Duct Involvement Research Articles

Longitudinal Assessment of Bile Duct Loss in Primary Biliary Cholangitis.

Bile duct involvement is a key finding of primary biliary cholangitis (PBC). The aim of this study was to evaluate baseline ductopenia and disease progression. Retrospective longitudinal histological follow-up of treatment-naive patients with PBC. Eighty-three patients were included, with ductopenia correlated to fibrosis stage at baseline. The cumulative incidence of severe ductopenia remained stable after 5 years, whereas fibrosis continually increased over time. Baseline AST-to-Platelet Ratio Index and elevated alkaline phosphatase >2 times the normal with abnormal bilirubin were associated with ductopenia progression. Bile duct injury does not seem to follow the same course as fibrosis in PBC.

Important CT Findings for Prediction of Incomplete (R1 or R2) Resection and Poor Survival of Perihilar Cholangiocarcinoma after Curative-Intent Surgery

Purpose: To evaluate CT findings to predict incomplete (R1 or R2) resection and poor survival in patients with perihilar cholangiocarcinoma using pre-operative CT.Materials and Methods: From 2006 to 2012, a total of 139 patients with perihilar cholangiocarcinoma who underwent pre-operative multiphase CT and subsequent curative-intent surgery were included. After two radiologists independently reviewed CT findings including the likelihood of bile duct (BD) involvement from intrapancreatic common bile duct (CBD) to bilateral second-order branches and peritumoral fat stranding using a 5-point scale, vessel involvement (no, abutment, encasement), and LN involvement, imaging findings were finalized by a consensus of two radiologists. When the likelihood scale of 4 or more on preoperative CT was regarded as BD involvement, the diagnostic ability of CT was analyzed by the receiver operating curve using histopathologic results as a reference standard. Residual tumor categorized into no residual tumor (R0) and residual tumor (R+; R1 or R2). Predictive factors of R+ resection on pre-operative CT were analyzed by logistic regression. Cox proportional hazard model was used to determine the prognostic factor for overall survival by using pre-operative CT findings and laboratory results.Results: Seventy-one patients were R0 and sixty-eight patients were R+ resection. For resectability evaluation, mid-CBD involvement (score ≥ 4) in pre-operative CT was significant factor for R+ resection in multivariable analysis (<i>P</i> < 0.01) with substantial interobserver agreement. In multivariable Cox regression, intrapancreatic CBD involvement (score ≥ 4, hazard ratio [HR] = 1.81, <i>P</i> < 0.01) as well as elevated total bilirubin (HR = 1.53, <i>P</i> = 0.04) and CA 19-9 level (HR = 1.75, <i>P</i> < 0.01) were significant predictors for poor survival. Diagnostic ability to predict mid-CBD and intrapancreatic CBD involvement on pre-operative CT were 0.71 and 0.72 (AUC values).Conclusions: Distal longitudinal extent of perihilar cancer on pre-operative CT is a significant factor for margin positive resection and poor survival on curative-intent surgery.

Histopathological evaluation of minor salivary gland aberrations in oral squamous cell carcinoma.

Oral squamous cell carcinoma (OSCC) is one of the most established oral cancers in India, with high morbidity and mortality. The most common etiological agent associated with it is tobacco (in any form), which releases chemical carcinogens that affect not only the oral epithelial lining but also deep stromal structures such as minor salivary glands. They may cause changes in ductal or acinar part of gland depending on tumor grade, thus providing a fertile soil for tumor growth and recurrence. To observe the frequency of minor salivary gland changes associated with tobacco as well as to measure the length and depth of ductal involvement in routine tissue sections of OED and OSCC. A total of 94 hematoxylin and eosinstained archival slides which included cases of well, moderate, and poorly differentiated OSCC and oral epithelial dysplasia were histopathologically evaluated to observe changes in different components of minor salivary gland. Ductal hyperplasia, ductal metaplasia, mucous pooling within duct, acinar degeneration, pattern of malignant cell invasion (single/clusters), inflammatory infiltrate, eosinophilic cuffing around the gland, and glandular/vascular involvement were evaluated in each slide and correlated with different grades of OSCC. Ductal hyperplasia, inflammatory cell infiltrate, mucous pooling, and pattern of malignant cell infiltration came out to be statistically significant with the highest percentage of changes being observed in poorly differentiated squamous cell carcinoma > moderately differentiated squamous cell carcinoma> well differentiated squamous cell carcinoma>oral epithelial dysplasia. Further, the results of this study suggest that extension of dysplasia or squamous cell carcinoma from overlying oral epithelium along salivary gland ducts is an uncommon finding. Conclusion: Hence, histopathological interpretation for OED and OSCC should also include changes related to associated minor salivary gland tissue as detection and eradication of the putative precursors are the best way of decreasing the overall morbidity caused by tumors.

