Article
Multidisciplinary Sciences
Shogo Kitahata, Yasunori Yamamoto, Osamu Yoshida, Yoshio Tokumoto, Tomoe Kawamura, Shinya Furukawa, Teru Kumagi, Masashi Hirooka, Eiji Takeshita, Masanori Abe, Yoshiou Ikeda, Yoichi Hiasa
Summary: The study investigated alterations in the small intestinal mucosa-associated microbiota (MAM) in patients with primary biliary cholangitis (PBC), revealing an overgrowth of Sphingomonadaceae and Pseudomonas that may be associated with the pathogenesis and pathological development of PBC.
SCIENTIFIC REPORTS
(2021)
Article
Gastroenterology & Hepatology
Julian Hercun, Mazen Noureddin, Nabil Noureddin, Jason Eccleston, Daniel Woolridge, T. Jake Liang, Michele Tana, David E. Kleiner, Gracia Viana Rodriguez, Christopher Koh, Jay H. Hoofnagle, Theo Heller
Summary: This study evaluated the involvement of bile duct and disease progression in patients with primary biliary cholangitis (PBC) and found that bile duct injury does not follow the same course as fibrosis, and certain indicators are associated with ductopenia progression.
AMERICAN JOURNAL OF GASTROENTEROLOGY
(2022)
Article
Multidisciplinary Sciences
Xi Li, Yan Li, Jintao Xiao, Huiwen Wang, Yan Guo, Xiuru Mao, Pan Shi, Yanliang Hou, Xiaoxun Zhang, Nan Zhao, Minghua Zheng, Yonghong He, Jingjing Ding, Ya Tan, Min Liao, Ling Li, Ying Peng, Xuan Li, Qiong Pan, Qiaoling Xie, Qiao Li, Jianwei Li, Ying Li, Zhe Chen, Yongxiu Huang, David N. Assis, Shi-Ying Cai, James L. Boyer, Xuequan Huang, Can-E Tang, Xiaowei Liu, Shifang Peng, Jin Chai
Summary: The numbers of DUOX2(+)ACE2(+) small cholangiocytes are inversely associated with disease severity in PBC. They may be the target of pIgR-mediated humoral responses. Protecting these cells and targeting anti-pIgR autoantibodies could be valuable strategies for therapeutic interventions in PBC.
NATURE COMMUNICATIONS
(2023)
Article
Gastroenterology & Hepatology
Debashis Haldar, Ashnila Janmohamed, Tim Plant, Matthew Davidson, Hannah Norman, Emily Russell, Olivia Serevina, Kenneth Chung, Kashif Qamar, Bridget Gunson, Bettina Hansen, Alex Richter, Palak J. Trivedi, Gideon M. Hirschfield
Summary: In patients with PBC, the presence of anti-gp210 autoantibodies is associated with adverse clinical manifestations, predicts treatment non-response, and independently predicts decreased transplant-free survival.
LIVER INTERNATIONAL
(2021)
Article
Gastroenterology & Hepatology
Thomas W. Warnes, Stephen A. Roberts, Alexander Smith, Victor M. Cope, Patricia Vales, Raymond McMahon
Summary: This study found that measurement of baseline portal pressure is of significant prognostic value in primary biliary cholangitis, providing additional predictive information beyond Mayo score and Ludwig stage.
LIVER INTERNATIONAL
(2023)
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, Research & Experimental
Hao Li, Yanling Guan, Chenchen Han, Yu Zhang, Qian Liu, We Wei, Yang Ma
Summary: Primary biliary cholangitis (PBC) is an autoimmune disease characterized by the destruction of intrahepatic small bile ducts, presence of antimitochondrial antibody (AMA), leading to liver fibrosis and cirrhosis. Genetic predisposition, environmental factors, epitopes exposure, and autoimmune tolerance imbalance contribute to the occurrence of PBC. Animal models play a crucial role in studying the pathogenesis and drug development of PBC.
