Journal of Clinical and Experimental Hepatology最新文献

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Endoscopic Transpapillary Gallbladder Stenting for Complicated Cholecystitis in Patients With Cirrhosis and High Surgical Risk: An Observational Study 内镜下经乳头胆囊支架置入术治疗肝硬化和高手术风险患者的复杂胆囊炎:一项观察性研究
IF 3.2
Journal of Clinical and Experimental Hepatology Pub Date : 2026-03-01 Epub Date: 2025-12-31 DOI: 10.1016/j.jceh.2025.103462
Arka De, Arpit Shastri, Sweta Rose, Sahaj Rathi, Arwinder Singh, Ganesh Cp, Naveen Bhagat, Babulal Meena, Nipun Verma, Madhumita Premkumar, Sunil Taneja, Ajay Duseja
{"title":"Endoscopic Transpapillary Gallbladder Stenting for Complicated Cholecystitis in Patients With Cirrhosis and High Surgical Risk: An Observational Study","authors":"Arka De,&nbsp;Arpit Shastri,&nbsp;Sweta Rose,&nbsp;Sahaj Rathi,&nbsp;Arwinder Singh,&nbsp;Ganesh Cp,&nbsp;Naveen Bhagat,&nbsp;Babulal Meena,&nbsp;Nipun Verma,&nbsp;Madhumita Premkumar,&nbsp;Sunil Taneja,&nbsp;Ajay Duseja","doi":"10.1016/j.jceh.2025.103462","DOIUrl":"10.1016/j.jceh.2025.103462","url":null,"abstract":"<div><h3>Background</h3><div>Patients with cholecystitis and underlying cirrhosis represent a high-risk group for surgical intervention. In patients otherwise meriting an endoscopic retrograde cholangiopancreatography (ERCP), endoscopic transpapillary gallbladder stenting (ETGS) can serve as a bridge to cholecystectomy while awaiting liver transplant or provide long-term gallbladder (GB) drainage in those who are not surgical candidates. We evaluated the technical feasibility, clinical outcomes and safety of ETGS for complicated cholecystitis in patients with cirrhosis and high surgical risk.</div></div><div><h3>Methods</h3><div>Data of all adult cirrhotic patients with complicated cholecystitis who underwent ETGS between January 2021 and March 2025 were reviewed. Primary indication for ERCP was choledocholithiasis with or without cholangitis. Primary outcome was technical success of ETGS (successful deployment of at least one GB stent). Secondary outcomes included procedure time, safety, clinical success (symptom resolution with improvement in laboratory parameters within 72-h of stent insertion), symptom recurrence, 6-month and 12-month biliary event-free survival, and differences in outcomes among patients with one or two GB stents.</div></div><div><h3>Results</h3><div>Among 40 patients (age: 56.9 ± 14.3 years, 62.5% females, model for end-stage liver disease score: 16.4) with cirrhosis taken up for ETGS, technical success was achieved in 34 (85%) patients. Among those with technical success, clinical success was achieved in 33 (97%) patients. One and two 7-Fr double-pigtail plastic stents were deployed in 13 (38.2%) and 21 (61.8%) patients, respectively. Elective stent exchanges were not done. The median follow-up duration was 13.5 (11–18) months. One (2.9%) patient had recurrent cholecystitis at 5 months. The 6- and 12-month biliary event-free survival rates were 94% and 86.7%, respectively. No significant difference in clinical success, symptom recurrence, or 6- and 12-month survival was observed among patients with one or two stents. Mild (grade I as per the Adverse events GastRointEstinal Endoscopy classification) procedure-related adverse events occurred in two (5%) patients.</div></div><div><h3>Conclusion</h3><div>ETGS is a technically feasible and safe, therapeutic option for complicated cholecystitis in cirrhotic patients with high clinical success and favourable intermediate-term outcomes.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"16 2","pages":"Article 103462"},"PeriodicalIF":3.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146022668","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Endovascular Interventions for the Secondary Prophylaxis of Gastric Varices Prevent Rebleeding but May Not Improve Overall Survival: A Real-world Cohort From Two Centers 胃静脉曲张二级预防的血管内干预预防再出血,但可能不能提高总生存率:来自两个中心的现实世界队列
IF 3.2