ISOLATED PANCREATIC INJURIES: A CASE SERIES

Context: Pancreatic injuries constitutes a set of rare abdominal solid organ injuries. Isolated pancreatic injuries are even more rare injuries and are often missed in initial clinical and radiological evaluation . such injuries present late and denitive guidelines to manage such injuries are lacking, considering rarity of such injuries. Unpredictable course involves traumatic pancreatitis, pancreatic necrosis, peripancreatic collections, infective complications and extra abdominal complication The aim of this study is to present clinical details and management outcomes of such casesAims: at our institute This study is a retrospective analytical study In this retrospective study medicalSettings and Design: Methods and Material: records of all the admissions were collected from trauma registry from may 2016 to april 2022 at Thanjavur medical college. All these records were screened for patients with isolated pancreatic injuries All continuous variables were analyzed with descriptive statisticsStatistical analysis used: and their mean, median, mode, range and frequencies were calculated. All categorical data are analyzed with frequencies. Comparison of data is done using: all nominal data is analyzed with parametric tests (Chi-square test, unpaired t test) and non-nominal data is analyzed with non- parametric tests (Fischer's exact test and Mann-whitney U test). In our study a total of 22 cases were included. Mean age being 33.91yearsResults: among which 81.8% were males. 72.7% of the patients suffered road trafc accidents. 77.3% of the patients had BMI less than 25. Main pancreatic duct (MPD) involvement was noticed in 22.7% patients. median levels of serum amylase levels were 390U/L. 22.7% patients underwent operative management were as rest were managed non-operatively. Two patients died due to severe pancreatitis. Isolated pancreatic injuries inConclusions: patients where surgery is otherwise not indicated due to other causes needs management tailored to the clinical needs of the patient. Diagnostic ambiguity is best resolved by combining computed tomography (CT) and Magnetic resonance cholangiopancreatography (MRCP). Patients with distal MPD injury are usually operated when presented early and pancreatic stenting is considered when proximal ductal injury is noticed. In selected cases conservative approach is not a contraindication, even in patients with MPD injury, considering high stula and peripancreatic collection rates in distal pancreatectomy.

Morphomolecular Classification Update on Hepatocellular Adenoma, Hepatocellular Carcinoma, and Intrahepatic Cholangiocarcinoma

Hepatocellular adenomas (HCAs), hepatocellular carcinomas (HCCs), and intrahepatic cholangiocarcinomas (iCCAs) are a highly heterogeneous group of liver tumors with diverse pathomolecular features and prognoses. High-throughput gene sequencing techniques have allowed discovery of distinct genetic and molecular underpinnings of these tumors and identified distinct subtypes that demonstrate varied clinicobiologic behaviors, imaging findings, and complications. The combination of histopathologic findings and molecular profiling form the basis for the morphomolecular classification of liver tumors. Distinct HCA subtypes with characteristic imaging findings and complications include HNF1A-inactivated, inflammatory, β-catenin-activated, β-catenin-activated inflammatory, and sonic hedgehog HCAs. HCCs can be grouped into proliferative and nonproliferative subtypes. Proliferative HCCs include macrotrabecular-massive, TP53-mutated, scirrhous, clear cell, fibrolamellar, and sarcomatoid HCCs and combined HCC-cholangiocarcinoma. Steatohepatitic and β-catenin-mutated HCCs constitute the nonproliferative subtypes. iCCAs are classified as small-duct and large-duct types on the basis of the level of bile duct involvement, with significant differences in pathogenesis, molecular signatures, imaging findings, and biologic behaviors. Cross-sectional imaging modalities, including multiphase CT and multiparametric MRI, play an essential role in diagnosis, staging, treatment response assessment, and surveillance. Select imaging phenotypes can be correlated with genetic abnormalities, and identification of surrogate imaging markers may help avoid genetic testing. Improved understanding of morphomolecular features of liver tumors has opened new areas of research in the targeted therapeutics and management guidelines. The purpose of this article is to review imaging findings of select morphomolecular subtypes of HCAs, HCCs, and iCCAs and discuss therapeutic and prognostic implications. Online supplemental material is available for this article. ©RSNA, 2022.

