Clinics and research in hepatology and gastroenterology最新文献

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Dose-Response Profiles in Incretin and Multi-Agonist Obesity Pharmacotherapy. 肠促胰岛素和多种激动剂肥胖药物治疗的剂量-反应谱。
IF 2.6 4区 医学
Clinics and research in hepatology and gastroenterology Pub Date : 2026-10-20 DOI: 10.1016/j.clinre.2026.102947
Mahdi Yousefian, Maryam Baharmast
{"title":"Dose-Response Profiles in Incretin and Multi-Agonist Obesity Pharmacotherapy.","authors":"Mahdi Yousefian, Maryam Baharmast","doi":"10.1016/j.clinre.2026.102947","DOIUrl":"https://doi.org/10.1016/j.clinre.2026.102947","url":null,"abstract":"<p><p>Incretin and multi-agonist therapies have transformed obesity pharmacotherapy, but dose selection remains less mature than efficacy assessment. This critical narrative review examines nonlinear relationships among administered dose, systemic exposure, weight and cardiometabolic benefit, gastrointestinal tolerability, treatment persistence, and maintenance. Across established and newer programs, average efficacy generally increases with dose, yet incremental gains may narrow at higher exposure while adverse-event burden and discontinuation can increase. The maturity of evidence is heterogeneous, and later-stage programs should not be interpreted as clinically equivalent to agents supported mainly by earlier-phase dose-ranging data. Moreover, an \"optimal dose\" is not an endpoint-independent quantity: the exposure most appropriate for weight loss may differ from that relevant to glycemic control, cardiovascular benefit, body composition, tolerability, or maintenance. We therefore distinguish nominal dose from exposure and present response-guided titration as a hypothesis-generating framework for prospective testing, not as an evidence-based dosing algorithm. Existing continuation and withdrawal studies support the importance of ongoing pharmacological treatment after successful weight loss but do not establish a minimum or optimal de-intensified maintenance dose. Future trials should jointly model efficacy, safety, body composition, patient-reported outcomes, pharmacokinetics, and discontinuation to test objective-specific, patient-level dosing strategies.</p>","PeriodicalId":10424,"journal":{"name":"Clinics and research in hepatology and gastroenterology","volume":" ","pages":"102947"},"PeriodicalIF":2.6,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149227532","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Effectiveness of Emotion Regulation-Based Therapy on Emotion Regulation, Cognitive Flexibility, and Symptom Severity in Patients with Irritable Bowel Syndrome. 基于情绪调节的治疗对肠易激综合征患者情绪调节、认知灵活性和症状严重程度的影响
IF 2.6 4区 医学
Clinics and research in hepatology and gastroenterology Pub Date : 2026-10-20 DOI: 10.1016/j.clinre.2026.102945
Hosein Haji Ali Akbari, Abbas Mokhtari
{"title":"The Effectiveness of Emotion Regulation-Based Therapy on Emotion Regulation, Cognitive Flexibility, and Symptom Severity in Patients with Irritable Bowel Syndrome.","authors":"Hosein Haji Ali Akbari, Abbas Mokhtari","doi":"10.1016/j.clinre.2026.102945","DOIUrl":"https://doi.org/10.1016/j.clinre.2026.102945","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to evaluate the effectiveness of emotion regulation-based therapy on emotion regulation, cognitive flexibility, and symptom severity in patients with irritable bowel syndrome.</p><p><strong>Method: </strong>In this randomized controlled trial, 40 patients with irritable bowel syndrome (diagnosed according to Rome IV criteria) referred to Hospital (2025) were randomly assigned to experimental and control groups (20 per group); 36 participants completed the study (18 per group). The experimental group received ten 90-minute group Emotion Regulation-Based Therapy (ERT) sessions based on the Mennin and Fresco (2014) protocol, with treatment fidelity monitored through supervision after each session. The control group received usual medical care. Data were collected using the Emotion Regulation Questionnaire (ERQ), Cognitive Flexibility Inventory (CFI), and Functional Bowel Disorder Symptom Severity Scale (FS-BSS) at pretest, posttest, and three-month follow-up, and analyzed using repeated-measures ANOVA in SPSS 26.