Journal of Clinical and Experimental Hepatology Pub Date : 2026-03-01 Epub Date: 2025-11-21 DOI: 10.1016/j.jceh.2025.103416
Sagnik Biswas , Santhosh E. Kumar , Sanchita Gupta , Pegatraju K. Bharadwaj , Shekhar Swaroop , Santhosh B. KB , Anuradha Sharma , Arnav Aggarwal , Umang Arora , Sarthak Saxena , Amitkumar Chavan , Rajkumar Bayye , Samagra Agarwal , Deepak Gunjan , Rahul Kumar , Shyamkumar N. Keshava , Shivanand Gamanagatti , Ashish Goel , Shalimar
{"title":"Endovascular Interventions for the Secondary Prophylaxis of Gastric Varices Prevent Rebleeding but May Not Improve Overall Survival: A Real-world Cohort From Two Centers","authors":"Sagnik Biswas ,&nbsp;Santhosh E. Kumar ,&nbsp;Sanchita Gupta ,&nbsp;Pegatraju K. Bharadwaj ,&nbsp;Shekhar Swaroop ,&nbsp;Santhosh B. KB ,&nbsp;Anuradha Sharma ,&nbsp;Arnav Aggarwal ,&nbsp;Umang Arora ,&nbsp;Sarthak Saxena ,&nbsp;Amitkumar Chavan ,&nbsp;Rajkumar Bayye ,&nbsp;Samagra Agarwal ,&nbsp;Deepak Gunjan ,&nbsp;Rahul Kumar ,&nbsp;Shyamkumar N. Keshava ,&nbsp;Shivanand Gamanagatti ,&nbsp;Ashish Goel ,&nbsp;Shalimar","doi":"10.1016/j.jceh.2025.103416","DOIUrl":"10.1016/j.jceh.2025.103416","url":null,"abstract":"<div><h3>Background</h3><div>There is limited literature on the long-term outcomes of patients with cirrhosis following an index gastric variceal (GV) hemorrhage.</div></div><div><h3>Methods</h3><div>Patients with cirrhosis and hemorrhage from gastroesophageal varices type-2 (GOV-2) or isolated gastric varices type-1 (IGV-1) from two tertiary care centers over 8 years were retrospectively analyzed. All patients underwent endoscopic cyanoacrylate injection (ECI) for primary hemostasis. Modalities of secondary prophylaxis included endoscopic surveillance and beta-blockers (ECI and BBs), balloon-occluded retrograde transvenous obliteration (BRTO), and transjugular intrahepatic portosystemic shunt (TIPS). The incidence of rebleeding, mortality, and further decompensation among patients receiving ECI and BB was estimated and compared with a propensity score-matched (PSM) cohort of patients undergoing BRTO or TIPS.</div></div><div><h3>Results</h3><div>Three hundred and fifty-one patients were recruited. Of those 275 received ECI and BB, 54 underwent BRTO, and 22 underwent TIPS. The rebleeding rate at 1 year after ECI and BB was 19.2%, while the 1-year mortality and further decompensation rates were 19.7% and 27.8%, respectively. Rebleeding predominantly occurred from GV (76.1% of cases), with the cumulative 1-, 3-, and 5-year all-cause rebleeding rate among patients receiving ECI alone being 19.2%, 30%, and 35%, respectively. BRTO and TIPS significantly reduced all-cause rebleeding, but were associated with similar rates of further decompensation and mortality compared to ECI and BB in the PSM cohort.</div></div><div><h3>Conclusion</h3><div>BRTO and TIPS significantly reduce all-cause rebleeding as compared to ECI and BB, but their impact on further decompensation and overall survival requires further evaluation in larger cohorts.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"16 2","pages":"Article 103416"},"PeriodicalIF":3.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145735931","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Outgoing Editor-in-Chief: The Admirable Admiral 即将离任的总编:可敬的海军上将
IF 3.2
Journal of Clinical and Experimental Hepatology Pub Date : 2026-03-01 Epub Date: 2026-02-19 DOI: 10.1016/j.jceh.2026.103506
Pankaj Puri
{"title":"The Outgoing Editor-in-Chief: The Admirable Admiral","authors":"Pankaj Puri","doi":"10.1016/j.jceh.2026.103506","DOIUrl":"10.1016/j.jceh.2026.103506","url":null,"abstract":"","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"16 2","pages":"Article 103506"},"PeriodicalIF":3.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147537520","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Septic Shock Management in Cirrhosis: Beyond “One-Size-Fits-All” Resuscitation With POCUS-Guided Precision 肝硬化脓毒性休克的管理:超越“一刀切”的复苏与pocus引导的精度
IF 3.2