Molecular Identification of Cystoisospora Belli in Patients Infected with the Human Immunodeficiency Virus

Infection with Cystoisospora belli causes severe diarrhea, bile duct involvement, and Extra-intestinal spread in patients with acquired immunodeficiency syndrome (AIDS). This study included eight adult patients with AIDS and chronic diarrhea diagnosed stool cystosisporiasis (oocysts) and / or duodenal biopsies (asexual and sexual stages in epithelium). Identification was carried out at the molecular level using a nested-polymerase chain reaction (PCR) technique that amplifies a fragment of the ribosomal ribonucleic acid (RNA) small subunit gene, using DNA extracted from feces and biopsies. This work allowed us to make the diagnosis of cystosisporiasis through analysis coproparasitological, optimize the DNA extraction protocol from samples fecal and implement the nested -PCR technique for the diagnosis of C. belli in biological samples from infected patients.

Abstract 3387: A plasma metabolomic signature improves prediction of malignant intraductal papillary mucinous neoplasm

Abstract Pancreatic cancer is one of the deadliest cancers primarily because most patients are diagnosed at advanced, unresectable stages. To reduce the incidence and mortality of pancreatic cancer, it is crucial to timely identify and optimally treat its precursors. One such precursor is intraductal papillary mucinous neoplasm (IPMN) as some of these cyst lesions have potential to progress to invasive cancer. Current clinical guidelines for predicting and treating malignant IPMN have a satisfactory sensitivity (>90%) but a dismal specificity (25-30%), leading to a number of unnecessary pancreatic resections in benign IPMN. Therefore, there is an urgent unmet need to identify molecular biomarkers to improve malignant IPMN prediction. The present study was thus conducted to identify metabolomic biomarkers that can preoperatively differentiate malignant IPMN from benign IPMN. A global plasma metabolomic analysis of 607 metabolites was performed on Xevo GS-X2 quadrupole time-of-flight (TOF) mass spectrometers (MS) using a 2D column configuration in 125 cases of low/moderate-grade IPMN and 50 cases of high-grade/invasive IPMN, confirmed by surgical pathology. Of the 607 metabolites, 19 were selected based on their p values for the differences between the two groups compared (all p<0.005). All potential combinations (1 to 8 metabolites) of the 19 metabolites were examined by 3-fold cross validation. The training and test sets included 67% and 33% of total samples, respectively, while keeping the same ratio of low/moderate-grade IPMN to high-grade/invasive IPMN. We repeated such cross validation 100 times by random selection of the training and test sets. The metabolite combination that can distinguish malignant IPMN from benign IPMN with the highest average AUC (area under the receiver operating characteristics curve) (=0.70) consisted of four lipid-related metabolites. AUCs (95% CIs) were 0.63 (0.53 - 0.72), 0.64 (0.55 - 0.73), 0.65 (0.56 - 0.74), and 0.64 (0.54 - 0.73) for the metabolites 1-4, respectively. CA19-9 is the only established biomarker for pancreatic cancer. As shown above, AUCs for the four individual metabolites and for their combination were greater than the AUC of CA19-9 [0.58 (0.47 - 0.69)]. Adding the four metabolites and CA19-9 to the model that contained clinical and imaging variables (including age, sex, BMI, main pancreatic duct diameter, and radiographic main duct involvement) yielded an AUC (95% CI) of 0.82 (0.75 - 0.90). In summary, we identified a plasma signature of four metabolites that outperformed CA19-9 in the discrimination of malignant IPMN from benign IPMN. If replicated in other independent patient populations, the findings of the present study can aid in the risk stratification of IPMN patients prior to surgery. Citation Format: Johannes Fahrmann, Hao Fan, Michele Yip-Schneider, Huangbing Wu, Ziyue Liu, Jun Wan, Sheng Liu, Samir Hanash, C. Max Schmidt, Jianjun Zhang. A plasma metabolomic signature improves prediction of malignant intraductal papillary mucinous neoplasm [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2022; 2022 Apr 8-13. Philadelphia (PA): AACR; Cancer Res 2022;82(12_Suppl):Abstract nr 3387.

