Pancreatology最新文献

{"title":"Obituary Martin Sarner, London 1935–2024","authors":"Markus M. Lerch","doi":"10.1016/j.pan.2024.12.019","DOIUrl":"10.1016/j.pan.2024.12.019","url":null,"abstract":"","PeriodicalId":19976,"journal":{"name":"Pancreatology","volume":"25 1","pages":"Pages 1-2"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143145753","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Three-tiered critical care management of acute pancreatitis","authors":"Piroska Pázmány ,&nbsp;Anna Kanjo ,&nbsp;Zsanett Macht-Szalai ,&nbsp;Noémi Gede ,&nbsp;Nelli Farkas ,&nbsp;Bálint Erőss ,&nbsp;Andrea Szentesi ,&nbsp;Áron Vincze ,&nbsp;Roland Hagendorn ,&nbsp;Zsolt Márton ,&nbsp;Andrea Párniczky ,&nbsp;Péter Hegyi ,&nbsp;Zsolt Molnár","doi":"10.1016/j.pan.2024.11.021","DOIUrl":"10.1016/j.pan.2024.11.021","url":null,"abstract":"<div><h3>Introduction and aims</h3><div>Acute pancreatitis (AP) can rapidly progress from a stable condition to multiple organ failure with high mortality. We aimed to describe the characteristics of AP patients requiring admission to a critical care facility and to identify predictors of disease progression.</div></div><div><h3>Methods</h3><div>We conducted a post-hoc analysis using prospectively collected data from AP patients admitted to the high dependency unit (HDU) and intensive care unit (ICU) at the University of Pécs, Hungary, from 2016 to 2019. Patients were categorized according to critical care needs and severity. Daily records of organ function, organ support and laboratory parameters were kept. Descriptive analysis and predictive models were developed to forecast the need for escalated critical care and mortality.</div></div><div><h3>Results</h3><div>Analysis of 92 cases (65 % male, mean age 63 (range 19–92) years) revealed a median critical care stay of 8 days (range 1–69) and a mortality rate of 47 %. Naive Bayes prediction models using admission C-reactive protein (CRP) and amylase levels achieved 75 % accuracy in predicting mortality and a 65 % probability of requiring HDU and/or ICU admission. CRP levels increased significantly (47 vs 62 mg/l, p: 0.015) from 48 to 24 h before critical care admission, contrasting with controls, resulting in significantly higher CRP levels in critical care patients (62 vs 32 mg/l, p: 0.007) 24 h before admission.</div></div><div><h3>Conclusion</h3><div>Our findings suggest that on-admission CRP and amylase cannot reliably predict progression of AP. However, elevated and increasing levels of CRP and amylase may indicate the need for early HDU admission to enable closer monitoring.</div></div>","PeriodicalId":19976,"journal":{"name":"Pancreatology","volume":"25 1","pages":"Pages 39-47"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854965","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"No definite associations between opioid doses and severity of acute pancreatitis – Results from a multicentre international prospective study","authors":"Cecilie Siggaard Knoph ,&nbsp;Nejo Joseph ,&nbsp;James Lucocq ,&nbsp;Søren Schou Olesen ,&nbsp;Wei Huang ,&nbsp;Jahnvi Dhar ,&nbsp;Jayanta Samanta ,&nbsp;Rupjyoti Talukdar ,&nbsp;Gabriele Capurso ,&nbsp;Paoletta Preatoni ,&nbsp;Enrique de-Madaria ,&nbsp;Dhiraj Yadav ,&nbsp;John Windsor ,&nbsp;Asbjørn Mohr Drewes ,&nbsp;Manu Nayar ,&nbsp;PAINAP Collaborative ,&nbsp;Sanjay Pandanaboyana","doi":"10.1016/j.pan.2024.12.014","DOIUrl":"10.1016/j.pan.2024.12.014","url":null,"abstract":"<div><h3>Background</h3><div>Abdominal pain is the cardinal symptom of acute pancreatitis (AP), often requiring opioid therapy. This study aimed to investigate the dose-dependent relationship between opioid therapy and moderately severe or severe AP.