Article
Gastroenterology & Hepatology
Kristel K. Leung, Gideon M. Hirschfield
Summary: Testing for autoantibodies is crucial for diagnosing PBC, evaluating related disease processes, and identifying potential prognostic factors. Further characterization of autoantibody profiles in PBC will contribute to our understanding of the pathophysiological mechanisms, as well as provide new insights into risk stratification and therapeutic targets.
CLINICS IN LIVER DISEASE
(2022)
Review
Gastroenterology & Hepatology
Eirini Rigopoulou, Dimitrios P. Bogdanos
Summary: Primary biliary cholangitis (PBC) is a chronic liver disease characterized by immune-mediated destruction of small bile ducts, which can progress to hepatic failure. Early diagnosis and ursodeoxycholic acid treatment have been shown to prevent disease progression in most cases. The presence of disease-specific autoantibodies, including anti-mitochondrial antibodies, anti-sp100 antibodies, and anti-gp210 antibodies, is used for the diagnosis of PBC. These autoantibodies not only aid in the diagnosis of the disease but also have prognostic value. This article summarizes the current knowledge on the detection and clinical significance of PBC-related autoantibodies and discusses the potential prognostic role of novel autoantibodies in PBC patients.
WORLD JOURNAL OF GASTROENTEROLOGY
(2023)
Review
Gastroenterology & Hepatology
Juan Trivella, Binu V. John, Cynthia Levy
Summary: Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by destructive cholangitis and the presence of antimitochondrial antibodies. The prevalence of PBC varies globally and may be higher in males and racial minorities than previously thought. Early recognition and treatment are crucial for PBC outcomes, which can be influenced by provider bias and socioeconomic factors. Ursodeoxycholic acid is the initial treatment choice, with other drugs being considered as add-on therapy. This article provides a comprehensive summary of the epidemiology, prognosis, and treatment of PBC for holistic management.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Chemistry, Multidisciplinary
Diana Ferreira, Cristiana Neto, Jose Lopes, Julio Duarte, Antonio Abelha, Jose Machado
Summary: In this study, different Data Mining techniques were compared to predict the survival of patients with Primary Biliary Cholangitis. The model using the Random Forest classifier and Split Validation method performed the best, accurately predicting patient survival rate.
APPLIED SCIENCES-BASEL
(2022)
Review
Biochemistry & Molecular Biology
Annarosa Floreani, Daniela Gabbia, Sara De Martin
Summary: Primary biliary cholangitis (PBC) is a rare autoimmune liver disease with limited treatment options. Ursodeoxycholic acid (UDCA) is the first-line therapy but is ineffective for a significant portion of patients. Obeticholic acid (OCA) is an effective second-line treatment for UDCA-non responders. Future therapies for PBC are promising.
Article
Gastroenterology & Hepatology
Jessica Katharine Dyson, Alasdair Blain, Mark David Foster Shirley, Mark Hudson, Steven Rushton, David Emrys Jeffreys Jones
Summary: This study investigates the potential environmental contributors to autoimmune liver diseases (AILD) such as primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Significant spatial clustering of PBC and PSC patients was observed, with distinct risk profiles and associations with different environmental factors and socio-economic statuses identified. This suggests different triggers and alternative pathways determine the phenotypic expression of autoimmunity in the affected population.
Article
Gastroenterology & Hepatology
Annarosa Floreani, Daniela Gabbia, Sara De Martin
Summary: Primary biliary cholangitis (PBC) is a chronic liver disease characterized by immune-mediated destruction of intrahepatic bile ducts. It is predominantly seen in females and is associated with autoimmune conditions. The pathogenesis involves both immune response to autoantigens and a defect in biliary umbrella, leading to the abnormal composition of bile. This review provides an up-to-date analysis of the pathogenesis of PBC.
EXPERT REVIEW OF GASTROENTEROLOGY & HEPATOLOGY
(2023)
Article
Gastroenterology & Hepatology
Valentina Guatibonza-Garcia, Paula Valentina Gaete, Agustin Perez-Londono, Danna Kathalina Puerto-Baracaldo, Sebastian Antonio Gutierrez-Romero, Carlos O. Mendivil, Monica Tapias
Summary: This study assessed the performance of autoantibodies for diagnosing PBC in Latin American individuals, finding a very low positivity rate for AMA and limited performance of all antibodies, highlighting the urgent need for better PBC biomarkers.