Imaging of fibropolycystic liver disease.

Fibropolycystic liver diseases (FLDs) make up a rare spectrum of heritable hepatobiliary diseases resulting from congenital ductal plate malformations (DPMs) due to the dysfunction of proteins expressed on the primary cilia of cholangiocytes. The embryonic development of the ductal plate is key to understanding this spectrum of diseases. In particular, DPMs can result in various degrees of intrahepatic duct involvement and a wide spectrum of cholangiopathies, including congenital hepatic fibrosis, Caroli disease, polycystic liver disease, and Von Meyenberg complexes. The most common clinical manifestations of FLDs are portal hypertension, cholestasis, cholangitis, and (in rare cases) cholangiocarcinoma. This article reviews recent updates in the pathophysiology, imaging, and clinical management of FLDs.

Assessment of tumor extension to the ductal system of submucosal glands in patients with superficial esophageal squamous neoplasms: Implications for endoscopic resection.

Endoscopic submucosal dissection (ESD) has become the standard treatment for superficial esophageal squamous cell neoplasia (SESCN); however, local recurrence still occurs occasionally even in patients who meet the current curative criteria. Esophageal ducts of the submucosal gland may serve as a pathway for the spread of SESCN to a deeper layer. However, the clinical impact of ductal involvement (DI) in patients undergoing ESD has yet to be investigated. We consecutively enrolled patients with SESCN who were treated with ESD. The resected specimens were meticulously reviewed in multiple section slices for the presence and resected margins of DI, and their correlations with clinical factors were evaluated. A total of 210 lesions were analyzed, of which 78 (37.1%) presented with DI. The presence of submucosal invasion, lymphovascular invasion (LVI), and DI were indicators of worse prognosis (P<.05). Deep extended DIs were misdiagnosed as deep submucosal invasive cancer in 4 cases (2%). Of the 185 patients who met the criteria for curative ESD (ie, R0 resection and no deep submucosal invasion or LVI), 11 (5.9%) developed local recurrence/metastasis during a mean follow-up of 55.2months (range, 6 to 140) months. Compared with patients with without DI, patients with DI had worse recurrence-free survival (P=.008, log-rank test) and a higher local risk of recurrence (12.7% vs 2.5%) after curative ESD (hazard ratio, 4.20; P=.038). A precise histological assessment of DI in SESCN is crucial after ESD, given that DI is common and associated with worse outcome. Whether total removal of esophageal glands/ducts can improve outcome requires future study.

Intraductal tubulopapillary neoplasm (ITPN) of the pancreas: a distinct entity among pancreatic tumors.

AimsIntraductal tubulopapillary neoplasm (ITPN) of the pancreas is a recently recognized pancreatic tumor entity. Here we aimed to determine the most important features with a systematic review coupled with an integrated statistical approach.Methods and resultsPubMed, SCOPUS, and Embase were searched for studies reporting data on pancreatic ITPN. The clinicopathological, immunohistochemical, and molecular data were summarized. Then a comprehensive survival analysis and a comparative analysis of the molecular alterations of ITPN with those of pancreatic ductal adenocarcinoma (PDAC) and intraductal papillary mucinous neoplasm (IPMN) from reference cohorts (including the International Cancer Genome Consortium‐ ICGC dataset and The Cancer Genome Atlas, TCGA program) were conducted. The core findings of 128 patients were as follows: (i) Clinicopathological parameters: pancreatic head is the most common site; presence of an associated adenocarcinoma was reported in 60% of cases, but with rare nodal metastasis. (ii) Immunohistochemistry: MUC1 (>90%) and MUC6 (70%) were the most frequently expressed mucins. ITPN lacked the intestinal marker MUC2; unlike IPMN, it did not express MUC5AC. (iii) Molecular landscape: Compared with PDAC/IPMN, the classic pancreatic drivers KRAS, TP53, CDKN2A, SMAD4, GNAS, and RNF43 were less altered in ITPN (P < 0.001), whereas MCL amplifications, FGFR2 fusions, and PI3KCA mutations were commonly altered (P < 0.001). (iv) Survival analysis: ITPN with a “pure” branch duct involvement showed the lowest risk of recurrence.ConclusionITPN is a distinct pancreatic neoplasm with specific clinicopathological and molecular characteristics. Its recognition is fundamental for its clinical/prognostic implications and for the enrichment of potential targets for precision oncology.

