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Cholangiopathies are poorly understood disorders with no effective therapy. The extrahepatic biliary tree phenotype is less studied compared to the intrahepatic biliary injury in both human disease and Mdr2−/− mice, the established cholestatic mouse model. This study aimed to characterize the extra hepatic biliary tree of Mdr2−/− mice at various ages and to determine if injury can be repaired with the antioxidant and glutathione precursor N-acetyl-L-Cysteine treatment (NAC). We characterized extra hepatic bile ducts (EHBD)s at various ages from 2 to 40 weeks old FVB/N and Mdr2−/− mice. We examined the therapeutic potential of local NAC ex vivo using EHBD explants at early and late stages of injury; and systematic therapy by in vivo oral administration for 3 weeks. EHBD and liver sections were assessed by histology and immunofluorescent stains. Serum liver enzyme activities were analyzed, and liver spatial protein expression analysis was performed. Mdr2−/− mice developed progressive EHBD injury, similar to extrahepatic PSC. NAC treatment of ex vivo EHBD explants led to improved duct morphology. In vivo, oral administration of NAC improved liver fibrosis, and decreased liver enzyme activities. Spatial protein analysis revealed cell-type specific differential response to NAC, collectively indicating a transition from pro-apoptotic into proliferative state. NAC treatment should be further investigated as a potential therapeutic option for human cholangiopathies. [ABSTRACT FROM AUTHOR]

Background: In recent years, Raoultella spp. have attracted clinical attention as a new type of pathogen. The most common of human infection with Raoultella are bacteremia, urinary tract infections, abdominal infections, etc. Abdominal infection is a serious and complex infection problem. However, there have been no systematic reports of abdominal infections caused by Raoultella. The objective of this study was to explore the clinical characteristics of Raoultella abdominal infections and provide a reference for clinical practice. Methods: A review of publications on abdominal infections caused by the genus Raoultella between 2009 and 2024 is carried out. This review studied seven parameters: infection type, number of cases, gender, age, comorbidities, treatment, and outcome, and descriptive statistical methods were used to analyze the results. Results: A total of 40 cases (16 Raoultella ornithinolytica and 24 Raoultella planticola) were analyzed: 20 cases of biliary tract infection, 5 cases of liver infection, and 4 cases of peritonitis. Fever and abdominal pain were the main symptoms, and some patients present with multiple skin flushes, systemic erythema. Of the 40 cases, 92.5% of patients had underlying diseases. Among them, malignant disease, immunodeficiency, and invasive operations increase the risk of infection. On the basis of the drug susceptibility results, the preferred antibiotics are quinolone, third generations of cephalosporins, carbapenems, and aminoglycoside. Last, patients with abdominal infections caused by Raoultella spp. mostly have a good prognosis after early use of sensitive antibiotics. Conclusions: According to existing literature reports, the main type of abdominal infection caused by Raoultella is biliary tract infection, and most patients have other underlying diseases. Malignancy, immune deficiency, and invasive procedures are risk factors for bacterial infections. This review also emphasizes that Raoultella spp. is a rarely found opportunistic pathogen, which can cause a high incidence of healthcare-associated infections after invasive procedures. [ABSTRACT FROM AUTHOR]

Background: Lynch syndrome (LS) is a hereditary cancer syndrome caused by pathogenic germline variants in mismatch repair (MMR) genes, which predisposes to various types of cancers showing deficient MMR (dMMR). Identification of LS probands is crucial to reduce cancer-related deaths in affected families. Although universal screening is recommended for colorectal and endometrial cancers, and age-restricted screening is proposed as an alternative, LS screening covering a broader spectrum of cancer types is needed. In the current study, we elucidated the rate of dMMR tumors and evaluated the outcome of LS screening in young-onset extra-colorectal LS-associated cancers. Methods: Immunohistochemistry for MMR proteins were retrospectively performed in a total of 309 tissue samples of endometrial, non-mucinous ovarian, gastric, urothelial, pancreatic, biliary tract, and adrenal cancers in patients < 50 years of age. Clinicopathological information and the results of genetic testing were obtained from medical charts. Results: There were 24 dMMR tumors (7.8%) including 18 endometrial, three ovarian, two urothelial, and one gastric cancer. Co-occurrence of colorectal cancer and family history of LS-associated cancers was significantly enriched in patients with dMMR tumors. Among the 16 patients with dMMR tumors who were informed of the immunohistochemistry results, five with endometrial and one with urothelial cancer were diagnosed as LS with positive pathogenic variants in MMR genes. Conclusions: We report the outcome of immunohistochemistry for MMR proteins performed in multiple types of young-onset extra-colorectal LS-associated cancers. Our study demonstrates the feasibility of a comprehensive LS screening program incorporating young-onset patients with various types of extra-colorectal LS-associated cancers. [ABSTRACT FROM AUTHOR]

Background: Numerous recent studies have explored the association between metabolic dysfunction‐associated steatotic liver disease (MASLD) and the risk of various extrahepatic cancers. However, the conclusions were inconclusive. The aim of this study was to clarify this relationship by conducting a robust meta‐analysis. Methods: Systematic searches were conducted on PubMed, Embase and Web of Science databases to identify relevant cohort studies published prior to February 2024. Hazard ratios (HRs) and their corresponding 95% confidence intervals (95% CIs) were combined using a random‐effects model in this meta‐analysis. Results: Eighteen cohort studies (approximately 16.7 million participants) were finally included in this meta‐analysis. MASLD was linked to a higher risk of extrahepatic cancers, such as gastric (n = 10, HR = 1.47, 95% CI: 1.07–2.01), colorectal (n = 13, HR = 1.33, 95% CI: 1.16–1.53), pancreatic (n = 8, HR = 1.41, 95% CI: 1.11–1.79), biliary tract (n = 5, HR = 1.27, 95% CI: 1.18–1.37), thyroid (n = 6, HR = 1.46, 95% CI: 1.02–2.09), urinary system (n = 10, HR = 1.45, 95% CI: 1.25–1.69), breast (n = 11, HR = 1.17, 95% CI: 1.08–1.26) and female genital organ cancers (n = 10, HR = 1.36, 95% CI: 1.11–1.66). However, there was no statistically significant association between MASLD and the risk of head and neck (n = 6, HR = 1.03, 95% CI: 99–1.07), oesophageal (n = 9, HR = 1.26, 95% CI: 0.86–1.86), lung (n = 9, HR = 1.01, 95% CI: 0.92–1.10), prostate (n = 9, HR = 1.06, 95% CI: 0.94–1.19) or small intestine cancer (n = 2, HR = 1.75, 95% CI: 1.00–3.06). Conclusions: This latest large‐scale meta‐analysis indicated that MASLD was associated with an increased risk of various extrahepatic cancers, such as gastric, colorectal, pancreatic, biliary duct, thyroid, urinary system, breast, skin and female genital cancers. Further research is needed to investigate the mechanisms underlying these associations. [ABSTRACT FROM AUTHOR]

Background: The frequency of major cancer surgery in the elderly (≥80 years) has increased concomitantly with the rise in average age of the population. We assessed early postoperative mortality following hepato-pancreato-biliary (HPB) and gastrointestinal (GI) procedures for common malignancies stratified by age. Methods: The National Cancer Database (2004-2017) was queried for patients who underwent resection for GI (gastroesophageal and colorectal) or HPB (pancreatic adenocarcinoma, biliary tract, and primary liver) cancers. We compared early postoperative mortality (30 d and 90 d) stratified by age (65-79 vs ≥80 years) and procedure, and compared survival outcomes by age and operative vs nonoperative management. Results: A total of 709,358 patients were included. The 30-day mortality ranged from 1.8% to 5.8% among patients 65-79 years and from 3.2% to 12.4% among patients ≥80 years depending on procedure. The 90-day mortality ranged from 3.6% to 10.6% in patients 65-79 years compared to 8.4%-21.0% among patients ≥80 years. The overall 90-day mortality was 5.2% for patients 65-79 years and 12.0% for patients ≥80 years (P < .001). Age ≥80 was associated with worse survival among operatively managed patients with each upper GI, HPB, and lower GI malignancy relative to younger patients on multivariable analysis. However, operative management of patients ≥80 years was associated with improved survival relative to nonoperative management. Discussion: Elderly patients suffer higher postoperative mortality after major GI and HPB cancer surgery, but operative management is associated with improved survival among patients ≥80 years as compared to nonoperative management. These data are important to contextualize when counseling elderly patients on their treatment options for localized GI and HPB cancers. [ABSTRACT FROM AUTHOR]