</p><p><strong>Results: </strong>The intervention significantly increased cognitive reappraisal and cognitive flexibility and decreased expressive suppression and symptom severity at posttest and follow-up compared with the control group (p < .05).</p><p><strong>Conclusions: </strong>Emotion regulation-based therapy appears to be an effective adjunctive intervention for improving emotional and cognitive processes and reducing symptom severity in patients with irritable bowel syndrome.</p>","PeriodicalId":10424,"journal":{"name":"Clinics and research in hepatology and gastroenterology","volume":" ","pages":"102945"},"PeriodicalIF":2.6,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149227476","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Antiviral Treatment Adherence and Associated Factors in Chronic Hepatitis B: Evidence From an Underrepresented Region of Vietnam. 慢性乙型肝炎抗病毒治疗依从性和相关因素:来自越南代表性不足地区的证据。
IF 2.6 4区 医学
Clinics and research in hepatology and gastroenterology Pub Date : 2026-10-20 DOI: 10.1016/j.clinre.2026.102949
Thuy Hoang, Ngan Pham, Bao Phan, Thao Hoang, Tu Tran, Thanh Pham, An Nguyen, Tuyet Hoang, Hieu Hoai Vo, Tuan Tran
{"title":"Antiviral Treatment Adherence and Associated Factors in Chronic Hepatitis B: Evidence From an Underrepresented Region of Vietnam.","authors":"Thuy Hoang, Ngan Pham, Bao Phan, Thao Hoang, Tu Tran, Thanh Pham, An Nguyen, Tuyet Hoang, Hieu Hoai Vo, Tuan Tran","doi":"10.1016/j.clinre.2026.102949","DOIUrl":"https://doi.org/10.1016/j.clinre.2026.102949","url":null,"abstract":"","PeriodicalId":10424,"journal":{"name":"Clinics and research in hepatology and gastroenterology","volume":" ","pages":"102949"},"PeriodicalIF":2.6,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149227490","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Outcomes of Early Versus Delayed ERCP in Gallstone Pancreatitis, Stratified by BISAP Severity. 按BISAP严重程度分层的胆石性胰腺炎早期与延迟ERCP的预后
IF 2.6 4区 医学
Clinics and research in hepatology and gastroenterology Pub Date : 2026-10-17 DOI: 10.1016/j.clinre.2026.102936
Elodie Elliott Baumgartner, Lexi Weltin, Jacob Wurster, Muhammad Asghar, John Farrell
{"title":"Outcomes of Early Versus Delayed ERCP in Gallstone Pancreatitis, Stratified by BISAP Severity.","authors":"Elodie Elliott Baumgartner, Lexi Weltin, Jacob Wurster, Muhammad Asghar, John Farrell","doi":"10.1016/j.clinre.2026.102936","DOIUrl":"https://doi.org/10.1016/j.clinre.2026.102936","url":null,"abstract":"<p><strong>Background/objectives: </strong>Endoscopic retrograde cholangiopancreatography (ERCP) is commonly used for biliary decompression in gallstone pancreatitis (GSP), but its optimal timing in uncomplicated GSP remains controversial. We evaluated outcomes of early versus delayed ERCP by disease severity.</p><p><strong>Methods: </strong>We conducted a retrospective cohort study of 207 adults with GSP who underwent ERCP at a tertiary center (2019-2025). Patients were categorized as early (<48 hours) or delayed (>48 hours). Outcomes included ERCP-related adverse events, pancreatitis-related complications, length of stay (LOS), intensive care unit (ICU) admission, and mortality. Continuous variables were analyzed using Welch's t-test or Mann-Whitney U test, and categorical variables using chi-square or Fisher's exact tests, with relative risks (RRs) and 95% confidence intervals.