NON-INVASIVE IMAGING OF CHOLANGIO-PANCREATIC DISEASES USING MAGNETIC RESONANCE CHOLANGIOPANCREATOGRAPHY AND CORRELATION WITH ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY

NON-INVASIVE IMAGING OF CHOLANGIO-PANCREATIC DISEASES USING MAGNETIC RESONANCE CHOLANGIOPANCREATOGRAPHY AND CORRELATION WITH ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY

Liver Transplantation for Hepatocellular Carcinoma With Bile Duct Tumor-Associated Thrombi: A Systematic Review and Pooled Analysis.

The significance of bile duct tumor-associated thrombi in patients undergoing transplantation for hepatocellular carcinoma (HCC) is controversial. Therefore, we performed a systematic review of the literature with pooled analysis to investigate the impact of biliary invasion on HCC recurrence and patient survival. Of 1,584 references screened, eight were included for analysis. Demographics, patient and tumor factors, recurrence, and survival data were analyzed. Time to recurrence and death were extracted from each paper by cross-referencing survival curves. A total of 35 patients across eight studies were pooled for analysis when follow-up data were available. At 1 year, 92.9% of patients undergoing transplantation for HCC with bile duct thrombi were alive. Overall survival at 3 and 5 years was 65.5 and 49.6%, respectively. At 1 year, 21.6% of patients had recurrence of their disease, while at 3 years, 50.4% of patients had recurrence. Of those patients with recurrence in the first year, 71.4% recurred within the first 3 months after transplantation. Overall patient survival decreased within the first 5 years, but then stabilized. The 5-year survival rate of 49.6% in this pooled analysis is lower than that reported for patients undergoing transplantation for HCC within the Milan criteria (50-78%) or recent reports in patients with portal vein involvement (63.6%), though data is limited by a lack of long-term follow-up in this understudied population. Transplantation for patients with HCC with bile duct involvement may be a viable treatment option, warranting further investigation.

Small duct primary sclerosing cholangitis: A discrete variant or a bridge to large duct disease, a practical review.

The natural history, associations with inflammatory bowel disease (IBD), and long-term outcomes of large duct primary sclerosing cholangitis (ldPSC) have been well documented. Small duct primary sclerosing cholangitis (sdPSC) is a much less common and relatively more benign variant. The natural history of sdPSC has been difficult to characterize given the limited number of studies in the literature especially with regards to the subset of patients who progress to large duct involvement. It has been unclear whether sdPSC represented a subset of ldPSC, an earlier staging of ldPSC, or a completely separate and distinct entity of its own. Strong associations between sdPSC and IBD have been established with suspicion that concurrent sdPSC-IBD may be a key prognostic factor in determining which patients are at risk of progression to ldPSC. Little is known regarding the discrete circumstances that predisposes some patients with sdPSC to progress to ldPSC. It has been suspected that progression to large biliary duct involvement subjects this subset of patients to potentially developing life-threatening complications. Here the authors conducted a thorough review of the published sdPSC literature using Pubmed searches and cross-referencing to compile all accessible studies regarding cohorts of sdPSC patients in order better characterize the subset of sdPSC patients who progress to ldPSC and the associated outcomes.

Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) for advanced hepatocellular carcinoma with macrovascular invasion.