WORLD JOURNAL OF GASTROENTEROLOGY
(2021)
Article
Immunology
Yi-jun Zhou, Gao-xiang Ying, Shi-lei Dong, Bo Xiang, Qiao-fei Jin
Summary: The gut microbiota of PBC patients from Zhejiang Province showed lower diversity and specific alterations compared to healthy controls. Six specific genera were identified as optimal biomarkers for the diagnosis of PBC. The changes in gut microbiota were associated with lipid metabolism and biosynthesis of secondary metabolites.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Immunology
Xiaoli Zeng, Siting Li, Shiyi Tang, Xi Li, Guoyuan Zhang, Mengtao Li, Xiaofeng Zeng, Chaojun Hu
Summary: This study investigated the glycosylation profile of serum IgG in PBC patients using high-throughput lectin microarrays technology, revealing distinct glycan differences in different PBC subgroups positive for various autoantibodies. The altered levels of glycosylation may be related to the occurrence and development of the disease.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Ji-Won Park, Jung-Hee Kim, Sung-Eun Kim, Jang Han Jung, Myoung-Kuk Jang, Sang-Hoon Park, Myung-Seok Lee, Hyoung-Su Kim, Ki Tae Suk, Dong Joon Kim
Summary: Cholangiopathies are various biliary diseases that affect the biliary epithelium and can lead to cholestasis and liver cirrhosis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most important progressive cholangiopathies in adults, with research focusing on genetic risk, epigenetic changes, dysregulated mucosal immunity, and altered biliary epithelial cell function. Understanding the molecular pathogenesis of these diseases can help clinicians prevent and treat them more effectively.
Review
Medicine, General & Internal
Francesco Martini, Daniele Balducci, Martina Mancinelli, Valerio Buzzanca, Elena Fracchia, Giuseppe Tarantino, Antonio Benedetti, Marco Marzioni, Luca Maroni
Summary: Primary Biliary Cholangitis (PBC) is a liver disease with a varied presentation, progression, and response to treatment. Current risk assessment relies on analyzing demographic data, clinical findings, antibody profiles, and liver fibrosis evaluation to identify patients at higher risk of disease progression. The response to therapy after one year remains a significant factor influencing the prognosis of PBC patients. Tailored treatment strategies are emerging to replace the one-size-fits-all approach, requiring the development of risk prediction models to assess patients' course, outcome, and response to treatment at the time of diagnosis. This review provides an overview of current and emerging risk stratification tools and speculates on their potential impact on PBC management.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Medicine, General & Internal
Panagiotis A. Papamichalis, Kalliopi Zachou, Roidoula A. Papamichali, Maria Ioannou, Nikolaos K. Gatselis, George N. Dalekos, George K. Koukoulis
Summary: The study evaluated the potential of detecting promyelocytic leukemia protein (PML) as a specific and sensitive marker for diagnosing PBC. Results showed that PML-score was significantly higher in PBC patients compared to controls, with high sensitivity and specificity for confirming PBC. Patients with higher PML-scores were typically older, more often female, and had elevated levels of ALP, γ-GT, and IgM.
JOURNAL OF TRANSLATIONAL INTERNAL MEDICINE
(2021)
Article
Immunology
Alicja Bauer, Andrzej Habior
Summary: This study found significantly elevated levels of MMP-3 in PBC patients, which were correlated with the state of liver fibrosis, suggesting that MMP-3 may play an important role in the pathophysiology of hepatic fibrosis in PBC.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Gastroenterology & Hepatology
Surain B. Roberts, Gideon M. Hirschfield, Lawrence J. Worobetz, Catherine Vincent, Jennifer A. Flemming, Angela Cheung, Karim Qumosani, Mark Swain, Dusanka Grbic, Hin Hin Ko, Kevork Peltekian, Nazia Selzner, Lusine Abrahamyan, Bishoi Aziz, Ellina Lytvyak, Kattleya Tirona, Aliya F. Gulamhusein, Harry L. A. Janssen, Aldo J. Montano-Loza, Andrew L. Mason, Bettina E. Hansen
Summary: In a diverse Canadian cohort of PBC patients, Indigenous Canadians have poorer liver transplant-free and event-free survival compared to White patients. They are more likely to have a clinical event before diagnosis and present with higher liver function test indicators, which persist during follow-up.