</div></div><div><h3>Methods</h3><div>This was a post-hoc analysis of the prospective PAINAP database, which recruited patients with first-time AP from 118 centres across 27 countries between April–June 30, 2022. Baseline demographic details, opioid treatment dose, and AP outcome characteristics were extracted. The intravenous morphine-equivalent doses (MEDs) of each opioid administered were calculated based on daily doses and duration. They were subsequently summarised into cumulative MEDs. Furthermore, mean daily intravenous MEDs were registered. Using multivariable regression analysis, associations between opioid doses and the severity of AP were explored.</div></div><div><h3>Results</h3><div>The final cohort consisted of 1,043 patients receiving various doses of opioids (51 % male; median age 54 years). Most (79 %) patients had mild, 14 % moderately severe, and 7 % severe AP. Median cumulative MED was 20 mg (IQR, 8–48), whereas median daily MED was 6 (IQR, 3–11), and median duration was 3 days (IQR, 2–5). There was a significant association between moderately severe or severe AP and cumulative intravenous MEDs per 10 mg (OR 1.02 (IQR 1.00–1.03), P = 0.01). When considering daily intravenous MEDs, this association was non-significant (P = 0.15).</div></div><div><h3>Conclusions</h3><div>The association between opioid doses and AP severity was dose-dependent with cumulative opioid doses but not with daily doses. In the absence of adequate evidence and potential reverse causation bias, future studies are warranted to assess the safety of opioids in AP.</div></div>","PeriodicalId":19976,"journal":{"name":"Pancreatology","volume":"25 1","pages":"Pages 12-19"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915441","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Optimal indication of adding pancreatic juice cytology in the diagnosis of malignant intraductal papillary mucinous neoplasm of the pancreas","authors":"Takeshi Mori ,&nbsp;Yasutaka Ishii ,&nbsp;Yumiko Tatsukawa ,&nbsp;Shinya Nakamura ,&nbsp;Juri Ikemoto ,&nbsp;Sayaka Miyamoto ,&nbsp;Kazuki Nakamura ,&nbsp;Masaru Furukawa ,&nbsp;Yumiko Yamashita ,&nbsp;Noriaki Iijima ,&nbsp;Yasuhiro Okuda ,&nbsp;Risa Nomura ,&nbsp;Koji Arihiro ,&nbsp;Kenichiro Uemura ,&nbsp;Shinya Takahashi ,&nbsp;Hideki Ohdan ,&nbsp;Shiro Oka","doi":"10.1016/j.pan.2024.12.010","DOIUrl":"10.1016/j.pan.2024.12.010","url":null,"abstract":"<div><h3>Background/Objectives</h3><div>Positive pancreatic juice cytology (PJC) is an important finding when considering surgical resection in patients with intraductal papillary mucinous neoplasm (IPMN); however, guidelines do not recommend endoscopic retrograde cholangiopancreatography (ERCP) for PJC. This study aimed to clarify the findings worthy of adding PJC for diagnosing high-grade dysplasia (HGD) and invasive carcinoma (IC) in patients with IPMN.</div></div><div><h3>Methods</h3><div>Patients with IPMN who underwent preoperative PJC and surgical resection at Hiroshima University Hospital were enrolled, and the diagnostic yield of malignant IPMN based on PJC and clinical and imaging findings and the incidence of post-ERCP pancreatitis (PEP) were retrospectively analyzed.</div></div><div><h3>Results</h3><div>Of the 129 eligible patients, 61 (47%) had malignant tumors (29 HGD and 32 IC). The diagnostic yields of PJC were as follows: 33%, 97%, 91%, 62%, and 67% for sensitivity, specificity, and positive predictive value, and negative predictive value, respectively. Multivariate analysis revealed that an abrupt change in the pancreatic duct caliber was an independent predictive factor of true-positive PJC (hazard ratio: 15.81, <em>P</em> = 0.001), with a diagnostic sensitivity of 86% for PJC in these patients. The incidence rate of PEP was 19%, and the pancreatic body and tail lesions, main pancreatic duct diameter &lt;10 mm, and placement of a nasopancreatic drainage catheter were significant risk factors for PEP.