17,209 results match your criteria: "Primary Biliary Cirrhosis"

Evaluation of Cardiac Function in Children Undergoing Liver Transplantation.

Pediatr Cardiol

November 2024

Division of Pediatric Cardiology, Children's Hospital at Montefiore, Albert Einstein College of Medicine, 3415 Bainbridge Ave-R1, Bronx, NY, 10467, USA.

Article Synopsis
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Elafibranor alleviates alcohol-related liver fibrosis by restoring intestinal barrier function.

World J Gastroenterol

November 2024

Jiangsu Key Laboratory for Pharmacology and Safety Evaluation of Chinese Materia Medica, Nanjing University of Chinese Medicine, Nanjing 210023, Jiangsu Province, China.

We discuss the article by Koizumi published in the . Our focus is on the therapeutic targets for fibrosis associated with alcohol-related liver disease (ALD) and the mechanism of action of elafibranor (EFN), a dual agonist of peroxisome proliferator-activated receptor α (PPARα) and peroxisome PPAR δ (PPARδ). EFN is currently in phase III clinical trials for the treatment of metabolic dysfunction-associated fatty liver disease and primary biliary cholangitis.

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Seladelpar: First Approval.

Drugs

November 2024

Springer Nature, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Seladelpar (LIVDELZI) is an oral delpar [i.e. a selective peroxisome proliferator-activated receptor (PPAR)δ agonist] being developed by Gilead Sciences for the treatment of primary biliary cholangitis (PBC).

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Article Synopsis
  • - Bile acid transporters (BATs) are important proteins that help move bile acids in the body, especially in the liver, kidney, and small intestine, and are linked to fat absorption and liver function.
  • - Altered levels of bile acids are associated with cancer, but the specific role of BATs in cancer progression—like tumor growth and spread—has not been fully explored yet.
  • - This review suggests that researching BATs further could lead to new cancer treatments, especially for liver and gastrointestinal cancers, and encourages targeting these transporters with different techniques for potential therapies.
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Background: Bowel colonization with antimicrobial-resistant bacteria has been associated with worse clinical outcomes in patients with cirrhosis; however, it has not been studied in patients with acute-on-chronic liver failure (ACLF). We evaluated whether fecal isolation of carbapenem-resistant gram-negative bacteria (CR-GNB) among patients with ACLF affects short-term outcomes.

Methods: Patients of APASL-ACLF (n = 339) were screened between June 2020 and December 2021, and 150 were included.

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Anesthesia management for total robotic liver transplantation: Inaugural case series in Europe.

Ann Hepatobiliary Pancreat Surg

November 2024

Department of Anaesthesiology, Local Health Unit of São José, Lisbon, Portugal.

Robotic liver transplantation represents a cutting-edge technique that may surpass traditional open surgery. Nonetheless, it introduces unique anesthetic challenges, including extended pneumoperitoneum, restricted patient access, and a risk of undetected blood loss. This article describes an anesthetic approach and patient outcomes for the first four total robotic liver transplants performed at a tertiary university hospital in Portugal, along with inaugural procedures of their kind in Europe.

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Background: Current biliary dilatation (BD) classifications are complex and based on cases including secondary BD, leading to unclear distinctions. Notably, congenital and secondary BD differ in etiology, symptoms, and prognosis.

Objective: To propose a more concise and more suitable classification of congenital biliary dilatation (CBD), and exploring the feasibility and effectiveness of this classification in diagnosis and treatment.

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BACKGROUND In living-donor liver transplantation, biliary complications are considered an Achilles' heel. Consequently, various attempts have been made to reduce their incidence, and multiple innovations in surgical techniques have been reported. We herein report a case involving an intraoperative ultrasound cholangiogram in the recipient's abdominal cavity after reperfusion of the graft.

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Background And Aims: The pathological characteristics of lymphocyte infiltration in the hepatic portal tracts of patients with primary biliary cholangitis (PBC) remain unclear. Tertiary lymphoid structures (TLSs) are ectopic lymphoid tissues associated with the exacerbation of autoimmune reactions. Here, we evaluate the role of TLSs in PBC and investigate their potential therapeutic value.