Indocyanine green (ICG) is a nontoxic, fluorescent iodide dye with rapid hepatic clearance that has been used in medical applications since the mid-1950s. In abdominal surgery, ICG can be used to identify anatomical structures such as the biliary tract, urinary tract, blood vessels, tissue perfusion on different organs, or anastomosis in the digestive system. The application of ICG during bariatric surgery may be useful in primary and revisional procedures at different steps of the surgery. The purpose of this study is to examine the role of indocyanine green during the SADI-S procedure and to highlight the key aspects of this procedure. We present a case series of 22 patients with morbid obesity who underwent a multidisciplinary evaluation and were selected for the SADI-S procedure via a laparoscopic approach. The ICG dose and timing were as recommended in the International Society for Fluorescence Guided Surgery guidelines. For arteriography, 3 ml (7.5 mg) was used, and the detection time was 30-60 s after intravenous administration. For visualization of the common bile duct, we used 1 ml (2.5 mg) applied intravenously at least 45 min before the procedure. For the leak test, a dilution of 1 ml (2.5 mg) of ICG + 5 ml of methylene blue in 100 cc of sterile water, which was prepared just before the test, was used, and the mixture was passed through an orogastric tube. We expose the surgical steps in which ICG can improve outcomes or prevent complications when performing the SADI-S procedure. Key Points • The role of ICG during SADI-S is described. • The step-by-step results of ICG during the SADI-S procedure are highlighted. • ICG was shown to be safe and useful during bariatric surgery in morbidly obese patients. [ABSTRACT FROM AUTHOR]

Access to accurate statistical data is paramount in the pursuit of effective cancer control activities, including research, policy development, and clinical care. This paper presents a comprehensive statistical report on the incidence, mortality, survival, and treatment of major digestive organ cancers, including those of the esophagus, stomach, colon, rectum, liver, extrahepatic biliary tract, and pancreas, in Japan. We compiled data from the National Cancer Center's "Cancer Information Services" and government "e‐Stat" websites and offered a succinct overview of basic statistics by using tables and graphical presentations. Our findings underscore the critical role of the National Cancer Registry introduced by the Cancer Registry Act of 2016, which mandates hospitals across Japan to report cancer cases. This system ensures more accurate incidence statistics. Mortality data sourced from the National Vital Statistics System and survival rates derived from hospital‐based cancer registries offer insights into the outcomes and efficacy of treatment modalities. These data indicate a downward trend in mortality for stomach and liver cancers and stable or declining rates for other cancers except pancreatic cancer, which has the lowest survival rate. Treatment patterns indicate an increase in endoscopic procedures for esophageal and stomach cancers, with stable treatment approaches for colorectal cancer. This statistical overview aims to improve the understanding and inform research, policy, and clinical decisions in the field of digestive organ cancers. [ABSTRACT FROM AUTHOR]

Background: To potentially lessen injuries and associated complications, fluorescence cholangiography has been suggested as a technique for enhancing the visualization and identification of extrahepatic biliary anatomy. The most popular way to administer indocyanine green (ICG) is intravenously, as there is currently little data on ICG injections directly into the gallbladder. In order to visualize extrahepatic biliary anatomy during laparoscopic cholecystectomy (LC), we compared the two different ICG administration techniques. We also examined variations in visualization time, as well as the effectiveness, benefits, and drawbacks of each modality. Methods: In this prospective randomized clinical study, 60 consecutive adult patients with chronic and acute gallbladder disease were included. Our study conducted from 2022 to 2024 in Surgical Department of Theodor Bilharz Research Institute. Thirty patients underwent LC with intravenous ICG administration (IV-ICG), thirty patients received a direct injection of gallbladder through transhepatic ICG (IC-ICG) and Preoperative, intraoperative, and postoperative patient data were examined. Results: In terms of their perioperative and demographic features, the groups were similar. Without a statistically significant difference, the IV-ICG group's total operating time was less than that of the IC-ICG group (p 0.140). Compared to the transhepatic IC-ICG method, IV-ICG was more accurate in identifying the duodenum and the common hepatic duct (p = 0.029 and p = 0.016, respectively). In the transhepatic IC-ICG and IV-ICG groups, the cystic duct could be identified prior to dissection in 66.6% and 73.3% of cases, respectively, and this increased to 86.6% and 93.3% following dissection. In the transhepatic IC-ICG group, the common bile duct was visible in 93.3% of cases; in the IV-ICG group, it was visible in 90% of cases. Two cases in the IC-ICG group and every case following IV-ICG administration had liver fluorescence (6.6% versus 100%; p < 0.001). Conclusion: The current study shows that for both administration methods, ICG-fluorescence cholangiography can be useful in identifying the extrahepatic biliary anatomy during Calot's triangle dissection. By avoiding hepatic fluorescence, the transhepatic IC-ICG route can increase the bile duct-to-liver contrast with less expense and no risk of hypersensitivity reactions than the intravenous ICG injection method. We recommend to use both techniques in case of acute cholecystitis with cystic duct obstruction. In cases of liver cirrhosis, we recommend transhepatic IC-ICG as IV-ICG is limited. [ABSTRACT FROM AUTHOR]

Background: The anatomy of the biliary tree is complex with anatomical variations which can be found in ~50% of the patients. Purpose: Existing research on these variations primarily stems from studies in developed countries, with uncertain applicability to the Ugandan population due to noted ethnic differences in incidence rates. Objective: The study was aimed at describing the anatomical variations of the gallbladder and bile ducts. Methods: This retrospective cross‐sectional study conducted at Kampala MRI Centre from January 2017 to December 2022 analyzed MRCP images and reports from 231 patients to document gallbladder and bile duct variations. Results: 53.2% of patients exhibited typical cystic duct anatomy, and 51% had Type 1 variations in intrahepatic ducts. Importantly, the study identified a significant correlation between age and common bile duct diameter. Conclusion: The findings showed various anatomical variations that were slightly higher in the study population than those reported in the literature. This study emphasizes the critical need for comprehensive knowledge to enhance surgical safety, minimize iatrogenic trauma, and improve the accuracy of diagnostic imaging and hepatobiliary procedures. [ABSTRACT FROM AUTHOR]

Background: Rhabdomyosarcoma (RMS) is a rare malignant soft tissue sarcoma (STS), accounting for almost 50% of pediatric STSs. Due to its heterogeneity, RMS presents challenges in diagnosis and treatment, with prognosis varying depending on multiple factors. Tumors localized in the other site (OTH)—including the paraspinal, perianal, thoracic, abdominal, pelvic, and perineal regions—are generally classified as unfavorable. This study assesses the clinical features and prognoses of RMS in OTH locations depending on its site of origin. Methods: An explorative analysis of RMS cases from the SEER 17 database 2000–2020 was conducted. Patients of all ages with histologically confirmed RMS as primary malignant disease classified under OTH, were included. OTH was categorized in four granular site classifications. Overall survival (OS) and disease‐specific survival (DSS) were analyzed using Kaplan–Meier estimators. Factors independently influencing survival, including a site classification model presented in this study, were identified through Cox regression analysis. Results: Out of 4168 patients with RMS, 990 cases of RMS with the OTH site met the inclusion criteria. The median age was 16 years. The predominant histological subtypes were embryonal (33.0%) and alveolar (25.5%). Most tumors were ≥ 5 cm (median 9 cm) and located primarily in the pelvic region (41.5%). The 3‐, 5‐, and 10‐year OS rates were 45.4% ± 3.332 (95% CI), 40.7 ± 3.332, and 38.6% ± 3.332, respectively, while DSS rates were 43.3% ± 3.136 (95% CI), 38.3% ± 3.136, and 35.1% ± 3.332. In the multivariate analysis age, histological type, site in a granular categorization, stage, regional lymph node examination, and regional lymph node involvement (pathologically proven) were independently associated with survival. Through both univariate and multivariate analyses, an OTH favorable group could be established. The OTH favorable group consists of the anal region, gallbladder and biliary tract, and breast. Conclusion: RMS in OTH shows significant differences in prognosis, putting the current categorization as unfavorable into question and making a more detailed classification necessary. Furthermore, pathological regional lymph node assessment is specifically in the OTH localization recommended. [ABSTRACT FROM AUTHOR]

Copyright of Shipin Kexue/ Food Science is the property of Food Science Editorial Department and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