</div></div><div><h3>Conclusions</h3><div>Although PJC is generally not recommended for patients with IPMN, it is worth considering for the determination of treatment strategies in patients with abrupt changes in the caliber of the pancreatic duct with distal pancreatic atrophy.</div></div>","PeriodicalId":19976,"journal":{"name":"Pancreatology","volume":"25 1","pages":"Pages 118-124"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142896690","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"High prevalence of pancreatic steatosis in pancreatic cancer patients: A meta-analysis and systematic review","authors":"Cătălina Vlăduț ,&nbsp;Corinna Steiner ,&nbsp;Matthias Löhr ,&nbsp;Dilara Turan Gökçe ,&nbsp;Patrick Maisonneuve ,&nbsp;Thomas Hank ,&nbsp;Daniel Öhlund ,&nbsp;Malin Sund ,&nbsp;Sanne A. Hoogenboom","doi":"10.1016/j.pan.2024.11.010","DOIUrl":"10.1016/j.pan.2024.11.010","url":null,"abstract":"<div><h3>Objective</h3><div>In the last decade there has been increasing interest in defining pancreatic steatosis (PS) and establishing its association with pancreatic ductal adenocarcinoma (PDAC). However, no consensus guidelines have yet been published on the management of PS. In this systematic review and meta-analysis performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we investigated the association between PS and PDAC.</div></div><div><h3>Design</h3><div>Medical literature between 2007 and 2023 was reviewed for eligible trials investigating the prevalence of PS in patients with PDAC. Eligible studies reporting on PS, assessed via imaging or histology, were included. The primary objective was to determine the association between PDAC and PS by comparing the prevalence of PS in individuals with- and without PDAC. Secondary, an evaluation was conducted to establish whether the method of assessment correlated with the association of PDAC and PS, and the prevalence of PDAC in individuals with PS. Measures of effect size were determined using odds ratios (ORs) and corresponding 95 % confidence intervals (95 % CI).</div></div><div><h3>Results</h3><div>The systematic review identified a total of 23 studies, of which seventeen studies examined PS prevalence among PDAC patients and were included in the meta-analysis. Overall, the pooled prevalence of PS in patients with PDAC was 53.6 % (95 % CI 40.9–66.2). No significant difference in PS prevalence was observed across various diagnostic methods or geographical regions. Overall, the pooled OR for PS in patients with PDAC compared to controls was 3.23 (95 % CI 1.86–5.60).</div></div><div><h3>Conclusions</h3><div>PDAC patients have a high prevalence of PS, and they are significantly more likely to have PS compared to controls. These findings emphasize the need to prioritize a standardized approach to the diagnosis, follow-up, and treatment of PS, with future studies focusing on identifying patients who would benefit from PDAC surveillance programs.</div></div>","PeriodicalId":19976,"journal":{"name":"Pancreatology","volume":"25 1","pages":"Pages 98-107"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142872783","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Associations between ABO, FUT2 and chronic pancreatitis: A comprehensive meta-analysis of multiple cohorts and public biobanks","authors":"Zeng-Kan Du ,&nbsp;Yuan-Chen Wang ,&nbsp;Ya-Hui Wang ,&nbsp;Xiao-Yu Li ,&nbsp;Yi-Zhou Zheng ,&nbsp;Di Wu ,&nbsp;Wei-Ming Qu ,&nbsp;Zhuan Liao ,&nbsp;Wen-Bin Zou","doi":"10.1016/j.pan.2024.12.016","DOIUrl":"10.1016/j.pan.2024.12.016","url":null,"abstract":"<div><h3>Objectives</h3><div>Associations of ABO blood group specifying transferases A/B (<em>ABO</em>) and fucosyltransferase 2 (<em>FUT2</em>) with CP remain inconclusive. We aimed to comprehensively investigate the associations by Chinese sequencing cohorts and external cohorts.