Patients with advanced hepatocellular carcinoma (HCC) and macrovascular invasion (MVI) have dismal prognosis and are referred to systemic treatment or palliation. To investigate the outcomes of patients with HCC and MVI undergoing the associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) procedure. Demographics and operative data were retrospectively reviewed. All types of hepatectomies and all types of ALPPS modifications were included. MVI was categorized according to the Japanese Liver Cancer Study Group classification. 28 patients were included. Viral aetiology was the most common cause of chronic liver disease (89.3%). 85.7% of patients were cirrhotic, with a median MELD score of 9 (7-10). MVI of the hepatic veins or inferior vena cava was diagnosed in 46.4% of patients while portal vein involvement was present in 64.2% of cases. Four patients (14.2%) were diagnosed with bile duct involvement. No patients died after Step 1 while complications occurred in 21.4% of cases. Following step 2, 3 patients (11.5%) died and 20 (69.2%) developed complications. Grade B and C post-hepatectomy liver failure occurred in 57.6% and 11.5% of patients, respectively. After a median follow-up of 18months (7-35), median survival was 22months (3-40). Eleven patients (39.3%) recurred. Median disease-free survival was 15months (5-26). The ALPPS procedure is an extreme rescue approach in otherwise inoperable advanced HCC with MVI. The procedure is associated with high morbidity and mortality and patients' selection is pivotal. Oncological outcomes are safe and should be further investigated.

Serial EUS-Guided FNA for the Surveillance of Pancreatic Cysts: A Study of Long-Term Performance of Tumor Markers.

The natural history of KRAS mutations in mucinous pancreatic cysts (MPCs) over time remains to be fully understood. The aim of this study was to examine the performance of DNA markers and assess changes of KRAS mutationsover time. Patients who underwent EUS-FNA of pancreatic cysts with at least two separate molecular analysis results were included in the study. We assessed the baseline patient and cyst characteristics, and DNA fluid analysis. The presence of either a KRAS mutation, or a CEA > 192ng/ml was used as the diagnostic standard for mucinous cysts when surgical pathology was not available. A total of 933 pancreatic cyst fluid samples were collected, including 117 with ≥ 2 FNAs. Examinations were performed over a median of 30months (range 1-115months). Forty-three (36%) had a mutant KRAS on the index analysis out of which 26 had a change in their KRAS status to the wild-type. Eighty-one (64%) had a wild-type KRAS on the index analysis out of which 18 had change in their KRAS status to mutant type. There was no significant difference in the index cyst characteristics,presence of symptoms, or main duct involvement based on KRAS status change. Increasing age was associated with a changing KRAS mutation status (p = 0.023). KRAS mutations gain and loss in pancreatic cyst fluid appears to occur frequently during long-term surveillance of MPCs. Age appears to be the only predictor for KRAS change over time.

Inflammatory Bowel Diseases Affect the Phenotype and Disease Course of Coexisting Immune-Mediated Inflammatory Diseases: A Systematic Review With Meta-Analysis.

It is unclear whether inflammatory bowel diseases (IBDs) affect the phenotype and severity of co-occurring immune-mediated inflammatory diseases (IMIDs). We aimed to investigate the characteristics of IMIDs in relation to co-occurring IBD. We conducted a systematic review of Medline and EMBASE databases from inception to September 2020. We identified studies reporting the phenotype, severity, or disease course of IMIDs among patients with or without co-occurring IBD. A meta-analysis was conducted using random effects models. The electronic search yielded 13220 studies that we narrowed down to 73 eligible studies for full-text review, including 42 on primary sclerosing cholangitis, 12 on axial spondyloarthropathies, and 8 studies on psoriasis. In primary sclerosing cholangitis, IBD was associated with less frequent involvement of extrahepatic bile ducts (risk ratio [RR], 0.50; 95% confidence interval [CI], 0.33-0.75), longer liver transplantation-free survival (hazard ratio, 0.70; 95% CI, 0.60-0.82), and no increased risk of cholangiocarcinoma (RR, 0.88; 95% CI, 0.59-1.31). Patients with axial spondyloarthropathies and co-occurring IBD were characterized by an increased risk of dactylitis (RR, 2.06; 95% CI, 1.24-3.42), a lower Bath Ankylosing Spondylitis Radiology Index (mean difference [MD] = -2.28; 95% CI, -3.26 to -1.30), and better Schober's test results (MD = 1.07; 95% CI, 0.64-1.49). Psoriasis and co-occurring IBD was associated with reduced disease severity (RR, 1.41; 95% CI, 1.02-1.96) and less frequent presentation in nails (RR, 0.14; 95% CI, 0.05-0.42), with no apparent impact on psoriatic arthritis (RR, 0.94; 95% CI, 0.27-3.31). This systematic review with meta-analysis found IBD is associated with a distinct disease phenotype among the IMIDs investigated. Our findings emphasize the importance of multidisciplinary approaches to patients with co-occurring IMIDs and IBD.