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Article Synopsis
  • * The International Multicenter Pediatric Portal Hypertension Registry (IMPPHR) was created to gather data on pediatric mortality from variceal hemorrhage and strategies for prevention.
  • * IMPPHR was initiated following discussions at pediatric portal hypertension meetings, began in 2020, and aims to collect data from over 700 subjects across 44 centers until data closure in 2024.
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Primary biliary cholangitis (PBC) and gut microbiota (GM) are epidemiologically correlated but the causal inter-relationships remain poorly understood. We aim to explore the causal relationships between GM and PBC. Using the MiBioGen consortium, GWAS data for GM at the species level and the largest publicly available PBC GWAS data to date, we performed a bidirectional two-sample Mendelian randomization by the inverse variance weighted, MR-Egger, weighted median, weighted model and MR-PRESSO to elucidate the potential causal role of GM in PBC.

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Since the Asian Pacific Association for the Study of the Liver (APASL) published guidelines on non-cirrhotic portal fibrosis/idiopathic portal hypertension in 2007, there has been a surge in new information, especially with the introduction of the term porto-sinusoidal vascular disorder (PSVD). Non-cirrhotic intra-hepatic causes of portal hypertension include disorders with a clearly identifiable etiology, such as schistosomiasis, as well as disorders with an unclear etiology such as non-cirrhotic portal fibrosis (NCPF), also termed idiopathic portal hypertension (IPH). This entity is being increasingly recognized as being associated with systemic disease and drug therapy, especially cancer therapy.

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Background: The clinical course of esophagogastric varices (EGV) after sustained virological response (SVR) with direct-acting antiviral (DAA) therapy has not been clearly elucidated. The predictors for the worsening/improvement of EGV after SVR with DAA therapy were investigated.

Methods: Of the cirrhosis patients who achieved SVR with DAA therapy, 328 patients who underwent endoscopic examinations both before and after DAA therapy were enrolled.

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Mouse Models for the Study of Liver Fibrosis Regression and .

J Clin Transl Hepatol

November 2024

Division of Endocrinology, Department of Medicine, Leiden University Medical Center, Leiden, The Netherlands.

This review discussed experimental mouse models used in the pre-clinical study of liver fibrosis regression, a pivotal process in preventing the progression of metabolic dysfunction-associated steatohepatitis to irreversible liver cirrhosis. These models provide a valuable resource for understanding the cellular and molecular processes underlying fibrosis regression in different contexts. The primary focus of this review is on the most commonly used models with diet- or hepatotoxin-induced fibrosis, but it also touches upon genetic models and mouse models with biliary atresia or parasite-induced fibrosis.

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Generation of human induced pluripotent stem cell lines derived from patients of cystic biliary atresia.

Hum Cell

November 2024

iPS Cell Advanced Characterization and Development Team, BioResource Research Center, RIKEN, 3-1-1 Koyadai, Tsukuba, Ibaraki, 305-0074, Japan.

Article Synopsis
  • Biliary atresia (BA) is a serious liver condition caused by abnormal development of bile ducts, leading to liver damage and possible cirrhosis; type I cystic BA is a rare but important form of this condition.
  • Researchers have created a set of human induced pluripotent stem cells (hiPSCs) from five Japanese patients with type I cystic BA, which show potential for self-renewal and differentiation.
  • Whole genome analysis revealed mutations linked to hepatobiliary issues in these patients, and studying these hiPSCs may help improve understanding of the disease and lead to new treatments.
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Matching the opposites: liver transplantation from a situs viscerum inversus totalis donor.

Updates Surg

November 2024

Hepato-Pancreato-Biliary Surgery and Liver Transplantation Unit, University Hospital of Modena "Policlinico", University of Modena and Reggio Emilia, 41124, Modena, Italy.

Situs viscerum inversus totalis (SIT) is a rare congenital anomaly. Deceased donors with this condition are often declined because of the technical issues in both the organ's procurement and its transplant. Only eight cases of deceased donor organs with SIT were reported to be used for liver transplantation (LT).

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[Clinical characteristics and prognosis analysis of patients with IgG4-related hepatobiliary-pancreatic disease].

Zhonghua Gan Zang Bing Za Zhi

October 2024

Division of Gastroenterology and Hepatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Institute of Digestive Disease, Shanghai200001, China.