</div></div><div><h3>Methods</h3><div>First, we analyzed the distributions of ABO blood groups and FUT2 status, along with lead single nucleotide polymorphisms (SNPs) at <em>ABO</em> (rs8176693 C/T) and <em>FUT2</em> (rs632111 A/G) gene loci in Chinese low-coverage whole-genome sequencing discovery cohort. Subsequently, we investigated the associations of CP with <em>ABO</em> and <em>FUT2</em> SNPs in Chinese whole-exome sequencing validation cohort and three public biobanks (FinnGen, UK Biobank, and BioBank Japan). Finally, comprehensive meta-analysis was performed by integrating data from two Chinese cohorts, reported cohorts and public biobanks.</div></div><div><h3>Results</h3><div>Firstly, in Chinese discovery cohort, the distribution of blood types in CP patients showed no significant difference compared to healthy controls, and similar results were observed in subgroup analyses and in meta-analysis with the reported cohorts. Secondly, results indicated no association between rs8176693 or rs632111 and CP in Chinese cohorts and meta-analysis of three biobanks, though <em>ABO</em> SNP was found to be significantly associated with CP in UK Biobank (odds ratio [OR] = 1.27, <em>P</em> = 0.04). Finally, no association was observed between rs8176693 (OR = 1.03, <em>P</em> = 0.29) or rs632111 (OR = 1.04, <em>P</em> = 0.10) and CP in comprehensive meta-analysis.</div></div><div><h3>Conclusions</h3><div>No association was found between lead SNPs of <em>ABO</em> or <em>FUT2</em> and CP in meta-analysis, nor was there an association between ABO blood group or FUT2 secretor status and CP in Chinese cohort. <em>ABO</em> and <em>FUT2</em> might play limited role in CP development.</div></div>","PeriodicalId":19976,"journal":{"name":"Pancreatology","volume":"25 1","pages":"Pages 58-64"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142896688","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Feasibility and clinical utility of endoscopic ultrasound-guided tissue acquisition for comprehensive genomic profiling in pancreatic cancer: A systematic review and meta-analysis","authors":"Sung Woo Ko ,&nbsp;Ik Hyun Jo ,&nbsp;Seung Bae Yoon","doi":"10.1016/j.pan.2024.12.012","DOIUrl":"10.1016/j.pan.2024.12.012","url":null,"abstract":"<div><h3>Background</h3><div>Endoscopic ultrasound-guided tissue acquisition (EUS-TA) has become essential for diagnosing pancreatic ductal adenocarcinoma (PDAC) and is increasingly utilized for comprehensive genome profiling (CGP) to advance precision medicine. This systematic review and meta-analysis assess the feasibility and clinical utility of EUS-TA samples for CGP in PDAC.</div></div><div><h3>Methods</h3><div>We conducted a thorough systematic literature search in PubMed, EMBASE, and the Cochrane Library up to October 2023. Key outcomes included sequencing success rates, detection rates of four major driver genes and actionable genes, and concordance rates with other sample types or methodologies.</div></div><div><h3>Results</h3><div>A total of 23 studies met the inclusion criteria. The pooled sequencing success rate was 83.9 % [95 % confidence interval (CI): 75.8–89.7 %]. No significant difference was observed in sequencing success rates between fine needle aspiration and biopsy (odds ratio 1.77, 95 % CI 0.70–4.47). Meta-regression analysis revealed that the minimum DNA requirement for CGP significantly influenced sequencing success rates. The pooled mutation rate for K-ras was 86.4 % (95 % CI 83.6–88.8), while potentially actionable mutations had a pooled rate of 17.7 % (95 % CI 12.8–23.8). The concordance rate between CGP results from EUS-guided samples and surgical specimens was 81.6 % (95 % CI 68.2–90.1).