Journal of Clinical and Experimental Hepatology Pub Date : 2026-03-01 Epub Date: 2026-02-11 DOI: 10.1016/j.jceh.2026.103500
Nagasri Shankar, Kavish R. Patidar
{"title":"Septic Shock Management in Cirrhosis: Beyond “One-Size-Fits-All” Resuscitation With POCUS-Guided Precision","authors":"Nagasri Shankar,&nbsp;Kavish R. Patidar","doi":"10.1016/j.jceh.2026.103500","DOIUrl":"10.1016/j.jceh.2026.103500","url":null,"abstract":"","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"16 2","pages":"Article 103500"},"PeriodicalIF":3.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147538541","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Characterization of Both CD24+ and CD24- T Cells can Better Elucidate the Influence of CD24 on Cytokine Production CD24+和CD24- T细胞的表征可以更好地阐明CD24对细胞因子产生的影响
IF 3.2
Journal of Clinical and Experimental Hepatology Pub Date : 2026-03-01 Epub Date: 2026-01-01 DOI: 10.1016/j.jceh.2025.103467
Zhencheng Zhang, Zaixing Yang
{"title":"Characterization of Both CD24+ and CD24- T Cells can Better Elucidate the Influence of CD24 on Cytokine Production","authors":"Zhencheng Zhang,&nbsp;Zaixing Yang","doi":"10.1016/j.jceh.2025.103467","DOIUrl":"10.1016/j.jceh.2025.103467","url":null,"abstract":"","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"16 2","pages":"Article 103467"},"PeriodicalIF":3.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146022670","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Cortex in Context: Making Sense of Hepatic Encephalopathy as a Prognostic Factor in Cirrhosis-Related Hospitalization 背景下的皮质:肝性脑病作为肝硬化相关住院的预后因素的意义
IF 3.2
Journal of Clinical and Experimental Hepatology Pub Date : 2026-03-01 Epub Date: 2026-02-18 DOI: 10.1016/j.jceh.2026.103502
Adam Buckholz , Elliot B. Tapper
{"title":"The Cortex in Context: Making Sense of Hepatic Encephalopathy as a Prognostic Factor in Cirrhosis-Related Hospitalization","authors":"Adam Buckholz ,&nbsp;Elliot B. Tapper","doi":"10.1016/j.jceh.2026.103502","DOIUrl":"10.1016/j.jceh.2026.103502","url":null,"abstract":"","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"16 2","pages":"Article 103502"},"PeriodicalIF":3.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147538539","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Simple Laboratory-based Machine Learning Model Accurately Predicts Advanced Liver Fibrosis in Metabolic Dysfunction-associated Steatotic Liver Disease Patients 一个简单的基于实验室的机器学习模型准确预测代谢功能障碍相关脂肪变性肝病患者的晚期肝纤维化
IF 3.2
Journal of Clinical and Experimental Hepatology Pub Date : 2026-03-01 Epub Date: 2025-12-10 DOI: 10.1016/j.jceh.2025.103438
Shobha Sharma , Ashok Choudhury , Venkata S. Gupta Thadikemalla , Archana Rastogi , Tapan Gandhi , Shiv K. Sarin
{"title":"A Simple Laboratory-based Machine Learning Model Accurately Predicts Advanced Liver Fibrosis in Metabolic Dysfunction-associated Steatotic Liver Disease Patients","authors":"Shobha Sharma ,&nbsp;Ashok Choudhury ,&nbsp;Venkata S. Gupta Thadikemalla ,&nbsp;Archana Rastogi ,&nbsp;Tapan Gandhi ,&nbsp;Shiv K. Sarin","doi":"10.1016/j.jceh.2025.103438","DOIUrl":"10.1016/j.jceh.2025.103438","url":null,"abstract":"<div><h3>Background/Aim</h3><div>Metabolic dysfunction-associated steatotic liver disease (MASLD) is often silent and progressive, affecting nearly one-fourth of the global population. However, liver biopsy remains the only accurate but invasive modality for diagnosis. We aimed to develop a machine-learning model to assess liver fibrosis in MASLD patients.</div></div><div><h3>Method</h3><div>We retrospectively analyzed electronic medical records of biopsy-proven MASLD patients, incorporating demographic, clinical, and liver biopsy data (advanced fibrosis, F3/F4 as AF). Fourteen machine learning (ML) models were developed, with the best model compared against the fibrosis-4 (FIB-4) score and aspartate aminotransferase to platelet ratio index (APRI) for detecting AF.