UNASSIGNED: Biliary ultrasound is often utilized in the evaluation of abdominal pain in the Emergency Department (ED). Common bile duct (CBD) identification is traditionally a standard component of the biliary ultrasound examination but can be challenging to perform for the novice sonographer. Previous work has demonstrated that CBD dilatation is rare in cases of cholecystitis with normal liver function tests (LFTs). We sought to assess the frequency of CBD dilatation in the subset of ED patients undergoing hepatobiliary ultrasound who have normal LFTs and an absence of gallstones or biliary sludge on ultrasound. We also performed an assessment of changes in CBD diameter by age and cholecystectomy status.
UNASSIGNED: This was a retrospective chart review at a single academic ED. Patients were enrolled in the study if they underwent a radiology performed (RP) hepatobiliary ultrasound within the 2 year study period. Records were reviewed for the presence of gallstones or sludge, CBD diameter, age, clinical indication for the ultrasound, and LFTs. Descriptive analyses were performed, and interobserver agreement among data abstractors was assessed by K analysis for the presence of CBD dilatation. The Mann-Whitney test was utilized to assess statistical significance in the comparison of differences between CBD diameters amongst age groups.
UNASSIGNED: Of 1929 RP hepatobiliary ultrasounds performed in the study period, 312 were excluded and 1617 met inclusion criteria. Amongst these, there were 506 patients who had normal LFTs and an ultrasound with no stones or sludge. Ten patients within this group had a dilated CBD > 7 mm (1.98%, 95% CI of 1.08% to 3.6%). We also noted a statistically significant increase in CBD size in the older age cohort and in those individuals with a history of cholecystectomy.
UNASSIGNED: CBD dilation in ED patients who present with normal LFTs and an absence of gallstones and biliary sludge is rare. Physicians should be reassured that the routine identification of the CBD on ultrasound in this setting is of low yield and need not be pursued.
The common bile duct is often taught as part of the biliary point-of-care ultrasound examination. However, it is more challenging to identify than the gallbladder and thus may limit adoption of POCUS by ED physicians. Our study adds to the body of work demonstrating that omitting the common bile duct from an ultrasound evaluation is likely reasonable when both the gallbladder and liver function tests are normal. Our study also adds to the literature regarding the increase in common bile duct size with age and with post-cholecystectomy status.

Background: The cornerstone treatment for primary biliary cholangitis (PBC) is ursodeoxycholic acid (UDCA), but many patients exhibit an incomplete response, leading to disease progression. Risk prediction models like the GLOBE and UK-PBC scores hold promise for patient stratification and management. We aimed to independently assess the predictive accuracy of these risk scores for UDCA response in a prospective U.S. cohort. Methods: We conducted a prospective cohort study at a U.S. liver center, monitoring UDCA-treated PBC patients over a one-year follow-up. We evaluated the predictive efficacy of the GLOBE and UK-PBC scores for UDCA treatment response, comparing them to the Paris II criteria. Efficacy was assessed using univariate and multivariate analyses, followed by prognostic performance evaluation via receiver operating characteristic (ROC) curve analysis. Results: We evaluated 136 PBC patients undergoing UDCA therapy. Based on the Paris II criteria, patients were categorized into UDCA full-response and non-response groups. The GLOBE score identified a non-responder rate of 18% (p = 0.205), compared to 20% (p = 0.014) with the Paris II criteria. Multivariate analysis, adjusted for age and biochemical markers, showed that both the GLOBE and UK-PBC scores were strongly associated with treatment response (p < 0.001). The area under the ROC curve was 0.87 (95% CI 0.83-0.95) for the GLOBE score and 0.94 (95% CI 0.86-0.99) for the UK-PBC risk score. Conclusions: Our study demonstrates that GLOBE and UK-PBC scores effectively predict UDCA treatment response in PBC patients. The early identification of patients at risk of an incomplete response could improve treatment strategies and identify patients who may need second-line therapies.

Hepatobiliary complications of Cystic Fibrosis (CF) constitute a significant burden for persons with CF of all ages, with advanced CF liver disease in particular representing a leading cause of mortality. The causes of the heterogeneity of clinical manifestations, ranging from steatosis to focal biliary cholestasis and biliary strictures, are poorly understood and likely reflect a variety of environmental and disease-modifying factors in the setting of underlying CFTR mutations. This review summarizes the current understanding of the pathophysiology of hepatobiliary manifestations of CF, and discusses emerging disease models and therapeutic approaches that hold promise to impact this important yet incompletely addressed aspect of CF care.