</div></div><div><h3>Conclusion</h3><div>Comprehensive genomic profiling of PDAC using EUS-TA-derived samples demonstrated feasibility in clinical settings. Approximately 18 % of patients undergoing CGP exhibited potentially actionable mutations, highlighting the potential for personalized therapeutic approaches.</div></div>","PeriodicalId":19976,"journal":{"name":"Pancreatology","volume":"25 1","pages":"Pages 89-97"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142896689","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genotype-based prognosis prediction for MEN1-Related pancreatic neuroendocrine tumors in Korean patients a single-center retrospective study","authors":"Juwan Kim ,&nbsp;Seung Soo Hong ,&nbsp;Sung Hyun Kim ,&nbsp;Ho Kyoung Hwang ,&nbsp;Namki Hong ,&nbsp;Yumie Rhee ,&nbsp;Chang Moo Kang","doi":"10.1016/j.pan.2024.11.020","DOIUrl":"10.1016/j.pan.2024.11.020","url":null,"abstract":"<div><h3>Background</h3><div>Pancreatic neuroendocrine tumors (PNETs) are the leading cause of death related to multiple endocrine neoplasia type 1 (MEN1). Previous studies have linked certain mutations in the MEN1 gene and loss of interactions with MENIN's functional partners to the mortality or aggressiveness of PNETs. This study aimed to evaluate the genotype-phenotype correlations of MEN1-related PNETs in Korean patients and to summarize the treatment outcomes comprehensively.</div></div><div><h3>Methods</h3><div>We retrospectively analyzed 72 patients diagnosed with MEN1 at a tertiary care center in Korea between January 2003 and September 2022. MEN1 mutations were analyzed using direct or next-generation sequencing.</div></div><div><h3>Results</h3><div>Among 40 families with MEN1, 10 had exon 2 mutations, which were the most frequently observed. Of these, 50 (69.4 %) were diagnosed with PNETs; 20 underwent pancreatic resection. Patients with truncating mutations showed a significant difference in age-related penetrance of PNET (p = 0.029). No distinct genotype was associated with malignant transformation (lymph node or distant metastasis) in MEN1-related PNETs. In the subgroup Cox model, mutations in exons 3 or 10 showed significant differences in tumor progression in the observation group (adjusted hazard ratio: 8.164,(95 % CI: 1.648–40.436), p = 0.010, HR: 8.300, (95 % CI: 1.808–38.113), p = 0.007).</div></div><div><h3>Conclusion</h3><div>PNETs in Korean patients with MEN1 exhibit a stable prognosis. An individualized follow-up strategy may be necessary, particularly for young patients with truncating mutation in the MEN1 gene. In addition, those with mutations in exons 3 or 10 may require more active surveillance to decrease the risk of progression.</div></div>","PeriodicalId":19976,"journal":{"name":"Pancreatology","volume":"25 1","pages":"Pages 134-141"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142786435","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Misfolding PRSS1 variant p.Ala61Val in a case of suspected intrauterine pancreatitis","authors":"Máté Sándor ,&nbsp;David S. Vitale ,&nbsp;Zoltán Attila Nagy ,&nbsp;Sherif Y. Ibrahim ,&nbsp;Maisam Abu-El-Haija ,&nbsp;Maria Lazou ,&nbsp;Sandor Vajda ,&nbsp;Miklós Sahin-Tóth","doi":"10.1016/j.pan.2024.12.013","DOIUrl":"10.1016/j.pan.2024.12.013","url":null,"abstract":"<div><h3>Background/objectives</h3><div>Genetic variants in <em>PRSS1</em> encoding human cationic trypsinogen are associated with hereditary pancreatitis. The clinically frequent variants exert their pathogenic effect by increasing intrapancreatic trypsin activity, while a distinct subset of variants causes disease via mutation-induced trypsinogen misfolding and endoplasmic reticulum (ER) stress. Here, we report a novel misfolding <em>PRSS1</em> variant.