ABSTRACT Introduction/Objective Materials and Methods Results Conclusions Biliary brushing cytology (BB) to detect cholangiocarcinoma (CCA) is integral in the surveillance of patients with primary sclerosing cholangitis (PSC). Since reactive changes can mimic carcinoma, indeterminant results are frequent. Fluorescence in situ hybridization (FISH) using the UroVysion probe set has been advocated to enhance the detection of CCA. This study evaluates the performance of FISH for detecting CCA in patients with and without PSC.A query of our pathology database for atypical and suspicious BB with concurrent FISH results was performed from 2014 to 2021. FISH (using UroVysion probe set containing centromere enumeration probes to chromosomes 3, 7, and 17) was positive if at least 5 cells demonstrated polysomy. Electronic medical records were reviewed to identify patients with PSC and CCA. CCA was confirmed by pathology or clinical impression.Of the 65 patients (103 BB) in the PSC cohort, 59 patients (94 BB) without CCA and 6 patients (9 BB) with CCA were identified. 33 non‐PSC patients (41 BB) with CCA were included for comparison. Positive FISH was highest in non‐PSC patients with CCA (10/41 BB, 24%). Positive FISH was seen in both PSC with (1/9 BB, 11%) and without (2/94 BB, 2%) CCA.FISH positivity was lower than expected and was positive in PSC patients without CCA. These results question the clinical utility of FISH for CCA surveillance in PSC patients. [ABSTRACT FROM AUTHOR]

Bilothorax, the accumulation of bile in the pleural space, is an uncommon but serious condition often linked to biliary tract or diaphragmatic injury. This case report describes a 70‐year‐old female with decompensated liver cirrhosis due to primary sclerosing cholangitis, who developed a moderate sized pleural effusion following ERCP and biliary stenting. The patient's pleural effusion persisted for 2 months without respiratory symptoms, indicating a self‐limited low‐volume leak. She eventually underwent thoracentesis for a non‐resolving unilateral effusion, which drained 435 mL of bilious fluid with an elevated pleural fluid bilirubin level, confirming the diagnosis of bilothorax. This case highlights the importance of considering bilothorax as a cause of pleural effusion in patients with biliary tract disease and who undergo high risk procedures including ERCP. [ABSTRACT FROM AUTHOR]

Objective To analyse the biliary pharmacokinetics/pharmacodynamics (PK/PD) of continuous infusion (CI) meropenem-vaborbactam (MEM-VBM) in a case series of orthotopic liver transplant (OLT) recipients being treated for Klebsiella pneumoniae carbapenemase-producing Klebsiella pneumoniae (KPC-Kp) related biliary tract infections (BTIs) or as preemptive therapy of KPC-Kp rectal colonization. Methods Critical OLT recipients receiving CI MEM-VBM (2 g/2 g q8h over 8 h) because of KPC-Kp related BTIs or as preemptive therapy of KPC-Kp rectal colonization, having Kehr's tube positioned and undergoing simultaneous therapeutic drug monitoring of MEM and VBM in plasma and bile were retrospectively assessed. Bile-to-plasma ratio of free steady-state concentrations (f Css) of MEM and VBM was used for assessing biliary penetration. Optimal joint MEM-VBM PK/PD target attainment was defined as MEM f Css/MIC ratio >4 coupled with VBM free area under time–concentration curve (f AUC)/threshold concentration (CT) ratio >24. Results Overall, four critical OLT recipients were included. Median bile-to-plasma ratio was 0.32 for MEM (range 0.21–0.79) and 0.40 for VBM (range 0.20–0.77). Biliary MEM-VBM joint PK/PD target attainment was optimal in 3/4 OLT recipients and quasi-optimal in the other one. Conclusions The 1:1 proportion between MEM and VBM concentrations was maintained unchanged in the bile, allowing us to assume that the efficacy of MEM-VBM may be appropriate even in the treatment of BTIs. CI administration was an effective strategy for attaining aggressive biliary joint PK/PD targets against pathogens with an MIC up to 2 mg/L. [ABSTRACT FROM AUTHOR]

Background: Population-based studies that examine the associations between hyperthyroidism and cancer risk have yielded inconsistent results. It remains unclear whether the risks of different cancers increase in patients with Graves' disease (GD) who received antithyroid drugs (ATDs) as initial treatment. We aimed to determine whether cancer risk increases in patients with GD, compared with controls. Methods: This nationwide retrospective cohort study utilized data from the National Health Information Database of South Korea. We included 29,502 patients aged >20 years with GD, who received ATDs as initial treatment, and 57,173 age- and sex-matched controls. The primary outcome was the incidence of various types of cancers. Hazard ratios (HRs) with confidence intervals (CIs) for cancer risk were estimated using Cox proportional hazards models. We also analyzed HR by follow-up period since the diagnosis of GD, accounting for surveillance effect. Results: The risk of biliary tract and pancreatic cancers (HR: 1.41, CI: 1.24–1.60), thyroid cancer (HR: 15.51, CI: 12.29–19.57), prostate cancer (HR: 1.48, CI: 1.28–1.71), and ovarian cancer (HR: 1.31, CI: 1.13–1.52) was elevated in the GD group than in the control group even after the first year of follow-up was excluded. The increased risk of these cancers persisted after a follow-up period of more than 5 years. The risk of thyroid cancer in patients with GD was higher during the initial follow-up period (1 to <2 years) (HR: 19.35, CI: 7.66–48.87) compared with that in the follow-up period exceeding 2 years. The cancer risk estimates remained significant after excluding patients with GD who underwent subsequent radioactive iodine therapy. Conclusion: In this large-scale population-based study, GD was associated with increased risks of biliary tract and pancreatic, prostate, ovarian, and thyroid cancers. The increased risk of thyroid cancer, particularly during the initial follow-up period, may be a surveillance effect. [ABSTRACT FROM AUTHOR]

This review article discusses the most common benign biliary disorders and the various radiological findings on multiple modalities. A broad spectrum of diseases including various congenital disorders, infective and parasitic etiologies, immunological pathologies such as primary sclerosing cholangitis, and immunoglobulin G4-related sclerosing cholangitis are discussed along with obstructive diseases and ischemic cholangitis. The article emphasized the imaging differential diagnosis of the above lesions as well as clinical correlates those that are most relevant to radiologists. The article briefly touched upon management and intervention where relevant. [ABSTRACT FROM AUTHOR]

Copyright of Pamukkale Medical Journal is the property of Pamukkale Journal of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: This study aimed to investigate the efficacy, long-term prognosis and safety of combining chemotherapy with regorafenib and immune checkpoint inhibitors as first-line treatment for patients with advanced biliary tract carcinoma (BTC). Methods: In this single arm phase II trial, twenty-nine patients with advanced BTC were included, all of whom received gemcitabine-based chemotherapy combined with regorafenib and immune checkpoint inhibitors as the first-line treatment. And the study analyzed anti-tumor efficacy, long-term prognosis, and adverse reactions. Results: Among the patients, 0 patient achieved complete response, 18 patients (62.1%) achieved partial response, 8 patients (27.6%) had stable disease, and 3 patients (10.3%) experienced progressive disease. The corresponding objective response rate (ORR) was 18/29 (62.1%), and the disease control rate (DCR) was 26/29 (89.7%). The median overall survival (OS) was 16.9 months (95% confidence interval [CI]: 12.0 -21.8) and the median progress free survival (PFS) was 10.2 months (95% CI: 7.8-12.6). The 1-year OS and PFS were 65% (95% CI: 0.479-0.864) and 41% (95% CI: 0.234-0.656), respectively. The incidence of adverse reactions was 27/29 (93.1%), and the incidence of grade III/IV adverse reactions was 5/29 (17.2%). Conclusion: The combination of chemotherapy, regorafenib, and immune checkpoint inhibitors as a first-line treatment for patients with advanced BTC may has good anti-tumor efficacy without causing serious adverse reactions, and can significantly improve the long-term prognosis. [ABSTRACT FROM AUTHOR]

Context It is unclear if nonfunctional adrenal tumors (NFAT) are associated with higher cancer incidence. Objective To analyze the cancer incidence in patients with NFAT. Methods In this national register-based retrospective cohort study, consecutive patients with NFAT identified in Sweden 2005-2019 and matched control individuals without adrenal tumors were followed up to 15 years. Outcome data were collected from national registers and adjusted for confounders. Both cases and controls were followed until newly diagnosed malignancy, death, or until 2019. Individuals with adrenal hormonal excess or prior malignancy were excluded. Results Among 17 726 cases, 10 777 (60.8%) were women, and the median age was 65 (IQR, 57-73) years. Among 124 366 controls, 69 514 (55.9%) were women, and the median age was 66 (IQR, 58-73) years. The incidence of any cancer was higher in patients with NFAT compared to controls (hazard ratio [HR] 1.35 95% CI 1.29-1.40; adjusted HR 1.31, 95% CI 1.26-1.37). NFAT was associated with a higher incidence of adrenal, thyroid, lung, stomach and small intestine, kidney, pancreatic, breast, and colorectal cancer. Sensitivity analyses did not change the overall results, but associations were not significantly increased after adjustment in patients with NFAT and appendicitis or gallbladder/biliary tract/pancreas disorders. Cancer incidence may have been underestimated by adjusting for unclear and benign tumors. Conclusion The incidence of cancer was increased in patients with NFAT. Long-term follow-up may be indicated. [ABSTRACT FROM AUTHOR]