P258 Impact of inflammatory bowel diseases on the phenotype and severity of primary sclerosing cholangitis - a systematic review with meta-analysis

Abstract Background Data regarding the impact of co-occurring inflammatory bowel diseases (IBD) on the phenotype and severity of primary sclerosing cholangitis (PSC) are emerging. The purpose of our study was to characterize the phenotype and disease course of PSC in relation to co-occurring IBD. Methods The purpose of our study was to characterize the phenotype and disease course of PSC in relation to co-occurring IBD. The systematic review and meta-analysis were conducted according to Cochrane’s recommendations. PubMed and EMBASE were searched from database inception till January 2020 for studies reporting disease phenotype and severity of PSC in association with co-occurrence of IBD. Meta-analyses were performed using a random-effects model. The quality of the studies was assessed by the Newcastle-Ottawa Scale (NOS). Results The electronic search yielded 12,220 studies which were narrowed down to 132 after screening based on study titles and abstracts. Of these, a full-text review identified 42 eligible studies, including a total of 8,081 and 5,728 patients with PSC and with or without co-occurring IBD, respectively (Figure 1). The mean NOS score was 6.4 ad included 23 (54.8%) and 18 (42.9%) studies of moderate and high methodological quality, respectively. The meta-analysis demonstrated that co-occurrence of IBD was associated with significantly less frequent involvement of the extra-hepatic ducts (risk ratio (RR)=0.50 (95% CI 0.33–0.75), p&amp;lt;0.01, I2=0%) as compared with patients without co-occurring IBD. However, IBD-PSC and PSC without IBD had similar rate of small-duct PSC, histologic stages of PSC, risk of developing cirrhosis, and features of decompensation. Furthermore, in comparison to patients with isolated PSC, co-occurring IBD was associated with an increased overall risk of malignancies (RR=1.53 (95% CI 1.01–2.32), p=0.04, I2=46%), which was ascribed to colorectal cancers (RR=4.77 (95% CI 2.50–9.09), p&amp;lt;0.01, I2=0%) and not cholangiocarcinomas (RR=0.88 (95% CI 0.59–1.31), p=0.54, I2=31%), gallbladder cancers (RR=2.28 (95% CI 0.39–13.21), p=0.36, I2=0%), or hepatocellular carcinomas (RR=4.49 (95% CI 0.57–35.46), p=0.15, I2=0%). Regarding long-term PSC outcomes, having IBD was not associated with the need of liver transplantation (RR=0.84 (95% CI 0.50–1.41), p=0.52, I2=60%) or mortality (RR=0.74 (95% CI 0.37–1.50), p=0.40, I2=37%), but was associated with a longer liver transplantation-free survival (hazard ratio = 0.70 (95% CI 0.60–0.82), p&amp;lt;0.01, I2=5%). Conclusion This systematic review with meta-analysis suggests that IBD is associated with a distinct phenotype and course of PSC, thus emphasizing the importance of a multidisciplinary approach to patients with co-occurring PSC and IBD.

Utilizing CT soft-tissue markers as a screening tool for acute invasive fungal sinusitis.

Acute invasive fungal sinusitis (AIFS) is a rapidly progressive disease, whose delayed identification results in poor outcomes, especially in immunocompromised individuals. A surge in of AIFS in the wake of the COVID-19 pandemic has lent additional morbidity and mortality to an already precarious clinical scenario. Early detection of AIFS in individuals who are symptomatic/ at risk can allow early therapy, enabling better patient outcomes. Our study aims to determine optimal soft-tissue markers on CT for the early detection of AIFS. In this case-control study, 142 patients with equal distribution of subjects were chosen based on histopathological diagnosis of AIFS; and their non-contrast CT scans were retrospectively assessed to determine the diagnostic utility of specific soft-tissue markers that would enable diagnosis of AIFS. A total of nine markers with adequate sensitivity and specificity were identified, including pterygopalatine and sphenopalatine fossae, inferior orbital fissure and nasolacrimal duct involvement, premaxillary thickening, retro-antral and orbital stranding, and infratemporal muscle oedema. It was determined that the combined occurrence of any three out of nine markers was 91.5% sensitive and 95.9% specific for diagnosis of AIFS (p < 0.005). Early, accurate detection of AIFS in predisposed individuals is possible with identification of soft-tissue markers on NECT, enabling early intervention. Being the aggressive disease that it is, AIFS may be managed early if the index of suspicion is held high via CT imaging; which our diagnostic checklist aims at enabling.