OBJECTIVE: This retrospective analysis aimed to assess the feasibility and safety of endoscopic retrograde cholangiopancreatography (ERCP) in pediatric patients by examining ERCP-related adverse events (AEs) occurring over a decade at a single center.
METHODS: Pediatric patients under 18 years old who underwent ERCP at the Second Hospital of Hebei Medical University from 1/2013 to 11/2023 were included. ERCP-related AEs were defined according to ERCP-related adverse events: European Society of Gastrointestinal Endoscopy (ESGE) Guideline. Clinical data of patients experiencing ERCP-related AEs were obtained from electronic medical records for analysis.
RESULTS: Over the past decade, a total of 76 pediatric patients underwent 113 ERCP procedures, including 26 patients who underwent repeat ERCP, totaling 63 procedures. There were 32 males and 44 females, with a median age of 13 years (range 3 years and 5 months-17 years and 9 months). Among all ERCP procedures, 14 (12.4%) were diagnostic and 99 (87.6%) were therapeutic, with a 100% success rate. 16 cases (14.2%) of ERCP-related AEs, all post-ERCP pancreatitis (PEP), were observed, while no other AEs defined by ESGE such as bleeding, perforation, cholangitis, cholecystitis, or sedation-related events were noted. Additionally, 23 cases (20.4%) of ERCP-related AEs not included in the ESGE definition were observed, including post-ERCP abdominal pain in 20 cases (17.7%), post-ERCP nausea and vomiting in 2 cases (1.8%), and unplanned reoperation in 1 case (0.9%). In the 26 cases of pediatric patients who underwent repeat ERCP, we observed that AEs occurred in 15 cases (57.7%) during their initial ERCP, which was much higher than the overall average level.
CONCLUSIONS: Post-ERCP abdominal pain and PEP are the most common ERCP-related AEs in pediatric patients, while severe AEs such as bleeding and perforation are rare. The incidence of AEs after initial ERCP in pediatric patients who received repeat ERCP is higher than the overall average level. Based on our center\'s experience, we believe that ERCP can be safely performed in children over 3 years old with biliary and pancreatic diseases and obtain reliable clinical benefits. However, active monitoring and management of ERCP-related AEs are essential to improve the clinical outcomes of pediatric ERCP.

BACKGROUND: The detailed mechanisms underlying the development of ischemia-type biliary lesions (ITBLs) in aged donor grafts remain unclear. In the present study we aimed to investigate the impact of aging on the response of the peribiliary gland (PBG) to ischemia-reperfusion injury (IRI) and its temporal changes.
METHODS: Experiments were performed using a 90-min partial warm liver ischemia model in male Wistar rats of two age groups: young (7-8 weeks old) and old (52-60 weeks old). Liver tissues were obtained 24, 72, and 168 h after IRI. Histopathological and immunohistochemical assessments of the perihilar bile duct (PHBD), including the PBG, distal to the clip-clamped site were performed.
RESULTS: Young rats showed little change in the bile duct tissues after IRI. However, old rats showed an increased PBG volume in the PHBD and marked PBG cell proliferation 24 h after IRI. Bile duct wall thickening with narrowing of the lumen peaked 72 h after IRI. Mucus production and oxidative stress in the PBG were significantly higher in old than in young rats after IRI. These findings showed a trend toward improvement 168 h after IRI.
CONCLUSIONS: Age-dependent differences in the response of the PBG to IRI may be related to differences in ITBL frequency.

Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) stand as distinct diseases, yet occasionally intertwine with overlapping features, posing diagnostic and management challenges. This recognition traces back to the 1970s, with initial case reports highlighting this complexity. Diagnostic scoring systems like IAIHG and simplified criteria for AIH were introduced but are inherently limited in diagnosing variant syndromes. The so-called Paris criteria offer a diagnostic framework with high sensitivity and specificity for variant syndromes, although disagreements among international guidelines persist. Histological findings in AIH and PBC may exhibit overlapping features, rendering histology alone inadequate for a definitive diagnosis. Autoantibody profiles could be helpful, but similarly cannot be considered alone to reach a solid and consistent evaluation. Treatment strategies vary based on the predominant features observed. Individuals with overlapping characteristics favoring AIH ideally benefit from corticosteroids, while patients primarily manifesting PBC features should initially receive treatment with choleretic drugs like ursodeoxycholic acid (UDCA).