</p><p><strong>Results: </strong>Early (n=103) and delayed (n=104) groups were similar in demographics and Bedside Index of Severity in Acute Pancreatitis (BISAP) distribution, though delayed ERCP patients had higher Atlanta severity (15.4% vs 5.8%; p=0.040). Early ERCP was associated with fewer ERCP-related complications (2.9% vs 17.3%; RR 0.17; p=0.0008), less worsening pancreatitis (1.0% vs 12.5%; RR 0.08; p=0.0013), fewer acute necrotic collections (0% vs 5.8%; p=0.029), and lower ICU admission (8.7% vs 19.2%; p=0.044). Among BISAP 0-1 patients, early ERCP reduced LOS by 2.17 days (p=0.013). LOS differences in BISAP ≥2 patients were not significant. Mortality was low and did not differ significantly.</p><p><strong>Conclusions: </strong>Early ERCP in GSP is associated with fewer complications, reduced ICU utilization, and shorter hospitalization in mild disease. These findings support early intervention in mild-to-moderate GSP and underscore the need for prospective multicenter studies.</p>","PeriodicalId":10424,"journal":{"name":"Clinics and research in hepatology and gastroenterology","volume":" ","pages":"102936"},"PeriodicalIF":2.6,"publicationDate":"2026-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149215064","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The role of mechanosensitive ion channel Piezo1 in the intestinal barrier function changes and related gastrointestinal disease. 机械敏感离子通道Piezo1在肠屏障功能改变及相关胃肠道疾病中的作用
IF 2.6 4区 医学
Clinics and research in hepatology and gastroenterology Pub Date : 2026-10-16 DOI: 10.1016/j.clinre.2026.102941
Keyu Chen, Wenjin Xie, Hong Zheng, Huaquan Yuan, Hao Xiong, Yu Gan, Zhihong Wu, Linlin Chen
{"title":"The role of mechanosensitive ion channel Piezo1 in the intestinal barrier function changes and related gastrointestinal disease.","authors":"Keyu Chen, Wenjin Xie, Hong Zheng, Huaquan Yuan, Hao Xiong, Yu Gan, Zhihong Wu, Linlin Chen","doi":"10.1016/j.clinre.2026.102941","DOIUrl":"https://doi.org/10.1016/j.clinre.2026.102941","url":null,"abstract":"<p><p>Intestinal barrier integrity is fundamental to the maintenance of body homeostasis. The mechanosensitive ion channel Piezo1, acting as a pivotal hub for mechanical signal transduction, converts mechanical stimuli into biochemical signals and plays a central role in the regulation of the intestinal barrier. This review systematically delineates the basic structure and kinetic properties of Piezo1 and comprehensively elaborates on its macro-regulatory functions across the multidimensional network of the intestinal mechanical, chemical, biological, and immune barriers. Furthermore, it investigates the mechanistic association between Piezo1-mediated mechanical signaling abnormalities and the pathogenesis of digestive system barrier dysfunction disorders, as well as potential targeted intervention strategies. By summarizing current research on Piezo1 and intestinal barrier function, this review aims to provide novel perspectives for elucidating the pathogenesis and advancing clinical targeted therapies for intestinal barrier dysfunction disorders.</p>","PeriodicalId":10424,"journal":{"name":"Clinics and research in hepatology and gastroenterology","volume":" ","pages":"102941"},"PeriodicalIF":2.6,"publicationDate":"2026-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149206693","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Prior Decompensation Shapes the Clinical Phenotype and the Prognostic Performance of Biochemical Liver-Failure Criteria in Hospitalised Cirrhosis patients: A Multicentre Study. 先前失代偿影响住院肝硬化患者生化肝功能衰竭标准的临床表型和预后表现:一项多中心研究
IF 2.6 4区 医学
Clinics and research in hepatology and gastroenterology Pub Date : 2026-10-16 DOI: 10.1016/j.clinre.2026.102940
Akash Roy, Arun Valsan, Karan Kumar, Pasham Satwika, Gowripriya Nair, Arathi Venu, Sowmya Iyengar, Manasa Alla, Shantan Venishetty, Mithun Sharma, Vivek Saraswat, Mahesh Goenka, Nagaraj Rao Padaki, D Nageshwar Reddy, Anand V Kulkarni