BIOMEDICINE & PHARMACOTHERAPY
(2021)
Article
Medicine, Research & Experimental
Nidhi Jalan-Sakrikar, Abid Anwar, Usman Yaqoob, Can Gan, Anthony B. Lagnado, Alexander Q. Wixom, Diana Jurk, Robert C. Huebert
Summary: This study investigated the mechanistic relationship between telomere damage and biliary fibrosis in primary sclerosing cholangitis (PSC). The results showed telomere attrition and reduced telomerase reverse transcriptase (TERT) expression in patients with PSC. Cellular models demonstrated that telomere dysfunction can lead to senescence and fibrosis, which can be rescued by modulating TERT levels.
Article
Gastroenterology & Hepatology
Makayla Watt, Ashley Hyde, Emily Johnson, Gail M. Wright, Shauna Vander Well, Chikku Sadasivan, Dayna Lee-Baggley, John C. Spence, Andrew Mason, Hin Hin Ko, Edward Tam, Puneeta Tandon
Summary: This study aimed to evaluate the impact of an online mind-body intervention on mental and physical symptoms in patients with primary biliary cholangitis. The results showed significant improvements in depression, perceived stress, and certain indicators of quality of life in the intervention group. Although no improvements in fatigue were observed in the main analysis, a significant benefit was found in a subgroup of intervention participants who adhered to the mind-body video routine.
HEPATOLOGY COMMUNICATIONS
(2023)
Review
Immunology
Yanling Zhao, Shizhang Wei, Lisheng Chen, Xuelin Zhou, Xiao Ma
Summary: Primary biliary cirrhosis (PBC) is a chronic liver disease characterized by persistent cholestasis and immune response. The pathogenesis of PBC is not fully understood, but research has made progress in understanding mechanisms such as bile acid metabolism and autoimmune response. Current clinical treatments are insufficient, and new treatment options are urgently needed. Drug candidates targeting specific PBC mechanisms are emerging, but multitarget drugs may hold promise for future PBC drug development.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Genetics & Heredity
Bin Ke, Chunyu Li, Huifang Shang
Summary: Hematologic abnormalities are associated with the susceptibility of primary biliary cholangitis (PBC), but whether there is a causal association is still unclear. In this study, we conducted analyses to explore the causative role of hematological traits in the risk of PBC. The results showed that genetically higher hemoglobin and hematocrit levels are associated with a reduced risk of PBC, providing potential targets for disease prevention and treatment.
JOURNAL OF HUMAN GENETICS
(2023)
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
Mark R. Pedersen, Garrett Greenan, Sumant Arora, Arvind R. Murali, Marlyn J. Mayo
Summary: UDCA after LT reduces the odds of BC and bile stones and sludge in all-comer LT recipients and reduces or delays the incidence of rPBC in patients transplanted for PBC. UDCA use after LT could be considered in all LT recipients to reduce the odds of BC and may be particularly beneficial for patients transplanted for PBC by reducing the incidence of rPBC.
LIVER TRANSPLANTATION
(2021)
Article
Gastroenterology & Hepatology
Naw April Phaw, Jessica Katharine Dyson, George Mells, David Jones
Summary: This study identified potential discrete subgroups of fatigued patients with and without cognitive symptoms in primary biliary cholangitis. Patients with fatigue accompanied by cognitive symptoms were younger, more likely to have severe fatigue, and experienced greater social and emotional impact, representing a coherent cohort for clinical trials.
DIGESTIVE DISEASES AND SCIENCES
(2021)
Review
Gastroenterology & Hepatology
Vasiliy Ivanovich Reshetnyak, Igor Veniaminovich Maev
Summary: Primary biliary cholangitis (PBC) is a chronic liver disease characterized by progressive damage to cholangiocytes and intrahepatic cholestasis. Accumulation of bile acids in biliary epithelial cells (BECs) is likely to be associated with their damage. The impaired production of bicarbonate (HCO3-) due to epigenetic changes in expression of microRNA 506 in BECs can lead to uncontrolled bile acid intake into BECs. This review focuses on understanding the molecular mechanisms underlying the development of PBC.
WORLD JOURNAL OF GASTROENTEROLOGY
(2023)