Article
Gastroenterology & Hepatology
Na Sun, Ceng Zhang, Yi-Te Lee, Benjamin V. Tran, Jing Wang, Hyoyong Kim, Junseok Lee, Ryan Y. Zhang, Jasmine J. Wang, Junhui Hu, Zhicheng Zhang, Manaf S. Alsudaney, Kuan-Chu Hou, Hubert Tang, Tiffany X. Zhang, Icy Y. Liang, Ziang Zhou, Mengxiang Chen, Angela Hsiao-Jiun Yeh, Wenyuan Li, Xianghong Jasmine Zhou, Helena R. Chang, Steven-Huy B. Han, Saeed Sadeghi, Richard S. Finn, Sammy Saab, Ronald W. Busuttil, Mazen Noureddin, Walid S. Ayoub, Alexander Kuo, Vinay Sundaram, Buraq Al-Ghaieb, Juvelyn Palomique, Kambiz Kosari, Irene K. Kim, Tsuyoshi Todo, Nicholas N. Nissen, Maria Lauda Tomasi, Sungyong You, Edwin M. Posadas, James X. Wu, Madhuri Wadehra, Myung-Shin Sim, Yunfeng Li, Hanlin L. Wang, Samuel W. French, Shelly C. Lu, Lily Wu, Renjun Pei, Li Liang, Ju Dong Yang, Vatche G. Agopian, Hsian-Rong Tseng, Yazhen Zhu
Summary: The study developed an HCC EV-based surface protein assay for early detection of HCC, showing promising potential in detecting early-stage HCC.
Article
Gastroenterology & Hepatology
Christopher L. Bowlus, Lionel Arrive, Annika Bergquist, Mark Deneau, Lisa Forman, Sumera I. Ilyas, Keri E. Lunsford, Mercedes Martinez, Gonzalo Sapisochin, Rachna Shroff, James H. Tabibian, David N. Assis
Article
Gastroenterology & Hepatology
Cynthia Levy, Stuart Kendrick, Christopher L. Bowlus, Atsushi Tanaka, David Jones, Andreas E. Kremer, Marlyn J. Mayo, Nazneen Haque, Robyn von Maltzahn, Matthew Allinder, Brandon Swift, Megan M. McLaughlin, Gideon M. Hirschfield, GLIMMER Study Grp
Summary: GLIMMER evaluated the dose-response, efficacy, and safety of linerixibat in the treatment of cholestatic pruritus associated with primary biliary cholangitis (PBC). The study found that linerixibat did not show significant difference in itch reduction compared to placebo in the primary intent-to-treat analysis, but it demonstrated a significant dose-dependent reduction in itch in the per protocol population. A well-tolerated dose was identified for Phase 3 investigation in the treatment of cholestatic pruritus in PBC.
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY
(2023)
Article
Environmental Sciences
Jiajun Ou, Junlin Wen, Wenbing Tan, Xiaoshan Luo, Jiexuan Cai, Xiaosong He, Lihua Zhou, Yong Yuan
Summary: Linear and nonlinear models were used to monitor the structure dependence of humic substances (HS) redox activities. The PLS model showed moderate ability, while the ANN model achieved higher performance. Sensitivity analysis confirmed the accuracy of the models and provided a potential approach for studying the structure-activity relationships of HS.
ENVIRONMENTAL RESEARCH
(2023)
Letter
Gastroenterology & Hepatology
Christopher L. Bowlus, Lionel Arrive, Annika Bergquist, Mark Deneau, Lisa Forman, Sumera I. Ilyas, Keri E. Lunsford, Mercedes Martinez, Gonzalo Sapisochin, Rachna Shroff, James H. Tabibian, David N. Assis
Review
Environmental Sciences
Chenghao Huang, Nannan Yuan, Xiaosong He, Changhui Wang
Summary: The use of ceramsite as a filtration material in water treatment is popular, and the development of low-cost, high-performance, and environmentally friendly ceramsites has gained attention. This study focuses on exploring the use of drinking water treatment residue (DWTR) to make ceramsite for water treatment. The results show that DWTR-based ceramsites can be fabricated using sintering and non-sintering methods. Further research is needed to evaluate the performance and potential for pollution control of DWTR-based ceramsite-constructed filtration systems, as well as the stability and safety of raw DWTR-based ceramsite.