We previously demonstrated that in Ifnar1-/-Ifngr1-/- or Stat1-/- suckling mice lacking intact type I and type II interferon (IFN) signaling, rhesus rotavirus (RRV) infection causes a lethal disease with clinical manifestations similar to biliary atresia, including acholic stools, oily fur, growth retardation, and excess mortality. Elevated levels of viral RNA are detected in the bile ducts and liver of diseased pups together with severe inflammatory responses in these tissues. However, the viral determinants and the molecular mechanisms driving this process remain incompletely understood. Using an optimized rotavirus (RV) reverse genetics system, we generated a panel of recombinant RVs that encode non-structural protein 1 (NSP1) derived from different RV strains. We found that compared to the parental simian SA11 strain that is less biliary pathogenic, SA11 containing an RRV-derived NSP1 resulted in severe biliary obstructive disease comparable to that associated with RRV infection, reflected by high levels of viral RNA and inflammation in the biliary tract, liver, and pancreas. In contrast, RRV containing an SA11-originated NSP1 showed only mild biliary obstruction comparable to what was observed during SA11 infection. Infection with a monoreassortant RRV virus carrying NSP1 from the bovine RV UK strain also showed substantially reduced viral replication in extra-intestinal organs and did not develop clinical biliary diseases. Mechanistically, RRV NSP1 seemed to promote active viral replication in hepatocytes and this expanded tropism led to enhanced infiltration of CD4 and CD8 T cells, causing immunopathology and damage in the hepatobiliary system. These results highlight an unexpectedly important role of RV NSP1 in viral replication and disease progression in extra-intestinal tissues. Author summary: In our research, we investigated how specific proteins from the rotavirus, a virus known for causing gastrointestinal infections, influence the severity of liver diseases in a special mouse model. We found that a protein called NSP1, which varies between different strains of the virus, plays a key role in how severe the liver disease becomes. Mice that lacked certain immune system components developed a serious liver condition similar to biliary atresia—a disease that affects the bile ducts and liver. When these mice were infected with a strain of rotavirus carrying NSP1 from another strain, they showed much more severe liver damage compared to those infected with a different strain. Our findings highlight the significant role of NSP1 in promoting viral replication in the liver and bile ducts. This research may help us understand whether and why some rotavirus strains cause more severe liver disease than others and could guide future studies aiming to develop better treatments for such infections. By uncovering these details, we aim to improve our understanding of rotavirus and its impact on the liver, potentially leading to better strategies for managing and treating related diseases. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: A choledochal cyst (CC), or biliary cyst, is a congenital or acquired anomaly of the biliary tree. Pancreas divisum (PD) is a rare congenital anomaly due to incomplete fusion of pancreatic ducts, which can complicate the clinical course of choledochal cysts. This rare combination is a surgical management challenge. This report presents the diagnosis and management of a 23-year-old woman with a combined choledochal cyst and pancreas divisum treated with pancreaticoduodenectomy. Case Report: This article presents the case of a 23-year-old woman who presented with severe, stabbing abdominal pain radiating to the back and epigastric tenderness and was diagnosed with pancreatitis. Initial imaging revealed a choledochal cyst, prompting further investigation with ERCP that showed concomitant PD. She was treated via pancreaticoduodenectomy. During the following 9 years, she was hospitalized over 2 dozen times for recurrent pancreatitis. Conclusions: This report presents a complex case of a combined choledochal cyst and pancreas divisum, which was surgically managed by pancreaticoduodenectomy. The association of CC with PD should be suspected in patients with recurrent acute pancreatitis and/or cholangitis with no identifiable cause. Surgical treatment of CC with PD depends on the classification of the CC, and complications can include recurrent pancreatitis, although the prognosis is often favorable. The purpose of this report is to emphasize that pancreaticoduodenectomy is unlikely to provide favorable outcomes for CC with PD, especially considering there is evidence that less extensive surgical interventions produce better outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Focal autoimmune pancreatitis (AIP) without elevated serum IgG4 levels presents a diagnostic challenge compared to pancreatic tumors, often leading to surgical intervention. Case Presentation: We report a case of serum IgG4-negative focal AIP type 1 in a 52-year-old male. Despite repeated endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) and fine-needle biopsy (FNB), preoperative diagnosis was elusive. Initially, a 30-mm hypoechoic mass in the pancreatic head was detected by ultrasonography, with dynamic computed tomography revealing well-defined borders and homogeneous delayed enhancement. Serum IgG4 levels were within the normal range (115 mg/dL). Two EUS-FNAs and one EUS-FNB failed to provide a definitive diagnosis, leading to suspicion of a solid pseudopapillary neoplasm and subsequent pancreaticoduodenectomy. Postoperative histopathology confirmed focal AIP type 1 with IgG4-positive plasma cells. After 38 months, there is no recurrence, and serum IgG4 levels remain normal. Conclusion: Diagnosis of focal AIP, particularly when serum IgG4 is negative, warrants consideration despite its difficulty. Imaging findings, such as a well-defined mass with homogeneous delayed enhancement, should prompt evaluation for characteristic features like capsule-like rim, pancreatic duct penetration, and biliary tract wall thickening. [ABSTRACT FROM AUTHOR]

Purpose: The prevalence of biliary tract diseases, which are common gastrointestinal disorders, is steadily rising. If it progresses to sepsis or septic shock, it can endanger the patient's life. Therefore, it is crucial to promptly diagnose bacterial infection in individuals suffering from biliary diseases and comprehend the risk factors associated with infection. The objective of this study was to examine the types of bacteria present in the bile of patients with biliary tract diseases, assess any alterations in their susceptibility to antimicrobial agents, and identify the risk factors contributing to the development of infection in these patients. Patients and methods: From June 2019 to November 2022, 317 patients of biliary tract diseases with positive bile culture were included in this hospital-based descriptive analysis. The hospital's computerized medical records were used to collect data on demographic information (including gender, age, and occupation), laboratory, and clinical findings, physical examination results, comorbidities, basic diseases, treatment history, complications, and in-hospital outcomes. The study followed the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) principles. Results: Of the 317 patients with positive biliary tract diseases, 247 had benign diseases and 70 had malignant diseases. Patients with benign disease experienced a higher prevalence of statistically significant symptoms such as abdominal pain (81.4% vs. 57.1%, P = 0.000), nausea (31.2% vs. 14.3%, P = 0.005), vomiting (30.0% vs. 12.9%, P = 0.004), and chills (10.9% vs. 2.9%, P = 0.039), while jaundice (12.6% vs. 37.1%, P = 0.000) was more common in patients with malignant disease. At the species level, Escherichia coli (105; 40.5%), Klebsiella pneumoniae (41; 15.8%), and Pseudomonas aeruginosa (30; 11.6%) were the most commonly found Gram-negative bacterial strains in biliary tract infection. Escherichia coli, Klebsiella pneumoniae, and Pseudomonas aeruginosa were most susceptible to tigecycline, ertapenem and ceftazidime/avibactam, respectively. Conclusion: Gram-negative bacteria are the most commonly isolated biliary bacteria. Clinical doctors should pay attention to patients with malignant diseases with low hemoglobin, high total bilirubin and high alkaline phosphatase. Carbapenems, tigecycline, and minocycline are the recommended antibiotics for Enterobacteriaceae. In recent years, the proportion of enterococcus has gradually increased, and clinical attention should be paid to enterococcus infection. Linezolid and vancomycin were recommended for the treatment of Enterococci infections. Overall, this work can provide reference for clinical diagnosis, treatment and effective interventions. [ABSTRACT FROM AUTHOR]