To explore and analyze the clinical features of patients with immunoglobulin (Ig)G4-related hepatobiliary-pancreatic disease and the independent factors affecting the prognosis of IgG4-related sclerosing cholangitis (IgG4-SC). The clinical data of 179 adult cases diagnosed with IgG4-related hepato-pancreato-biliary disease in the Division of Gastroenterology and Hepatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine from January 2011 to December 2022 were retrospectively analyzed. Patients were divided into three groups: isolated IgG4-SC, IgG4-SC/type 1 autoimmune pancreatitis(type 1 AIP), and isolated AIP according to the clinical manifestations.

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Backgrounds: Malnutrition and sarcopenia are prevalent complications in cirrhosis. The relationship between sarcopenia and biliary infection in cirrhotic patients is not well understood. Our study aims to clarify this association.

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Cross-sectional study on the diagnostic significance of plasma exosomal miRNAs in HBV-related hepatocellular carcinoma.

J Transl Med

November 2024

Department of Hepatic-Biliary-Pancreatic Surgery, Guangzhou First People's Hospital, South China University of Technology, Guangzhou, Guangdong, China.

Purpose: Hepatocellular carcinoma (HCC) associated with Hepatitis B Virus (HBV) is one of the most severe malignancies in East Asia, where early diagnosis is crucial for improving patient prognosis. So we aim to identify effective early diagnostic model for HCC.

Design And Methods: We enrolled 108 early-stage HCC patients and 102 non-HCC individuals underlying HBV infection, collecting plasma exosomal miRNAs (exo-miRNAs) from all participants.

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Article Synopsis
  • - This study compared the effectiveness of Two-dimensional shear wave elastography (2D-SWE) and transient elastography (TE) in assessing esophagogastric varices risk and histological staging in patients with primary biliary cholangitis (PBC).
  • - Both methods showed similar diagnostic abilities for determining histological stages and detecting esophagogastric varices, with no significant differences in their effectiveness, although 2D-SWE had slightly better accuracy.
  • - The results indicate that 2D-SWE is just as reliable as TE for predicting risks associated with PBC, with minimal impact from laboratory values like transaminases and bilirubin on the measurements.
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  • Nonalcoholic fatty liver disease (NAFLD) can lead to severe liver issues and the study investigated how palmitic acid (PA), a common dietary fat, affects liver cells using organ-on-a-chip technology.
  • After exposure to palmitic acid, the liver cells showed a decrease in liver transcription factor activity and expression changes in 318 genes, indicating early signs of liver cell dedifferentiation.
  • Despite these changes, there was no lipid accumulation in the cells, but an increase in collagen production was observed, suggesting that palmitic acid contributes to the early stages of lipotoxicity associated with NAFLD.
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Obesity and risk for liver disease: a two-sample Mendelian randomisation study.

Br J Nutr

November 2024

Department of Gastroenterology, Beijing Ditan Hospital, Capital Medical University, Beijing100015, People's Republic of China.

Article Synopsis
  • * Findings showed that higher genetically predicted BMI and WHRadjBMI were linked to non-alcoholic fatty liver disease, liver fibrosis, and autoimmune hepatitis, but not to other liver conditions like primary biliary cholangitis or liver cancer.
  • * The research suggested that obesity has distinct causal effects on certain liver diseases and metabolic functions, particularly non-alcoholic fatty liver disease and liver fibrosis, but not on viral or autoimmune liver diseases.
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Background: End-stage liver diseases (ESLDs) are a significant global health challenge due to their high prevalence and severe health impacts. Despite the severe outcomes associated with ESLDs, therapeutic options remain limited. Targeting the activation of hepatic stellate cells (HSCs), key drivers of extracellular matrix accumulation during liver injury presents a novel therapeutic approach.

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Article Synopsis
  • - Biliary atresia (BA) is a severe liver condition in infants that causes inflammation and blockage of bile ducts, leading to cirrhosis within two years if untreated.
  • - Early diagnosis is crucial, and the best initial treatment is the Kasai procedure, which should be performed within the first two months of life to maximize the chances of success.
  • - The outcomes of the Kasai procedure vary, with potential long-term complications requiring liver transplant; pathologists need to differentiate between various liver damage patterns in successful versus failed surgeries for accurate diagnosis.
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