Liver Transplantation with Concurrent Pancreatoduodenectomy for Hilar Cholangiocarcinoma with Common Bile Duct Involvement

Introduction: Neoadjuvant chemoradiotherapy with orthotopic liver transplantation (OLT) is effective treatment for perihilar cholangiocarcinoma (pCCA). Although visible common bile duct (CBD) involvement is an exclusion, patients with primary sclerosing cholangitis (PSC) and microscopic CBD involvement detected by frozen section or endoscopic brushings prior to neoadjuvant therapy have undergone pancreatoduodenectomy (PD) or planned en-bloc hepatopancreatoduodenectomy (HPD) during OLT.

Analysis of Clinical, Serological, and Imaging Features of Autoimmune Pancreatitis and a Case-Control Study on Prognostic Factors in Response to Hormone Therapy.

Objective The paper aimed to analyze the clinical, serological, and imaging features of autoimmune pancreatitis (AIP) and the prognostic factors affecting hormone therapy. Methods A total of 106 patients with AIP enrolled in our hospital from March 2016 to August 2018 were treated with the hormone. The curative effect and recurrence were followed up. The patients were divided into relapse group (n = 42) and nonrelapse group (n = 64) according to the recurrence within 3 years after initial hormone therapy. The symptoms and signs, laboratory examination, and treatment were compared, and binary logistic regression was employed to explore the risk factors of AIP recurrence. Results Among the 106 patients included in this study, there were 78 males and 28 females, with a male-to-female ratio of 3:1. The average age of onset was 56.25 ± 8.87 years; the minimum age was 39 years; and the maximum age was 7 years. The main clinical symptoms were jaundice (67.92%), abdominal pain (48.11%), and abdominal distension (33.96%). In addition, there were symptoms of weight loss, nausea, vomiting, itching, and gray stool. Previous complications included 27.35% diabetes (29/106), 22.64% hypertension (24/106), 35.84% smoking (38/106), and 28.30% alcohol consumption (30/106). The serological characteristics were mainly the increase in serum IgG4 level; 92.45% (98/106) level was higher compared to the upper limit of normal value; the median level was 11.65 g/L; and the highest level was 35.79 g/L. A total of 88.67% (94/106) had an abnormal liver function. The results of imaging examination indicated that 58.49% (62/106) of extrapancreatic organs were involved, of which 46.22% (49/106) were the most common bile duct involvement. All the patients in the group reached a state of remission after hormone treatment. After the disease was relieved, the patients were followed up for 3 years. The recurrence rate was 39.62% (42/106), and the median time of recurrence (month) was 9 (range 2–36). The recurrence rates within 1, 2, and 3 years were 20.75%, 31.13%, and 39.62%, respectively. Among the recurrent patients, 52.38% (22/42) relapsed within 1 year, 78.57% (33/42) within 2 years, and 100.00% (42/42) within 3 years. Multivariate analysis showed that the short duration of glucocorticoid therapy and involvement of extrapancreatic organs were risk factors for relapse after glucocorticoid therapy in patients with type I AIP. Conclusion Type 1 AIP is more common in middle-aged and elderly men. The clinical symptoms of jaundice, abdominal pain, and abdominal distension are common, often accompanied by involvement of extrapancreatic organs, of which bile duct involvement is the most common. Type 1 AIP glucocorticoid treatment acceptance and disease remission are better, but the recurrence rate is higher after glucocorticoid treatment. Patients with a short time of glucocorticoid treatment and involvement of extrapancreatic organs may have a higher risk of recurrence.