</div></div><div><h3>Results</h3><div>Among 452 patients (median age: 41 years; interquartile range (IQR) 33–49; 75% male), 222 (49.1%) had AF. The fibrosis assessment by extra trees classifier (FAET) demonstrated the best performance, achieving 84% accuracy, an area under the curve (AUC) of 0.82, an F1 score of 0.77, a negative predictive value (NPV) of 0.81, and a positive predictive value (PPV) of 0.91 using 13 features. Compared to FIB-4, FAET improved accuracy by 33%, AUC by 34%, F1 score by 45%, NPV by 15.7%, and PPV by 71%. Similarly, compared to APRI, FAET improved accuracy by 58.4%, AUC by 49%, F1 score by 45.2%, NPV by 20.8%, and PPV by 111%. Using the top five features, the model achieved an accuracy of 82%, an AUC of 0.80, an F1 score of 0.76, a PPV of 0.79, and an NPV of 0.83 on the external test set, enabling the development of a simplified web-based tool.</div></div><div><h3>Conclusion</h3><div>FAET offers a significant advancement in the noninvasive assessment of advanced fibrosis in MASLD patients and has the potential for routine use pending further validation.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"16 2","pages":"Article 103438"},"PeriodicalIF":3.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145921876","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Peripheral Venous Access for Low-Volume Centrifugal Plasma Exchange to Treat Patients With Liver Disorders 小容量离心血浆置换治疗肝脏疾病患者的外周静脉通路
IF 3.2
Journal of Clinical and Experimental Hepatology Pub Date : 2026-03-01 Epub Date: 2026-01-07 DOI: 10.1016/j.jceh.2025.103465
Asisha M. Janeela , Gayathiri K. Chellaiya , Rohan Thomas , Alok Bansal , Snehil Kumar , Gnanadeepam Sunderraj , Sumathy Jayaraman , Santhosh E. Kumar , Vijay Alexander , Vinoi G. David , Santosh Varughese , Dolly Daniel , Sukesh C. Nair , Chundamannil E. Eapen , Ashish Goel , Uday G. Zachariah
{"title":"Peripheral Venous Access for Low-Volume Centrifugal Plasma Exchange to Treat Patients With Liver Disorders","authors":"Asisha M. Janeela ,&nbsp;Gayathiri K. Chellaiya ,&nbsp;Rohan Thomas ,&nbsp;Alok Bansal ,&nbsp;Snehil Kumar ,&nbsp;Gnanadeepam Sunderraj ,&nbsp;Sumathy Jayaraman ,&nbsp;Santhosh E. Kumar ,&nbsp;Vijay Alexander ,&nbsp;Vinoi G. David ,&nbsp;Santosh Varughese ,&nbsp;Dolly Daniel ,&nbsp;Sukesh C. Nair ,&nbsp;Chundamannil E. Eapen ,&nbsp;Ashish Goel ,&nbsp;Uday G. Zachariah","doi":"10.1016/j.jceh.2025.103465","DOIUrl":"10.1016/j.jceh.2025.103465","url":null,"abstract":"<div><h3>Background/Aims</h3><div>Centrifugal plasma exchange (PLEX), by virtue of low blood flow rates, can be performed via peripheral venous access. This study aims to assess the utilization, safety, and efficiency of low-volume centrifugal plasma exchange via peripheral venous access (P-PLEX) in liver disease patients.</div></div><div><h3>Methods</h3><div>This retrospective cohort study compared patients with liver disease who underwent low-volume centrifugal P-PLEX (2019–2024) with syndrome-matched patients who underwent plasma exchange via central venous access (C-PLEX). Data on PLEX procedure, venous access, and extraction efficiency for molecules of interest were noted.</div></div><div><h3>Results</h3><div>Of 448 liver disease patients who underwent centrifugal low-volume PLEX, 81 patients (18.1%) who underwent P-PLEX (M: 60; age: 37 [29.5–46.5] years; median, interquartile range) were compared with 81 syndrome-matched patients who underwent C-PLEX (M: 62; age: 37 [29.5–46.5] years; acute-on-chronic liver failure: 44, acute liver injury/acute liver failure: 21, others: 20). Targeted P-PLEX sessions were completed in 67 of 81 (82%). P-PLEX access was mostly 18-G (n = 64/72 for inlet/return) in antecubital fossa. Thirteen of 81 (16%) P-PLEX patients required access change to C-PLEX. Procedure time was longer in P-PLEX group (105 [82.5–120] min) than in the C-PLEX group (75 [60–91.3] min, <em>P</em> value&lt; 0.001) due to lower flow rates in P-PLEX. When controlled for exchange volumes and baseline values, extraction efficiency of bilirubin (P-PLEX: 34.3% [21.2–42.6], C-PLEX: 27.1% [10.3–41.7]; <em>P</em> value: 0.14) and von Willebrand factor antigen (P-PLEX: 36.1% [21.8–46.8], C-PLEX: 45.6% [32.8–58.3]; <em>P</em> value: 0.19) were similar in both groups. PLEX was performed in the ward (in high monitoring area) in majority of patients in the P-PLEX group (73 [90.1%]) and in the high-dependency unit (43 [53.1%]) in the C-PLEX group. Lower line-related complications with P-PLEX (2/81) vis-à-vis C-PLEX (9/81, <em>P</em>-value: 0.03) were noted.