</div></div><div><h3>Methods</h3><div>We used next-generation and Sanger sequencing to screen the index patient. We performed structural modeling and analyzed the functional effects of the <em>PRSS1</em> variant.</div></div><div><h3>Results</h3><div>A heterozygous c.182C&gt;T (p.Ala61Val) <em>PRSS1</em> variant was identified in a case of suspected intrauterine pancreatitis with pseudocyst formation. Recombinant p.Ala61Val trypsinogen autoactivated to lower trypsin levels, but activity of p.Ala61Val trypsin was similar to wild type. In cell culture experiments, the variant exhibited reduced secretion and intracellular retention. Cells expressing the p.Ala61Val variant showed signs of ER stress, as judged by elevated mRNA expression of <em>Hspa5</em> encoding the chaperone BiP, and increased mRNA splicing of the transcription factor <em>XBP1</em>. <strong>Conclusions</strong>: Taken together, the observations expand the repertoire of misfolding <em>PRSS1</em> variants and highlight the need for functional analysis to identify this rare form of genetic etiology.</div></div>","PeriodicalId":19976,"journal":{"name":"Pancreatology","volume":"25 1","pages":"Pages 70-81"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142903349","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Serum IgG4-negative and IgG4-positive type 1 autoimmune pancreatitis present with different clinicopathological features: An analysis of a nationwide survey in Japan","authors":"Takanori Sano ,&nbsp;Kazuhiro Kikuta ,&nbsp;Tetsuya Takikawa ,&nbsp;Ryotaro Matsumoto ,&nbsp;Kazuichi Okazaki ,&nbsp;Yoshifumi Takeyama ,&nbsp;Atsushi Masamune","doi":"10.1016/j.pan.2024.11.018","DOIUrl":"10.1016/j.pan.2024.11.018","url":null,"abstract":"<div><h3>Background/Objective</h3><div>Elevated serum IgG4 (sIgG4) is a useful diagnostic marker of type 1 autoimmune pancreatitis (AIP). This study aimed to clarify the clinicopathological characteristics of the type 1 AIP patients without elevated sIgG4 levels.</div></div><div><h3>Methods</h3><div>We analyzed the clinical data of patients registered in a nationwide epidemiological survey in Japan. AIP was diagnosed according to the International Consensus Diagnostic Criteria. Patients with sIgG4 levels ≥135 mg/dl at the diagnosis were classified as sIgG4-positive AIP, and those with sIgG4 levels &lt;135 mg/dl were as sIgG4-negative AIP.</div></div><div><h3>Results</h3><div>A total of 1285 patients with AIP were enrolled in this study; 1128 (87.8 %) had sIgG4-positive AIP and 157 (12.2 %) had sIgG4-negative AIP. Compared to patients with sIgG4-positive AIP, those with sIgG4-negative AIP more frequently experienced inflammatory bowel diseases (3.8 % vs. 0.4 %), and less frequently developed extrapancreatic lesions (53.5 % vs. 72.3 %), including sclerosing cholangitis (30.6 % vs. 40.7 %) and sialadenitis/dacryoadenitis (5.1 % vs. 24.7 %). Histopathological examinations were performed more frequently in patients with sIgG4-negative AIP. The criterion of abundant IgG4-positive plasma cells was less frequently fulfilled by patients with sIgG4-negative AIP (28.0 % vs. 43.1 %). A Kaplan-Meier analysis showed that relapse occurred less frequently in patients with sIgG4-negative AIP (<em>P</em> = 0.006). Results were similar even if the patients with AIP-not otherwise specified (n = 45) were excluded.</div></div><div><h3>Conclusions</h3><div>Patients with sIgG4-negative type 1 AIP and those with sIgG4-positive type 1 AIP present with different clinicopathological features which suggests heterogeneity of patients with type 1 AIP. Low serum IgG4 levels could indicate low disease activity in type 1 AIP.</div></div>","PeriodicalId":19976,"journal":{"name":"Pancreatology","volume":"25 1","pages":"Pages 82-88"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142792097","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}