Aims Methods and results Conclusions Neuroendocrine tumours (NETs) occurring in the extrahepatic biliary system are exceedingly rare. While NETs typically manifest as mass lesions, the occurrence of microscopic neuroendocrine cell proliferation without a distinct mass remains undocumented at this location. This study aims to characterise the clinicopathological features of a series of well‐differentiated neuroendocrine lesions involving the extrahepatic biliary tree, including mass forming NETs and microscopic non‐mass‐forming neuroendocrine cell proliferation, designated neuroendocrine cell micronests (NCMs).Surgical resections of NETs/NCMs involving the extrahepatic bile ducts and gallbladder were identified from electronic pathology databases among seven institutions spanning from January 2011 to September 2023. Clinical and histological findings were recorded. Ten patients (four female, six male: age range = 34–75 years) were included in the study. Histopathological examination revealed visible mass‐forming lesions in four cases (1.6–14.0 cm in size), identified in the gallbladder (n = two) or extrahepatic bile duct (n = two), all diagnosed as well‐differentiated NETs. The remaining six cases revealed incidental non‐mass‐forming NCMs in either the cystic duct (n = two), common bile duct (n = three) or gallbladder (n = one), ranging from < 0.1 to 0.4 cm; four were associated with biliary lithiasis. No evidence of metastasis or recurrence was seen in the follow‐up period (range = 0.1–11.2 years).This study highlights the spectrum of extrahepatic biliary well‐differentiated neuroendocrine lesions, ranging from incidental microscopic NCMs to grossly apparent mass‐forming NETs, potentially requiring different clinical management. Noteworthy is the frequent association of incidental microscopic neuroendocrine cell proliferations with biliary lithiasis, indicating a potential neuroendocrine metaplastic pathogenesis that merits further exploration. [ABSTRACT FROM AUTHOR]

Objective: To evaluate and compare the efficacy and safety of Endoscopic Nasobiliary Drainage (ENBD) and Percutaneous Transhepatic Cholangiography Drainage (PTCD) in patients with advanced Hilar Cholangiocarcinoma (HCCA) through a meta-analysis of clinical studies. Methods: We searched Chinese and English databases, including China National Knowledge Infrastructure (CNKI), Wanfang database, PubMed, Embase, Scopus, and Web of Science, for relevant literatures on PTCD and ENBD for advanced HCCA clinical trials. Two investigators independently screened the literatures, and the quality of the included studies was evaluated using the Newcastle-Ottawa Scale (NOS). The primary endpoint was the success rate of biliary drainage operation, while secondary endpoints included Total Bilirubin (TBIL) change, acute pancreatitis, biliary tract infection, hemobilia, and other complications. R software was used for data analysis. Results: A comprehensive database search, based on predefined inclusion and exclusion criteria, yielded 26 articles for this study. Analysis revealed that PTCD had a significantly higher success rate than ENBD [OR (95% CI) = 2.63 (1.98, 3.49), Z=6.70, P<0.05]. PTCD was also more effective in reducing TBIL levels post-drainage [SMD (95%CI) =-0.13 (-0.23, -0.03), Z=-2.61, P<0.05]. While ENBD demonstrated a lower overall complication rate [OR (95%CI) = 0.60 (0.43, 0.84), Z=-2.99, P<0.05], it was associated with a significantly lower incidence of post-drainage biliary hemorrhage compared to PTCD [OR=3.02, 95%CI: (1.94-4.71), Z= 4.89, P<0.01]. Conclusions: This meta-analysis compares the efficacy and safety of ENBD and PTCD for palliative treatment of advanced HCCA. While both are effective, PTCD showed superiority in achieving successful drainage, reducing TBIL, and lowering the incidence of acute pancreatitis and biliary infections. However, ENBD had a lower risk of post-drainage bleeding. Clinicians should weigh these risks and benefits when choosing between ENBD and PTCD for individual patients. Further research is needed to confirm these findings and explore long-term outcomes. [ABSTRACT FROM AUTHOR]

AbstractBackground Cholangitis is an uncommon and severe adverse reaction of nivolumab with unclear clinical features. The purpose of this study was to investigate the clinicopathological features, imaging and treatment of nivolumab induced cholangitis.Methods Case reports, case series and clinical studies of nivolumab induced cholangitis were retrospectively analyzed by searching Chinese and English databases from January 1, 2017 to December 31, 2023.Results Thirty-eight patients entered the study. The median number of cycles of cholangitis onset was 7 cycles after administration (range 1, 28) and the median time was 11 days (range 78, 390). Abdominal pain (42.1%) and fever (18.4%) were the most important initial symptoms. Some patients (15.8%) showed elevated liver enzymes without any clinical symptoms. The median alkaline phosphatase level was 1721IU/L (range 126, 9118), and the median γ-glutamyltranspeptidase level was 829IU/L (range 104, 3442). Anti-nuclear antibodies, anti-mitochondrial antibodies and IgG4 typically show negative results. Imaging shows extrahepatic bile duct and intrahepatic bile duct dilation, hypertrophy, and stenosis. Liver biopsy and biliary tract biopsy mainly found CD8 inflammatory cell infiltration. Systemic steroids (84.2%) and ursodeoxycholic acid (UDCA) (34.2%) were administered, and 24 patients (63.2%) had poor to moderate response to steroids. Thirty-one patients (81.6%) improved and 7 patients (18.4%) did not improve.Conclusions Clinicians must remain vigilant for patients experiencing cholestasis while on nivolumab and should assess for cholangitis and carry out appropriate imaging tests. Considering the excellent efficacy of UCDA in cholangitis, steroids combined with UDCA may be a viable treatment option in cases where steroids are ineffective for cholangitis. [ABSTRACT FROM AUTHOR]

Inflammatory pseudotumor (IPT) is a rare liver disease confused with liver tumors. It is a disease that should be known in the differential diagnosis for clinicians as the correct diagnosis of IPT will prevent unnecessary surgery. Demographic datas, diagnoses and imaging modalities of six patients with radiologically and/or histopathologically diagnosed hepatic IPT between 2016 and 2023 were retrospectively analyzed. Four out of six patients were female and median age was 57.5 (47-66). C-reactive protein was higher in four patients, and carbohydrate antigen 19.9 level was higher in one patient. We used magnetic resonance imaging (MRI) for diagnosis in five patients. Only in one patient computed tomography was enough for diagnosis. Tumor locations were segment 5 for two patients, segment 7-8 in two patients, segment 7 in one patient, and 8 in one patient. Liver biopsy was performed in five patients because it could not be distinguished from malignancy by imaging methods. Histopathological results of all these biopsies defined as IPT. Initial tumor median size was 31 (17-55) mm. Two patients were operated on. The first one underwent right hepatectomy due to a 2-fold increase in size within 11 months. The second one had a mass indistinguishable from hepatic adenoma by MRI and underwent nonanatomic resection. In one patient, IPT disappeared completely in the 18th month of follow-up period while it regressed in size in two patients. Two of our patients had a history of recurrent endoscopic retrograde cholangiopancreatography, which we noticed incidentally before IPT was diagnosed. IPTs are liver masses with low malignant potential and may shrink spontaneously during follow-up. We suspected that biliary tract interventions may be the cause of IPT. [ABSTRACT FROM AUTHOR]

As a rare biliary tract tumor, intraductal papillary neoplasm of the bile duct (IPNB) is most common in elderly men and can progress to cholangiocarcinoma- (CCa) if left untreated. It is reported that IPNB usually communicates with the bile duct. As a result, the downstream bile ducts are imaged asymmetrically dilated. However, a case of IPNB that we report here is different. Enhanced MRI revealed a lack of connectivity with the bile duct in this case. Based on this, the purpose of this case study is to suggest that the majority of imaging doctors should widely understand the various imaging manifestations of the disease to avoid misdiagnosis. In addition, although this feature was not indicated by ultrasound in this case, given previous studies and considering the convenience and non-ionizing radiation damage of CEUS, we recommend its use as a screening method for IPNB to improve diagnostic accuracy. [ABSTRACT FROM AUTHOR]

Background/Aims: Although endoscopic ultrasound-guided hepaticogastrostomy (EUS-HGS) is performed globally, the procedure remains challenging. Guidewire manipulation is the most difficult step, and there are few reports on the factors associated with unsuccessful guidewire manipulation. This study aimed to assess the significance of the puncture angle on EUS images and identify the most effective guidewire rescue method for patients with unsuccessful guidewire manipulation. Methods: We retrospectively enrolled 115 patients who underwent EUS-HGS between May 2016 and April 2022 at two centers. The puncture angle between the needle and the intrahepatic bile duct was measured through EUS movie records. Results: Guidewire manipulation was unsuccessful in 28 patients. Receiver operating characteristic (ROC) curves identified an optimal puncture angle cutoff value of 85° (cutoff value, 85°; area under the ROC curve, 0.826; sensitivity, 85.7%; specificity, 81.6%). Multivariate analysis demonstrated that a puncture angle <85° was a significant risk factor for unsuccessful guidewire manipulation (odds ratio, 19.8; 95% confidence interval, 6.42-61.5; p<0.001). Among the 28 unsuccessful cases, 24 patients (85.7%) achieved successful guidewire manipulation using various rescue methods. Conclusions: The puncture angle observed on EUS is crucial for guidewire manipulation. A puncture angle of <85° was associated with unsuccessful guidewire manipulation. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Cholecystolithiasis combined with choledocholithiasis represents a prevalent disease. At present, regarding the management of the common bile duct (CBD), T‐tube drainage (TTD) and primary duct closure (PDC) emerge as two prominent approaches for biliary tract repair after laparoscopic CBD exploration (LCBDE). Here, retrospective analysis was conducted on the clinical records of 157 patients who underwent LCBDE at our hospital between January 2019 and January 2022. All patients were categorized into the PDC group or the TTD group based on the chosen CBD treatment approach. A comparative assessment was made across demographic factors, preoperative conditions, surgical particulars, and postoperative complications. The results showed that PDC is recommended for patients with a limited number of small stones, particularly when the CBD is in the 10–15 mm diameter range. [ABSTRACT FROM AUTHOR]