{"title":"Prior Decompensation Shapes the Clinical Phenotype and the Prognostic Performance of Biochemical Liver-Failure Criteria in Hospitalised Cirrhosis patients: A Multicentre Study.","authors":"Akash Roy, Arun Valsan, Karan Kumar, Pasham Satwika, Gowripriya Nair, Arathi Venu, Sowmya Iyengar, Manasa Alla, Shantan Venishetty, Mithun Sharma, Vivek Saraswat, Mahesh Goenka, Nagaraj Rao Padaki, D Nageshwar Reddy, Anand V Kulkarni","doi":"10.1016/j.clinre.2026.102940","DOIUrl":"https://doi.org/10.1016/j.clinre.2026.102940","url":null,"abstract":"<p><strong>Background and aims: </strong>Index and recurrent decompensation have different impacts on the course of acute decompensation (AD). Contemporary biochemical liver failure definitions for acute-on-chronic liver failure (ACLF) do not distinguish between index and prior decompensation. We evaluated whether prior decompensation defines a clinically distinct phenotype and examined whether established biochemical liver failure criteria retain consistent prognostic performance across these phenotypes METHODS: This multicentre study evaluated 966 adults hospitalized with AD across four centres in India, stratified into first-presentation (n = 364) and prior-decompensation (n = 602) cohorts. Biochemical thresholds from APASL (bilirubin ≥5 mg/dL and INR ≥1.5), CLEARED (≥7.5 mg/dL and INR≥1.5), and EASL-CLIF (bilirubin ≥12 mg/dL) were evaluated against 30-day mortality.</p><p><strong>Results: </strong>Patients with prior decompensation were older, more often had MASLD-related cirrhosis (21.3% vs 10.7%), and showed greater extra-hepatic organ failures. Thirty-day mortality was higher in patients with prior decompensation independent of MELD and baseline infection (40.5% vs 30.5%; adjusted RR 1.31, 95% CI 1.10-1.56, p=0.003), largely attributable to hepatic encephalopathy, which was twice as common (60% vs 29%). In the overall cohort, APASL and CLEARED modestly predicted mortality (OR 1.36 and 1.41, respectively), whereas EASL-CLIF threshold did not. In decompensation phenotype-stratified analysis, prognostic performance varied; biochemical thresholds were associated with 30-day mortality only among prior decompensation (APASL: OR 1.57; CLEARED: OR 1.67; CLIF-C: OR 1.71), but not in the first-presentation phenotype. The bilirubin-only CLIF-C OF liver-failure component captured the fewest deaths and showed significant phenotype-dependent prognostic behaviour.</p><p><strong>Conclusion: </strong>Prior decompensation defines a clinically distinct, high-risk phenotype mediated by encephalopathy. Liver-failure criteria perform discordantly across phenotypes with different prognostic signals in first and prior decompensations.</p>","PeriodicalId":10424,"journal":{"name":"Clinics and research in hepatology and gastroenterology","volume":" ","pages":"102940"},"PeriodicalIF":2.6,"publicationDate":"2026-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149206640","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Separating Prognostic Association from Treatment-Effect Modification of a Baseline Mucosal Inflammatory-Response Score in Ulcerative Colitis Biologic Trials. 溃疡性结肠炎生物学试验中基线粘膜炎症反应评分的预后关联与治疗效果改变的分离
IF 2.6 4区 医学
Clinics and research in hepatology and gastroenterology Pub Date : 2026-10-16 DOI: 10.1016/j.clinre.2026.102943
Mengyao Jiang, Qingxi Meng, Linghan Xue