</div></div><div><h3>Conclusion</h3><div>In this report, low-volume centrifugal PLEX to treat liver disease was performed via peripheral venous access in 18% of patients. P-PLEX was done in the ward, with similar efficiency and better line-related safety; however, the PLEX duration was prolonged by 30 min.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"16 2","pages":"Article 103465"},"PeriodicalIF":3.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146074224","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Bortezomib Therapy in Autoimmune-BSEP Disease After Liver Transplantation: Case Report With Review of the Literature 硼替佐米治疗肝移植后自身免疫性bsep疾病:病例报告并文献复习
IF 3.2
Journal of Clinical and Experimental Hepatology Pub Date : 2026-03-01 Epub Date: 2025-12-09 DOI: 10.1016/j.jceh.2025.103418
Rohan Grotra , Prasenjit Das , Rajni Yadav , Lalita Mehra , Ashok Tiwari , Rohan Malik
{"title":"Bortezomib Therapy in Autoimmune-BSEP Disease After Liver Transplantation: Case Report With Review of the Literature","authors":"Rohan Grotra ,&nbsp;Prasenjit Das ,&nbsp;Rajni Yadav ,&nbsp;Lalita Mehra ,&nbsp;Ashok Tiwari ,&nbsp;Rohan Malik","doi":"10.1016/j.jceh.2025.103418","DOIUrl":"10.1016/j.jceh.2025.103418","url":null,"abstract":"<div><div>Progressive familial intrahepatic cholestasis type 2 (PFIC-2) is a rare autosomal recessive disorder caused by mutations in the <em>ABCB11</em> gene, which encodes the bile salt export pump (BSEP). Liver transplantation is the standard treatment for end-stage liver disease in PFIC-2; however, recurrence in the form of autoimmune BSEP disease (AIBD) is a recognized posttransplant complication. AIBD is characterized by cholestasis with normal gamma-glutamyl transpeptidase, severe pruritus, and the presence of anti-BSEP antibodies. We report a case of a PFIC-2 patient who developed recurrent cholestasis and pruritus three years posttransplant. Liver biopsy revealed canalicular cholestasis and giant cell transformation, while BSEP staining on immunohistochemistry was preserved. Serological testing confirmed anti-BSEP antibody positivity. Despite aggressive therapy including plasmapheresis, intravenous immunoglobulin, and rituximab, only partial improvement was achieved, and cholestasis persisted. Given the refractory nature of the disease, bortezomib—a proteasome inhibitor targeting antibody-producing plasma cells—was administered. The patient demonstrated complete clinical and biochemical resolution following bortezomib therapy. This case highlights the diagnostic complexity of AIBD and the potential role of bortezomib in cases unresponsive to conventional immunosuppressive therapies. Early diagnosis and personalized immunomodulation remain key to improving outcomes in this rare but challenging condition.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"16 2","pages":"Article 103418"},"PeriodicalIF":3.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145881175","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Collagen Proportionate Area and Histological Grading of Fibrosis as Predictors of Clinical Outcomes and Mortality in Patients with Steatotic Liver Disease 胶原比例面积和纤维化组织学分级作为脂肪变性肝病患者临床结局和死亡率的预测因子
IF 3.2
Journal of Clinical and Experimental Hepatology Pub Date : 2026-03-01 Epub Date: 2026-01-02 DOI: 10.1016/j.jceh.2025.103464
Andreas Bartholdy , Pernille Y. Nielsen , Lise L. Gluud , Laust H. Mortensen , Birgitte M. Viuff , Elisabeth D. Galsgaard , Majken K. Jensen
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