Feline gastrointestinal eosinophilic sclerosing fibroplasia (FGESF) is a rare and poorly understood disease characterised by the infiltration of eosinophils and the development of fibrous tissue within the gastrointestinal tract of cats. A 2‐year‐old female neutered Ragdoll was presented for signs consistent with extrahepatic biliary tract obstruction (EHBO), including jaundice, hyporexia and lethargy. Marked progressive hyperbilirubinemia and mild anaemia were also present. Abdominal ultrasonography suggested a duodenal mass and pancreatitis as the cause of EHBO. Cytopathological results from fine needle aspirates detected mast cells and eosinophils in the duodenal mass and eosinophils in the spleen and the liver, suggestive of a possible mast cell tumour. A cholecystojejunostomy and a duodenotomy were performed to divert the biliary outflow and obtain biopsy samples, respectively. Eosinophilic sclerosing fibroplasia in the duodenal mass and fungal elements in an abdominal lymph node were reported on histopathological examination. A pan‐fungal PCR targeting ITS2 performed on DNA extracted from an abdominal lymph node detected Candida albicans. This report adds to the growing body of evidence that FGESF can occur in association with fungal infections. [ABSTRACT FROM AUTHOR]

Objective: To determine the frequency of bile duct variant using magnetic resonance cholangiopancreatography (MRCP) on a magnetic field strength of 3.0 Tesla in liver donors. Study Design: Descriptive Cross-sectional study. Setting: Department of Radiology, Armed Forces Institute of Radiology and Imaging, Military Hospital Rawalpindi. Period: May 2019 to November 2019. Methods: The research comprised people who went to the hospital for magnetic resonance cholangiopancreatography. 85 liver transplant recipients between the ages of 20 and 70 were chosen for the research. According to Yoshida categorization, bile duct variation was noted. The MRCP methodology includes multiplanar imaging, maximum intensity projection pictures, thin slab axial HASTE, thick slab coronal HASTE, and T2 3D sequence. The Siemens Avanto 3 Tesla machine was used for the study. Results: The study’s participants ranged in age from 20 to 70, with a mean age of 41.73 ± 10.03 years. The majority of the 54 patients (63.53 percent) were aged 20 to 45. The male to female ratio was 1:1 among the 85 patients, with 43 (50.59 percent) men and 42 (49.41 percent) women. In my research of liver donors, 57 (67.05 percent) showed normal biliary tree structure and 28 (32.95 percent) exhibited abnormalities. Conclusion: This study concluded that normal biliary tree anatomy was seen in 67.05% and 32.95% had variations in biliary anatomy on MRCP on 3.0T in liver donors. [ABSTRACT FROM AUTHOR]

Simple Summary: Rhabdomyosarcoma of the extrahepatic and intrahepatic bile ducts is a rare location in children, and it poses a significant challenge for both oncologists and surgeons. Key elements of treatment where there are no clear guidelines include surgical treatment of RMS of the bile ducts, chemotherapy and radiotherapy. The authors wish to share the experience of a highly specialized center that has access to a wide range of therapeutic and treatment options, including the management approach and the outcomes achieved. We review cases treated at our center, discuss treatment approaches and outcomes and compare results with the existing literature and guidelines. Rhabdomyosarcoma (RMS) of the biliary tract is a rare tumor in children, constituting 0.5–0.8% of all pediatric RMS. Still, it is the most common malignancy in this location in children. Due to its rarity and location, it may cause diagnostic and treatment difficulties. Above all, there are no therapeutic guidelines specific for this tumor location. The aim of the study was to present an analysis of our experience with the treatment of children with biliary tract rhabdomyosarcoma (RMS) and discuss clinical recommendations for this specific location published in the literature. A retrospective analysis of medical records of eight children with biliary tree RMS treated in one center between 1996–2022 was performed. Records of eight children, five boys and three girls aged 2 yrs 6 mo to 16 yrs 9 mo (median—6 yrs) were analyzed. All patients presented with jaundice as the first symptom. In two patients, initial diagnosis of a tumor was established. For the remaining six, the primary diagnoses were as follows: choledochal cyst—one, malformation of the biliary ducts—one, choledocholithiasis—one, cholangitis—three. In four patients, the extrahepatic bile ducts were involved; in four patients, both the intrahepatic and extrahepatic bile ducts were involved. Embryonal RMS was diagnosed in seven patients (three botryoides type). Alveolar RMS was found in one patient. Biopsy (three surgical, four during endoscopic retrograde cholangiopancreatography (ERCP)) was performed in seven patients. One child underwent primary partial tumor resection (R2). Seven patients received neoadjuvant chemotherapy, followed by delayed resection in five, including liver transplantation in one (five were R0). Two patients did not undergo surgery. Radiotherapy was administered in four patients (two in first-line treatment, two at relapse/progression). Six patients (75%) are alive with no evidence of disease, with follow-up ranging from 1.2 yrs to 27 yrs (median 11 yrs. and 4 mo.). Two patients died from disease, 2 y 9 mo and 3 y 7 mo from diagnosis. Children presenting with obstructive jaundice should be evaluated for biliary tract RMS. The treatment strategy should include biopsy and preoperative chemotherapy, followed by tumor resection and radiotherapy for residual disease and in case of relapse. [ABSTRACT FROM AUTHOR]

Hepatobiliary mucoepidermoid carcinoma is a rare malignant tumor comprising mucous, intermediate, and epidermoid cells. Herein, we presented a case of primary liver mucoepidermoid carcinoma preoperatively misdiagnosed as conventional intrahepatic cholangiocarcinoma. A 67-year-old male was admitted to our hospital. Preoperative laboratory tests showed increased aspartate transaminase, alanine transaminase, and carbohydrate antigen 19–9. Abdominal Computer Tomography revealed a 4.8 × 4.9 cm liver mass in segment VI. A preliminary diagnosis of intrahepatic cholangiocarcinoma was made, with undergoing partial hepatectomy. However, on histopathology, the tumor comprised a mixture of epidermoid, mucous, and intermediate cells with diffuse infiltrating at the tumor margin. On special stains, mucous and intermedia cells were positive for mucicarmine and Alcian blue, whereas epidermoid cells were positive for Keratin 5/6 and p63. Intermediate cells are also positive for p63. All tumor cells were positive for Keratin 7. The Ki-67 index was 35%. The final diagnosis was primary hepatic mucoepidermoid carcinoma. Although rare, hepatic mucoepidermoid carcinoma should be considered in the intrahepatic cholangiocarcinoma differential diagnosis. We reviewed previous studies and found that hepatobiliary mucoepidermoid carcinoma is more likely to originate from the biliary tract adjacent to the tumor. [ABSTRACT FROM AUTHOR]