{"title":"Separating Prognostic Association from Treatment-Effect Modification of a Baseline Mucosal Inflammatory-Response Score in Ulcerative Colitis Biologic Trials.","authors":"Mengyao Jiang, Qingxi Meng, Linghan Xue","doi":"10.1016/j.clinre.2026.102943","DOIUrl":"https://doi.org/10.1016/j.clinre.2026.102943","url":null,"abstract":"<p><strong>Background and aims: </strong>Baseline mucosal inflammation may be associated with outcome without modifying treatment benefit. We separated these questions for a fixed inflammatory-response score in ulcerative colitis biologic studies.</p><p><strong>Methods: </strong>A fixed 200-gene score was calculated with platform-specific probe mapping and accession-level baseline standardization. Treatment-by-score interactions were estimated in three randomized active-versus-placebo datasets. Prognostic associations were synthesized across seven treated cohorts, longitudinal change across six paired cohorts, and cellular localization using donor-aware single-cell summaries.</p><p><strong>Results: </strong>The randomized branch included 681 participants. All three interaction odds ratios were below one (0.64, 0.46, and 0.76), but their 95% confidence intervals were wide and crossed one. Among 541 actively treated participants, higher baseline scores were associated with poorer outcome (pooled odds ratio 0.44, 0.33-0.58). Among 154 paired participants, responders had a lower baseline-adjusted follow-up score (-1.07 standardized units, -1.61 to -0.53). Analysis of 212,623 cells localized the programme across epithelial, myeloid, and fibroblast/pericyte compartments. Baseline compartment analyses included only 18-19 donors and did not establish a remission association.</p><p><strong>Conclusions: </strong>Across the analysed public datasets, baseline mucosal inflammation showed a consistent adverse prognostic association and response-associated decline. The randomized evidence did not establish treatment-effect modification, and prospective independent validation is required before clinical use.</p>","PeriodicalId":10424,"journal":{"name":"Clinics and research in hepatology and gastroenterology","volume":" ","pages":"102943"},"PeriodicalIF":2.6,"publicationDate":"2026-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149206644","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
"Clip-with-dental floss" traction as a valuable ERCP bailout for externalization of a challenging intradiverticular papilla. “夹牙线”牵引作为一种有价值的ERCP救助的挑战性的垂直内乳头外化。
IF 2.6 4区 医学
Clinics and research in hepatology and gastroenterology Pub Date : 2026-10-16 DOI: 10.1016/j.clinre.2026.102942
Vincent Zimmer
{"title":"\"Clip-with-dental floss\" traction as a valuable ERCP bailout for externalization of a challenging intradiverticular papilla.","authors":"Vincent Zimmer","doi":"10.1016/j.clinre.2026.102942","DOIUrl":"https://doi.org/10.1016/j.clinre.2026.102942","url":null,"abstract":"","PeriodicalId":10424,"journal":{"name":"Clinics and research in hepatology and gastroenterology","volume":" ","pages":"102942"},"PeriodicalIF":2.6,"publicationDate":"2026-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149206662","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Liver transplantation for adult presentations of MDR3 deficiency. 肝移植治疗成人MDR3缺乏症。
IF 2.6 4区 医学
Clinics and research in hepatology and gastroenterology Pub Date : 2026-10-16 DOI: 10.1016/j.clinre.2026.102944
Jeremy S Nayagam, Samantha M-A Campbell, Yooyun Chung, Michael A Heneghan, Deepak Joshi
{"title":"Liver transplantation for adult presentations of MDR3 deficiency.","authors":"Jeremy S Nayagam, Samantha M-A Campbell, Yooyun Chung, Michael A Heneghan, Deepak Joshi","doi":"10.1016/j.clinre.2026.102944","DOIUrl":"https://doi.org/10.1016/j.clinre.2026.102944","url":null,"abstract":"<p><strong>Background: </strong>MDR3 deficiency is associated with end-stage liver disease including liver failure, portal hypertension and primary liver cancer, with increasing recognition of adult presentations. We report our single centre experience of liver transplantation (LT) for adult presentations of MDR3 deficiency.