JOURNAL OF ENVIRONMENTAL MANAGEMENT
(2023)
Article
Environmental Sciences
Xiaowei Wang, Hongwei Zhang, Xiaosong He, Jianfei Liu, Zhiliang Yao, Hongbin Zhao, Dayang Yu, Baosen Liu, Tianfu Liu, Weilong Zhao
Summary: Limited information on contamination of PFASs from agricultural activities in water sources. Investigated PFASs contamination in agricultural groundwater and nearby surface water in North China. Found higher PFAS concentrations in surface water, indicating migration from surface to groundwater. Found significant correlations between PFASs and organic carbon/nutrients in water. Health risk assessment showed no significant harm to residents in the studied area. Provided information on sources and migration characteristics of PFASs in a typical agricultural area of North China.
FRONTIERS IN ENVIRONMENTAL SCIENCE
(2023)
Article
Gastroenterology & Hepatology
Michael Trauner, Chuhan Chung, Kate Sterling, Xiangyu Liu, Xiaomin Lu, Jun Xu, Clare Tempany-Afdhal, Zachary D. Goodman, Martti Faerkkilae, Atsushi Tanaka, Palak Trivedi, Kris V. Kowdley, Christopher L. Bowlus, Cynthia Levy, Robert P. Myers
Summary: This article presents the background of PSC and the design and implementation of the phase 3 PRIMIS trial, which aims to evaluate the efficacy and safety of cilofexor in noncirrhotic patients with large-duct PSC. The primary objective is to assess whether cilofexor reduces the risk of fibrosis progression compared with placebo.
BMC GASTROENTEROLOGY
(2023)
Review
Environmental Sciences
Qi Pan, Qing-Yu Liu, Jing Zheng, Yan-Hong Li, Song Xiang, Xiao-Jie Sun, Xiao-Song He
Summary: Gas emitted from landfills contains a large quantity of volatile organic compounds (VOCs) and semi-volatile organic compounds (SVOCs), some of which are carcinogenic, teratogenic, and mutagenic. The global hazards of VOCs and SVOCs in landfill gas to human health remain unclear. To quantify the global risk distributions of these pollutants, the composition and concentration data of VOCs and SVOCs from 72 landfills in 20 countries were collected and their human health risks were assessed. Organic compounds in landfill gas primarily result from the biodegradation of natural organic waste or the emissions and volatilization of chemical products. The respiratory system, particularly the lung, was found to be the major target organ of VOCs and SVOCs, with additional adverse health impacts ranging from headache and allergies to lung cancer. Aromatic and halogenated compounds were the primary sources of health risk, while ethyl acetate and acetone from the biodegradation of natural organic waste also exceeded the acceptable levels for human health. Overall, VOCs and SVOCs affected residents within 1,000 m of landfills. Air temperature, relative humidity, air pressure, wind direction, and wind speed were the major factors that influenced the health risks of VOCs and SVOCs. Currently, landfill risk assessments of VOCs and SVOCs are primarily based on respiratory inhalation, with health risks due to other exposure routes remaining poorly elucidated. In addition, potential health risks due to the transport and transformation of landfill gas emitted into the atmosphere should be further studied.