Purpose: Although the biliary tract is a common source of invasive infections, the epidemiology of cholangitis- and cholecystitis-associated bloodstream infection (BSI) is not well defined. The objective of this study was to determine the incidence, clinical determinants, microbiology of biliary tract-associated BSI, and predicted adequacy of common empiric therapy regimens. Methods: All biliary tract-associated BSI in Queensland during 2000–2019 were identified using state-wide data sources. Predicted adequacy of empiric antimicrobial therapy was determined according to microbiological susceptibility data. Results: There were 3,698 episodes of biliary tract-associated BSI occurred in 3,433 patients of which 2,147 (58.1%) episodes were due to cholangitis and 1,551 (41.9%) cholecystitis, for age- and sex-standardized incidence rates of 2.7, and 2.0 per 100,000 population, respectively. An increasing incidence of biliary tract-associated BSI was observed over the study that was attributable to an increase in cholangitis cases. There was a significant increased risk for biliary tract-associated BSI observed with advancing age and male sex. Patients with cholangitis were older, more likely to have healthcare associated infection, and have more comorbidities most notably liver disease and malignancies as compared to patients with cholecystitis. The distribution of infecting pathogens was significantly different with polymicrobial aetiologies more commonly observed with cholangitis (18.4% vs. 10.5%; p < 0.001). The combination of ampicillin/gentamicin/metronidazole was predicted to have the overall highest adequacy (96.1%), whereas amoxicillin/clavulanate had the lowest (77.0%). Amoxicillin/clavulanate (75.2% vs. 79.4%, p:0.03) and ceftriaxone/metronidazole (83.4% vs. 89.6%; p < 0.001) showed significantly inferior predicted adequacy for cholangitis as compared to cholecystitis. Conclusions: Bloodstream infections related to cholecystitis and cholangitis exhibit different epidemiology, microbiology, and requirements for empiric therapy. [ABSTRACT FROM AUTHOR]

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Obtaining diagnostic-quality magnetic resonance imaging (MRI) of the abdomen in critically ill patients can be difficult due to challenges with breath-holding and the inability to follow technologist instructions. Protocols that harness advances in commercially available MRI techniques provide a potential solution, particularly using the golden radial angle sparse parallel (GRASP) technique for dynamic post-contrast T1-weighted imaging. The GRASP technique uses a combination of free-breathing, a stack-of-stars radial acquisition, and compressed sensing reconstruction acquired over several minutes to produce motion-free images at time points defined by the user; these include the non-contrast, arterial, venous, and delayed images, which are typical of abdominal MRI protocols. The three cases discussed herein illustrate the use of this technique in providing both exquisite image quality and diagnostic value in the care of critically ill patients with hepatopancreaticobiliary diseases. Our work aims to raise awareness of this technique and its utility in imaging patients who cannot hold their breath for dynamic T1-weighted post-contrast imaging. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis type 2 is a relatively novel entity with some still controversial issues. The current diagnostic algorithm relies on imaging studies and histology. Therapy includes corticosteroids with consequently low risk of relapse in the following year. However, the pathogenesis remains unclear, and data are insufficient for long-term prognosis. We have treated a 17-year-old boy whose autoimmune pancreatitis type 2 was revealed during surgery for a pre-existing biliary tract anomaly with concurrent protozoal infection. We discuss the co-occurrence of these conditions in terms of eventual pathogenesis correlation and combined effect on long-term prognosis. [ABSTRACT FROM AUTHOR]

Congenital anomalies of the biliary tree are rare and present challenges for endoscopists performing interventions such as endoscopic retrograde cholangiopancreatography (ERCP). The reported incidence of ectopic biliary drainage ranges from 2% to 20%, and the condition is often associated with pancreatobiliary complications, including choledocholithiasis (56%), cholangitis (39%), and acute pancreatitis (18.2%). These developmental abnormalities originate during embryogenesis. Uncommon sites for the ampullary opening include the third or fourth part of the duodenum, the stomach, and the pancreatic duct. We report a rare case of ectopic ampulla opening into the pylorus, which presented with cholangitis and was successfully managed with a modified ERCP technique.

Obtaining diagnostic-quality magnetic resonance imaging (MRI) of the abdomen in critically ill patients can be difficult due to challenges with breath-holding and the inability to follow technologist instructions. Protocols that harness advances in commercially available MRI techniques provide a potential solution, particularly using the golden radial angle sparse parallel (GRASP) technique for dynamic post-contrast T1-weighted imaging. The GRASP technique uses a combination of free-breathing, a stack-of-stars radial acquisition, and compressed sensing reconstruction acquired over several minutes to produce motion-free images at time points defined by the user; these include the non-contrast, arterial, venous, and delayed images, which are typical of abdominal MRI protocols. The three cases discussed herein illustrate the use of this technique in providing both exquisite image quality and diagnostic value in the care of critically ill patients with hepatopancreaticobiliary diseases. Our work aims to raise awareness of this technique and its utility in imaging patients who cannot hold their breath for dynamic T1-weighted post-contrast imaging.

Thermal ablation is used to treat liver metastasis including those from breast cancer. The ablation is associated with pain, hemorrhage, and biliary structure damage leading to bilomas. Biloma is a collection of bile that can occur inside or outside the biliary system, which could happen as a rare complication of surgery (from procedures like abdominal surgery or diagnostic procedures), trauma, or spontaneously. We report a case of biloma development after microwave ablation (MWA) of a metastatic lesion in the liver. We present a 66-year-old female diagnosed with stage 4 intraductal carcinoma of the right breast with metastasis to the liver. She developed biloma and infarction of the left lobe of the liver following MWA, which was treated with percutaneous internal/external biliary drain placement. Her symptoms and liver function tests were completely resolved after 3 months, and her left hepatic lobe completely atrophied in the same period. Biloma is a rare but concerning complication of MWA, therefore high suspicion should be maintained in patients presenting with cholestatic symptoms and fever postprocedure. When identified, drainage with antibiotic therapy can effectively treat biloma and resolve the symptoms.

Percutaneous transhepatic biliary drainage (PTBD) is commonly used in the treatment of malign and benign biliary pathologies. Certain complications after PTBD may occur, such as biliary fistula, biliary leakage, bilioma, and hematoma. The purpose of this study was to evaluate the safety and effectiveness of using a sterile gelatin sponge to seal the biliary tract after PTBD in patients with liver transplants to prevent complications. A total of 131 biliary drainages were introduced in 97 patients, and a sterile gelatin sponge was used to seal the biliary tract after removal of the biliary drainage catheter. The patients were immediately examined for complications using ultrasound and then followed up clinically unless imaging was required. Five fluid collections within the liver with a diameter

Cholangiocarcinoma is a hepatobiliary malignancy which can manifest anywhere along the biliary tree. Intrahepatic cholangiocarcinoma occurs in the liver within or beyond the second order bile ducts. The prognosis for patients with intrahepatic cholangiocarcinoma is poor, even when successfully resected there is a very high rate of local recurrence. The available systemic therapies are currently limited and have high rates of toxicity. Percutaneous and transarterial liver-directed therapies can be used to treat intrahepatic cholangiocarcinoma with results comparable to current standard of care systemic therapies in some circumstances. This manuscript will review these the techniques and efficacy of percutaneous and transarterial liver-directed therapies for intrahepatic cholangiocarcinoma. [ABSTRACT FROM AUTHOR]

Background/Aims The optimal length of the uncovered portion of partially covered self-expandable metal stents (PCSEMSs) used in endoscopic ultrasound-guided hepaticogastrostomy (EUS-HGS) remains unclear. This study investigated the safety and efficacy of PCSEMSs with different uncovered lengths, with a focus on stent migration and time to recurrent biliary obstruction (RBO). Methods Outcomes of patients undergoing EUS-HGS using PCSEMSs with 5-mm and 20-mm uncovered portions at our institution from January 2016 to December 2021 were compared. Results Sixty-two patients underwent EUS-HGS using PCSEMS (5/20-mm uncovered portions: 32/30). Stent migration occurred only in the 5-mm group. There were no differences in RBO rates (28.1% vs. 40.0%) or median time to RBO (6.8 vs. 7.1 months) between the two groups. Median overall survival (OS) was longer in the 20-mm group (3.1 vs. 4.9 months, p=0.037) due to the higher number of patients that resumed chemotherapy after EUS-HGS (56.7% vs. 28.1%, p=0.029). Good performance status, absence of hepatic metastases, and chemotherapy after EUS-HGS were independent predictors of longer OS. Conclusions No migration was observed in patients treated with PCSEMS with 20-mm uncovered portions. Patients treated with PCSEMS with 20-mm uncovered portions performed at least as well as those treated with 5-mm uncovered portions in all material respects.