</p><p><strong>Methods: </strong>A single centre retrospective study of patients who underwent LT for MDR3 deficiency from 2013-2024 and were first diagnosed above the age of 16 years. We identified demographic and clinical data from the electronic patient records.</p><p><strong>Results: </strong>There were 7 patients with genetically confirmed MDR3 deficiency who underwent LT, including 3 (43%) females, and median age at LT was 31.9 years (range, 25-37). Severity of liver disease at assessment: median bilirubin 266 µmol/L (range, 38-726 µmol/L) and MELD 22 (range, 11-38). Due to severity of liver disease and clinical trajectory, organ allocation was prioritised in 4 (57%) patients, either internally or nationally. All patients had at least 1 suspected aetiology of liver disease prior to the diagnosis of MDR3 deficiency. Only 4 (57%) were diagnosed with MDR3 deficiency at time of LT assessment, and patients with a known diagnosis of MDR3 deficiency were assessed for LT at an earlier stage of liver disease.</p><p><strong>Conclusions: </strong>Patients with MDR3 deficiency can be assessed for LT at advanced stages of liver disease, and the diagnosis may be reached after transplant. Genetic testing can facilitate and accurate diagnosis and allow personalised management. The timing of LT assessment needs to be considered carefully in MDR3 deficiency.</p>","PeriodicalId":10424,"journal":{"name":"Clinics and research in hepatology and gastroenterology","volume":" ","pages":"102944"},"PeriodicalIF":2.6,"publicationDate":"2026-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149206635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Genetic hepatocellular cholestasis: an update. 遗传性肝细胞胆汁淤积症:最新进展。
IF 2.6 4区 医学
Clinics and research in hepatology and gastroenterology Pub Date : 2026-10-14 DOI: 10.1016/j.clinre.2026.102939
Antoine Gardin, William Pouillot, Marion Almes, Charlotte Mussini, Emmanuel Gonzales, Emmanuel Jacquemin
{"title":"Genetic hepatocellular cholestasis: an update.","authors":"Antoine Gardin, William Pouillot, Marion Almes, Charlotte Mussini, Emmanuel Gonzales, Emmanuel Jacquemin","doi":"10.1016/j.clinre.2026.102939","DOIUrl":"https://doi.org/10.1016/j.clinre.2026.102939","url":null,"abstract":"<p><p>Cholestasis, defined as a reduction of bile flow, may be secondary to obstruction of bile ducts or absence of production of bile by hepatocytes. Genetic hepatocellular cholestasis is a heterogenous group of diseases, including bile acid synthesis defects (BASD) and progressive familial intrahepatic cholestasis (PFIC). It affects a gene involved in either bile acid synthesis (BASD) or bile secretion by hepatocytes (PFIC). Recent advances in molecular biology and sequencing techniques have enabled the identification of many novel cholestatic disorders beyond the three historical PFIC. These diseases, of variable severity, are responsible for chronic cholestasis, intractable pruritus and often progress toward early liver cirrhosis, liver failure and liver cancer. Novel therapeutics such as ileal bile acid transporter inhibitors have been developed for the treatment of pruritus. Many patients with genetic hepatocellular cholestasis will require liver transplantation during childhood because of intractable pruritus or worsening of liver function. Specific subsets of BASD manifesting as chronic cholestasis are treatable with primary bile acid therapy with excellent native liver survival in the long-term. In this publication, we will review the clinical, biochemical and molecular features of genetic hepatocellular cholestasis, as well as their management.</p>","PeriodicalId":10424,"journal":{"name":"Clinics and research in hepatology and gastroenterology","volume":" ","pages":"102939"},"PeriodicalIF":2.6,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149179629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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