ENVIRONMENT INTERNATIONAL
(2023)
Article
Gastroenterology & Hepatology
Gideon M. Hirschfield, Mitchell L. Shiffman, Aliya Gulamhusein, Kris Kowdley, John M. Vierling, Cynthia Levy, Andreas E. Kremer, Ehud Zigmond, Pietro Andreone, Stuart C. Gordon, Christopher L. Bowlus, Eric J. Lawitz, Richard J. Aspinall, Daniel S. Pratt, Karina Raikhelson, Maria S. Gonzalez-Huezo, Michael A. Heneghan, Sook-Hyang Jeong, Alma L. Ladron de Guevara, Marlyn J. Mayo, George N. Dalekos, Joost P. H. Drenth, Ewa Janczewska, Barbara A. Leggett, Frederik Nevens, Victor Vargas, Eli Zuckerman, Christophe Corpechot, Eduardo Fassio, Holger Hinrichsen, Pietro Invernizzi, Palak J. Trivedi, Lisa Forman, David E. J. Jones, Stephen D. Ryder, Mark G. Swain, Alexandra Steinberg, Pol F. Boudes, Yun-Jung Choi, Charles A. McWherter
Summary: This study evaluated the efficacy and safety of seladelpar in patients with primary biliary cholangitis who had inadequate response or intolerance to ursodeoxycholic acid. The results showed that seladelpar significantly improved liver biochemistry and pruritus in these patients, and it was well tolerated.
Article
Gastroenterology & Hepatology
David N. Assis, Christopher L. Bowlus
Summary: Primary sclerosing cholangitis (PSC) is a chronic liver disease characterized by fibroinflammatory damage to the biliary tree. It is often associated with inflammatory bowel disease and carries an increased risk of liver failure and cholangiocarcinoma. The complexity of the disease and lack of effective therapy have been challenges, but recent advancements in patient characterization, prognostication, and medical management offer hope for progress in the future. This review summarizes current understanding of PSC and highlights areas for further investigation.
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY
(2023)
Correction
Gastroenterology & Hepatology
Marlyn Mayo, Elizabeth T. Carey, Helen R. Smith, Andrea Mospan, Megan McLaughlin, April L. Thompson, Heather Morris, Robert Sandefur, W. Ray Kim, Christopher Bowlus, TARGET PBC Investigators, Cynthia Levy
DIGESTIVE DISEASES AND SCIENCES
(2023)
Editorial Material
Gastroenterology & Hepatology
Christopher L. Bowlus, Cynthia Levy
Article
Oncology
Al B. Benson, Michael I. D'Angelica, Thomas Abrams, Daniel E. Abbott, Aijaz Ahmed, Daniel A. Anaya, Robert Anders, Chandrakanth Are, Melinda Bachini, David Binder, Mitesh Borad, Christopher Bowlus, Daniel Brown, Adam Burgoyne, Jason Castellanos, Prabhleen Chahal, Jordan Cloyd, Anne M. Covey, Evan S. Glazer, William G. Hawkins, Renuka Iyer, Rojymon Jacob, Lawrence Jennings, R. Kate Kelley, Robin Kim, Matthew Levine, Manisha Palta, James O. Park, Steven Raman, Sanjay Reddy, Sean Ronnekleiv-Kelly, Vaibhav Sahai, Gagandeep Singh, Stacey Stein, Anita Turk, Jean-Nicolas Vauthey, Alan P. Venook, Adam Yopp, Nicole McMillian, Ryan Schonfeld, Cindy Hochstetler
Summary: In 2023, the NCCN Guidelines for Hepatobiliary Cancers were divided into two separate guidelines: Hepatocellular Carcinoma and Biliary Tract Cancers. The Biliary Tract Cancers guidelines provide recommendations for the evaluation and comprehensive care of patients with gallbladder cancer, intrahepatic cholangiocarcinoma, and extrahepatic cholangiocarcinoma. A multidisciplinary panel of experts meets regularly to review new data and therapies.
JOURNAL OF THE NATIONAL COMPREHENSIVE CANCER NETWORK
(2023)
Article
Multidisciplinary Sciences
Panagiotis Fotiadis, Matthew Cieslak, Xiaosong He, Lorenzo Caciagli, Mathieu Ouellet, Theodore D. Satterthwaite, Russell T. Shinohara, Dani S. Bassett
Summary: Recent work has found that the relationship between structural and functional connectivity in the human brain varies across different regions, with reduced connectivity observed in the sensory-association cortical hierarchy. This study suggests that intracortical myelination and excitation-inhibition balance play a role in mediating the heterogeneous expression and temporal variance of structure-function coupling.
NATURE COMMUNICATIONS
(2023)