Background: The grim (<10% 5-year) survival rates for pancreatic ductal adenocarcinoma (PDAC) are attributed to its complex intrinsic biology and most often late-stage detection. The overlap of symptoms with benign gastrointestinal conditions in early stage further complicates timely detection. The suboptimal diagnostic performance of carbohydrate antigen (CA) 19–9 and elevation in benign hyperbilirubinaemia undermine its reliability, leaving a notable absence of accurate diagnostic biomarkers. Using a selected patient cohort with benign pancreatic and biliary tract conditions we aimed to develop a data analysis protocol leading to a biomarker signature capable of distinguishing patients with non-specific yet concerning clinical presentations, from those with PDAC. Methods: 539 patient serum samples collected under the Accelerated Diagnosis of neuro Endocrine and Pancreatic TumourS (ADEPTS) study (benign disease controls and PDACs) and the UK Collaborative Trial of Ovarian Cancer Screening (UKCTOCS, healthy controls) were screened using the Olink Oncology II panel, supplemented with five in-house markers. 16 specialized base-learner classifiers were stacked to select and enhance biomarker performances and robustness in blinded samples. Each base-learner was constructed through cross-validation and recursive feature elimination in a discovery set comprising approximately two thirds of the ADEPTS and UKCTOCS samples and contrasted specific diagnosis with PDAC. Results: The signature which was developed using diagnosis-specific ensemble learning demonstrated predictive capabilities outperforming CA19-9, the only biomarker currently accepted by the FDA and the National Comprehensive Cancer Network guidelines for pancreatic cancer, and other individual biomarkers and combinations in both discovery and held-out validation sets. An AUC of 0.98 (95% CI 0.98–0.99) and sensitivity of 0.99 (95% CI 0.98–1) at 90% specificity was achieved with the ensemble method, which was significantly larger than the AUC of 0.79 (95% CI 0.66–0.91) and sensitivity 0.67 (95% CI 0.50–0.83), also at 90% specificity, for CA19-9, in the discovery set (p = 0.0016 and p = 0.00050, respectively). During ensemble signature validation in the held-out set, an AUC of 0.95 (95% CI 0.91–0.99), sensitivity 0.86 (95% CI 0.68–1), was attained compared to an AUC of 0.80 (95% CI 0.66–0.93), sensitivity 0.65 (95% CI 0.48–0.56) at 90% specificity for CA19-9 alone (p = 0.0082 and p = 0.024, respectively). When validated only on the benign disease controls and PDACs collected from ADEPTS, the diagnostic-specific signature achieved an AUC of 0.96 (95% CI 0.92–0.99), sensitivity 0.82 (95% CI 0.64–0.95) at 90% specificity, which was still significantly higher than the performance for CA19-9 taken as a single predictor, AUC of 0.79 (95% CI 0.64–0.93) and sensitivity of 0.18 (95% CI 0.03–0.69) (p = 0.013 and p = 0.0055, respectively). Conclusion: Our ensemble modelling technique outperformed CA19-9, individual biomarkers and indices developed with prevailing algorithms in distinguishing patients with non-specific but concerning symptoms from those with PDAC, with implications for improving its early detection in individuals at risk. Author summary: Pancreatic ductal adenocarcinoma (PDAC) has one of the lowest 5-year survival rates among cancers, primarily due to its complex biology and late-stage diagnosis. Early symptoms often mimic benign gastrointestinal conditions, complicating timely detection. The standard biomarker, carbohydrate antigen (CA) 19–9, is not reliable due to its suboptimal performance and elevation in benign conditions, highlighting the need for better diagnostic tools. In our study, we aimed to develop a biomarker signature to distinguish between benign pancreatic/biliary conditions and PDAC using serum samples from the Accelerated Diagnosis of neuro Endocrine and Pancreatic Tumours and UK Collaborative Trial of Ovarian Cancer Screening studies. We screened 539 patient serum samples with state-of-the-art biomarker panels and developed a robust predictive signature by applying specialized machine learning methods. This new signature significantly outperformed CA19-9 and other panels reported in the literature. Our findings suggest that this new biomarker signature can improve early detection of PDAC in patients with ambiguous clinical symptoms, potentially leading to better outcomes for those at risk. [ABSTRACT FROM AUTHOR]

Cholangiocarcinoma (CCA) is a rare type of digestive tract cancer originating from the epithelial cells of the liver and biliary tract. Current treatment modalities for CCA, such as chemotherapy and radiation therapy, have demonstrated limited efficacy in enhancing survival rates. Despite the revolutionary potential of immunotherapy in cancer management, its application in CCA remains restricted due to the minimal infiltration of immune cells in these tumors, rendering them cold and unresponsive to immune checkpoint inhibitors (ICIs). Cancer cells within cold tumors deploy various mechanisms for evading immune attack, thus impeding clinical management. Recently, combination immunotherapy has become increasingly essential to comprehend the mechanisms underlying cold tumors to enhance a deficient antitumor immune response. Therefore, a thorough understanding of the knowledge on the combination immunotherapy of cold CCA is imperative to leverage the benefits of immunotherapy in treating patients. Moreover, gut microbiota plays an essential role in the immunotherapeutic responses in CCA. In this review, we summarize the current concepts of immunotherapy in CCA and clarify the intricate dynamics within the tumor immune microenvironment (TIME) of CCA. We also delve into the evasion mechanisms employed by CCA tumors against the anti-tumor immune responses. The context of combination immunotherapies in igniting cold tumors of CCA and the critical function of gut microbiota in prompting immune responses have also been annotated. Furthermore, we have proposed future directions in the realm of CCA immunotherapy, aiming to improve the clinical prognosis of CCA patients. [ABSTRACT FROM AUTHOR]

Abnormal findings in the biliary tree are frequently encountered in response to acute and chronic exposures to various compounds. The more common findings are described here in an overview of previous publications such as the INHAND Proliferative and Nonproliferative Lesions of the Rodent Liver and the Liver-Nonneoplastic Lesion Atlas NTP with comments regarding current considerations. This was presented at the 2023 Annual Meeting of the Society of Toxicologic Pathology. Histologic descriptions and some discussions regarding the pathogenesis of the various categories of non-neoplastic lesions in the biliary tree are presented. Discussions regarding the use of the term oval cell versus ductular reaction and the potentially neoplastic nature of cholangiofibrosis are presented in some detail. [ABSTRACT FROM AUTHOR]

Paget's disease of bone is a metabolic bone disorder characterized by localized areas of increased bone production and cycle with excessive and irregular bone formation. Rapid bone resorption and formation lead to irregular bone tissue. Most cases are diagnosed incidentally on radiographs or as an isolated elevation of serum alkaline phosphatase. Primary sclerosing cholangitis is a chronic progressive disorder of unknown etiology characterized by inflammation, fibrosis, and narrowing of medium and large ducts in the intrahepatic and/or extrahepatic biliary tree. The association of both diseases with other diseases has been reported, but there are few reports of the association of these two diseases together. This association can present the physician with an important challenge for both diseases because both diseases lead to an increase in alkaline phosphatase. Here, we present a 52-year-old man who was also diagnosed with Paget's disease of the femur on evaluation for abdominal pain and elevated alkaline phosphatase. Zoledronic acid 5 mg was prescribed for the patient. Six months later, the patient did not complain of hip pain. The femur graph remained unchanged and the patient's alkaline phosphatase decreased and reached the normal range. Laboratory findings, imaging, and histology of the patient were explained. It seems that in patients with high alkaline phosphatase and primary biliary cirrhosis, the presence of pain or deformity of bones and joints should be checked with imaging to detect the presence of co-morbidities. [ABSTRACT FROM AUTHOR]

Background: Gallbladder carcinoma is the most common biliary tract malignancy accounting for 80-95% of all biliary tract cancers. Gallbladder cancer shows geographical and racial variations. Gallstones appear to be an important associated factor for development of gallbladder carcinoma. The purpose of this study is, therefore, to find out, any differences in the incidence of gallbladder cancer in patients with and without gallstones, and any correlation between the number, type and size of gallstones with the incidence of gallbladder cancer. Materials and Methods : It is a hospital based prospective observational study conducted in Assam Medical College for one year. A detailed history and clinical examination followed by radiological investigations specially Ultrasound whole abdomen was done for all the patients. Follow up of the patients was done. Results: The proportion of females was found to be higher (70%) than males. Test of proportion showed that proportion of patients with stone (81.43%) was significantly higher than the patients without stone (18.57%) (p <0.0001). In our study, operative intervention was performed in 30 cases, out of which 11 underwent simple cholecystectomy, 15 underwent extended cholecystectomy and 4 had only laparotomy and biopsy. In our study, 17 of the operated patients (62.96%) had cholesterol stones, 2 patients (7.41%) had pigmented stones and 8 patients (29.63%) had mixed stones. In our study, 59 patients (84.29%) were diagnosed with Biliary Adenocarcinoma NOS. Majority of Adenocarcinoma patients had cholesterol stones (62.50%), followed by mixed stones (29.17%) and pigmented stones (8.33%). Conclusion: A high index of suspicion for the disease and health education are warranted for early diagnosis of gallbladder carcinoma in the North Eastern part of our country, especially Assam, where the incidence is the highest, and, the associated risk factors along with gallstones which makes the region a high-risk zone should be studied extensively. [ABSTRACT FROM AUTHOR]