Cholangiocarcinoma is an aggressive malignancy involving the epithelial cells of the intrahepatic, perihilar, or extrahepatic biliary tree. It is a disease that is often diagnosed late in its course and progresses quickly. Identifying genomic mutations may provide an important utility in predicting disease course and individualizing therapy for these patients. Mutations in BRCA1 or BCRCA2 genes have been increasingly documented in hepatobiliary malignancies, but they remain a relatively uncommon occurrence. Co-mutations in both BRCA1 and BRCA2 genes are even rarer, with no previously documented reports to our knowledge of BRCA co-positivity in a patient with a hepatobiliary malignancy. We present a case of a patient with cholangiocarcinoma found to have mutations in both BRCA1 and BRCA2 genes.

BACKGROUND: Patients with low socioeconomic status (SES) are disadvantaged in terms of access to health care. A novel metric for SES is the Distressed Communities Index (DCI). This study evaluates the effect of DCI on hospital choice and distance traveled for surgery.
METHODS: A Florida database was queried for patients with symptomatic cholelithiasis or chronic cholecystitis who underwent an outpatient cholecystectomy between 2016 and 2019. Patients\' DCI was compared with hospital ratings, comorbidities, Charlson Comorbidity Index, and distance traveled for surgery. Stepwise logistic regression was used to determine which factors most influenced distance traveled for surgery.
RESULTS: There were 54,649 cases-81 open, 52,488 laparoscopic, and 2,080 robotic. There was no difference between surgical approach and patient\'s DCI group (p = 0.12). Rural patients traveled the farthest for surgery (avg 21.29 miles); urban patients traveled the least (avg 5.84 miles). Patients from distressed areas more often had surgery at one- or two-star hospitals than prosperous patients (61% vs 36.3%). Regression indicated distressed or at-risk areas predicted further travel for rural/small-town patients, while higher hospital ratings predicted further travel for suburban/urban patients.
CONCLUSIONS: Compared to prosperous areas, patients from distressed areas have surgery at lower-rated hospitals, travel further if they live in rural/small-town areas, but travel less if they live in suburban areas. We postulate that farther travel in rural areas may be explained by a lack of health care resources in poor, rural areas, while traveling less in suburban areas may be explained by personal lack of resources for patients with low SES.

UNASSIGNED: The efficacy of uncovered self-expandable metal stents (UCSEMS) versus fully covered self-expandable metal stents for distal malignant biliary obstruction remains controversial. Additionally, the heterogeneity of the disease conditions has been indicated in previous studies because pancreatic and non-pancreatic cancers have different characteristics in clinical course. Therefore, the etiology of biliary obstruction necessitates investigations stratified by primary disease. This study aimed to evaluate the outcomes of UCSEMS, specifically for non-pancreatic cancer-induced distal malignant biliary obstruction.
UNASSIGNED: We conducted a single-center retrospective review to evaluate the time to recurrent biliary obstruction and frequency of adverse events (AEs) in patients receiving UCSEMS for unresectable non-pancreatic cancer-induced malignant biliary obstruction.
UNASSIGNED: Overall, 32 patients were enrolled in the study between January 2016 and December 2023. The median time to recurrent biliary obstruction was 140 days. AE rates were low at 3.1% for both pancreatitis and cholecystitis, suggesting a potential benefit of UCSEMS in reducing post-procedural AEs.
UNASSIGNED: UCSEMS may reduce the risk of post-procedural AEs and should be considered in patients at high risk of post-endoscopic retrograde cholangiopancreatography pancreatitis. However, the patency period may be shorter, necessitating future comparative research with fully covered self-expandable metal stents to determine the optimal stent choice.

E. innesii is a recently described Enterococcus species which may be difficult to differentiate from the more common E. casseliflavus. We present the first clinical report of invasive E. innesii infection, featuring two cases of biliary sepsis. Whole genome sequencing confirmed the taxonomic assignment and the presence of vanC-4. Analysis of public genomes identified 13 deposited E. innesii and 13 deposited E. casselifalvus/E.gallinarum genomes which could be reassigned as E. innesii. Improved laboratory diagnosis of E. innesii is expected to generate additional data concerning its clinical relevance and support the future diagnosis and treatment of this uncommon pathogen.