importance of early recognition and immunosuppressive treatment to prevent complications.

Background: Cirrhosis is associated with significant morbidity and mortality. Recent studies have highlighted the potential role of allopurinol in reducing the incidence of hepatic decompensation among individuals with cirrhosis. This study aimed to evaluate the association of allopurinol use with the incidence of hepatic decompensation and overall mortality in patients with cirrhosis in a large, propensity-matched cohort. Methods: A retrospective cohort study of adults with cirrhosis was conducted using the national TriNetX database, with 1:1 propensity score matching. Allopurinol exposure was assessed in three categories compared to individuals with no allopurinol use: 100 mg, 300 mg, and exposure at any dose. The primary outcome was the incidence of overall hepatic decompensation. Secondary outcomes included the incidence of ascites, esophageal variceal bleeding, hepatic encephalopathy (HE), hepatorenal syndrome, spontaneous bacterial peritonitis (SBP), hepatocellular carcinoma, and overall mortality. The outcomes were assessed at 6, 12, and 18 months. Odds ratios (ORs) with 95% confidence intervals (CIs) were calculated. Results: After propensity score matching, 5,358 patients who received allopurinol were compared with 5,358 controls. Dose-specific analyses included two additional matched cohorts: 2,124 patients receiving 100 mg of allopurinol and their matched controls, and 1,020 patients receiving 300 mg of allopurinol and their matched controls. At 18 months, a statistically significantly lower incidence of hepatic decompensation was seen in the overall allopurinol exposure cohort (OR: 0.77; 95% CI: 0.70–0.84), the 100 mg cohort (OR: 0.66; 95% CI: 0.57 to 0.76), and 300 mg cohort (OR: 0.76; 95% CI: 0.62 to 0.94). Allopurinol exposure was associated with a decreased incidence of esophageal variceal bleeding (OR: 0.71; 95% CI: 0.55 to 0.92), ascites (OR: 0.77; 95% CI: 0.69 to 0.84), HE (OR: 0.76; 95% CI: 0.63 to 0.92), SBP (OR: 0.61; 95% CI: 0.46 to 0.80), and overall death (OR: 0.86; 95% CI: 0.77 to 0.96) compared to the control group. Conclusion: In a propensity score-matched analysis of a large national database, individuals with cirrhosis and allopurinol use had significantly lower risk of hepatic decompensation and overall mortality. These findings suggest that allopurinol may play a potential role in managing cirrhosis, and randomized clinical trials are needed to confirm these findings.

Background: Full-thickness gastrointestinal tract defects (FGTDs) have been an unsettling occurrence in clinical practice, with an increasing incidence due to the growing use of third-space endoscopy and surgical procedures. With advancing technology, many of these events are now being managed endoscopically in a minimally invasive manner, and surgery is being increasingly reserved for refractory cases. Available devices for their treatment include through-the-scope clips, over-the-scope clips, endoscopic suturing devices, self-expandable metal stents, and endoscopic vacuum therapy. Summary: In this review, we present available literature on different techniques for endoscopic defect closure and discuss their indications, efficacy, and limitations. Key Messages: There is a scarcity of guidelines regarding FGTD endoscopic treatment. Selection of devices is still largely dependent on endoscopist’s preference, while considering defect size, chronicity, characteristics of adjacent mucosa, and device availability.

Introduction: Desmoid tumors invading the pancreas are rare. Preoperative diagnosis is uncommon either because a more common malignant lesion is suspected in a symptomatic patient that is submitted to surgery without tissue acquisition or biopsies are inconclusive given the variable cellularity of specimens obtained and difficult differential diagnosis. Only one case of successful diagnosis by endoscopic ultrasound-guided biopsies has been reported to date. Case Presentation: A 30-year-old male patient, followed for familial adenomatous polyposis with a past medical history of prophylactic proctocolectomy, presented in a routine abdominal ultrasound unilateral calyceal dilation. Further imaging studies described a 65 mm nodular, infiltrative, hypodense, neoplastic lesion, centered at the distal body and tail of the pancreas, enveloping the splenic vein, and a second similar lesion near the right common iliac vessels. The patient was asymptomatic and laboratory results were normal. The pancreatic mass was biopsied guided by endoscopic ultrasound, and cythopathological analysis concluded that desmoid fibromatosis was the most likely diagnosis. Three weeks post-biopsy, the patient had a rapid and severe clinical progression, with acute abdominal pain, hemorrhagic shock and active bleeding on imaging, demanding emergent exploratory laparotomy and extensive resection of the tumor, which involved the pancreatic tail and invaded adjacent structures. Histopathological analysis of the surgical specimen confirmed the preoperative diagnosis of desmoid fibromatosis. Given the R1 resection and the presence of a second large intra-abdominal lesion, the patient started systemic therapy with vinorelbine. Exocrine pancreatic insufficiency was diagnosed after surgery, with need of enzymatic replacement therapy. Conclusion: Endoscopic ultrasound-guided biopsies can play an important role in the diagnosis of pancreatic desmoid tumors, allowing for nonsurgical diagnostic work-up as surgical resection is no longer considered the first-line therapy.

Introduction: Hepatocellular injury is characterized by elevated levels of aspartate aminotransferase (AST) and alanine aminotransferase (ALT). In contrast, alkaline phosphatase levels are usually elevated in cholestatic diseases. Due to the multiples causes, it is crucial to define the etiology for proper management. The most common diagnoses are viral hepatitis, autoimmune hepatitis, Wilson’s disease, drug-induced liver injury, acute alcoholic hepatitis, and ischemic hepatitis. Extrahepatic causes such as endocrine and cardiovascular conditions should also be considered. Case Presentation: A 46-year-old male presented with a 2-month history of elevated transaminase levels that progressed to jaundice and dark urine (choluria) in the preceding 30 days. During the investigation, it was discovered that the hepatocellular injury was caused by hypoperfusion secondary to a retroperitoneal catecholamine-secreting sympathetic paraganglioma, in which the patient achieved a favorable outcome with complete preservation of liver function. Conclusions: This case highlights the importance of considering rare causes of hepatocellular injury.