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

Background: The growth hormone-insulin-like growth factor (GH-IGF-1) axis and its impairment with sarcopenia, frailty, bone health, complications, and prognosis are not well characterized in cirrhosis.

Methods: We investigated the adult decompensated cirrhosis out-patients at a tertiary care institute between 2021 and 2023 for serum GH and IGF-1 levels, and associated them with sarcopenia (CT-SMI in cm/m), liver frailty index (LFI), osteodystrophy (DEXA), clinical decompensations (overall, ascites, encephalopathy, infection, and bleed), and survival up to 180 days.

Results: One-hundred-seventy-two patients, 95% males, aged 46.

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The role of autotaxin (ATX)-lysophosphatidic acid (LPA) is yet to be explored in the context of liver cirrhosis and associated encephalopathy. Our objective of this study was to evaluate the role of an ATX inhibitor in biliary cirrhosis and associated hepatic encephalopathy in rats. The preliminary investigation revealed significant impairment in liver function, which eventually led to the development of hepatic encephalopathy.

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Objectives: Previous observational epidemiological studies have identified a potential association between inflammatory bowel disease (IBD) and sarcoidosis. Nonetheless, the precise biological mechanisms underlying this association remain unclear. Therefore, we adopted a Mendelian randomization (MR) approach to investigate the causal relationship between IBD with genetic susceptibility to sarcoidosis, as well as to explore the potential mediating role.

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Palmitic acid is the most abundant saturated fatty acid in circulation and causes hepatocyte toxicity and inflammation. As saturated fatty acid can also disrupt the circadian rhythm, the present work evaluated the connection between clock genes and NAD+ dependent Sirtuins in protecting hepatocytes from lipid-induced damage. Hepatocytes (immortal cells PH5CH8, hepatoma cells HepG2) treated with higher doses of palmitic acid (400-600μM) showed typical features of steatosis accompanied with growth inhibition and increased level of inflammatory markers (IL-6 IL-8, IL-1α and IL-1β) together with decline in NAD+ levels.

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Background: Liver and splenic stiffness measurements (LSM and SSM) are useful to predict varices and clinical decompensation in cirrhosis. SSM values are highly variable and overlapping and no guidelines exists on what constitutes normal SSM, that might limit interpretation of results.

Methods: Consecutive subjects with LSM < 6 kPa and reliable SSM (FibroScan630 Expert device with spleen-dedicated module) and no vascular liver disease were analysed for significant correlations of SSM values with age, sex, BMI, portal and splenic vein diameter, splenic diameter, liver fat and diabetes.

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Clinically significant portal hypertension in patients with primary biliary cholangitis: Clinicopathological features and prognostic value.

Ann Hepatol

September 2024

Department of Hepatology, Hepatology Research Institute, The First Affiliated Hospital, Fujian Medical University, Fuzhou 350005, Fujian Province, PR China; Department of Hepatology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou 350212, Fujian Province, PR China. Electronic address:

Introduction And Objectives: Primary biliary cholangitis (PBC) may progress to clinically significant portal hypertension (CSPH) before the development of cirrhosis. This study aimed to investigate CSPH incidence as well as the clinicopathological characteristics and predictive value of these features for the prognosis of patients with PBC, especially at early histologic stage.

Patients And Methods: Patients diagnosed with PBC between January 2013 and April 2022 were retrospectively enrolled.

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Central role for cholangiocyte pathobiology in cholestatic liver diseases.

Hepatology

September 2024

Department of Medicine, Division of Gastroenterology and Hepatology, Mayo College of Medicine and Science, Mayo Clinic, Rochester, Minnesota, USA.

Cholangiopathies comprise a spectrum of chronic intrahepatic and extrahepatic biliary tract disorders culminating in progressive cholestatic liver injury, fibrosis, and often cirrhosis and its sequela. Treatment for these diseases is limited, and collectively, they are one of the therapeutic "black boxes" in clinical hepatology. The etiopathogenesis of the cholangiopathies likely includes disease-specific mediators but also common cellular and molecular events driving disease progression (eg, cholestatic fibrogenesis, inflammation, and duct damage).

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Glutamine synthetase staining patterns in cirrhosis.

Hum Pathol

November 2024

Department of Pathology, University of California, San Francisco, CA, United States. Electronic address:

Advanced liver fibrosis can regress following the elimination of causative injuries. Glutamine synthetase (GS) immunohistochemical expression is normally in centrizonal perivenular hepatocytes but can be present in periportal hepatocytes in cases of regressed cirrhosis. This study identified periportal staining and investigated the spectrum of GS staining patterns seen in a range of cirrhotic livers with varying disease processes.

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Background: Infections have a poor prognosis in inpatients with cirrhosis. We aimed to determine regional variations in infections and their association with clinical outcomes in a global cohort of inpatients with cirrhosis.

Methods: In this prospective cohort study initiated by the CLEARED Consortium, we enrolled adults (aged >18 years) with cirrhosis who were non-electively admitted to 98 hospitals from 26 countries or regions across six continents between Nov 5, 2021, and Dec 10, 2022.

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Background: Progressive familial intrahepatic cholestasis is an autosomal recessive genetic disorder that manifests primarily with jaundice and pruritus and can progresses from persistent cholestasis to cirrhosis and late childhood liver failure. Classically, progressive familial intrahepatic cholestasis is classified into three subtypes: 1, 2, and 3 and results from a defect in a biliary protein responsible for bile formation and circulation in the liver. In the last decade and with the increased use of genetic testing, more types have been known.

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Article Synopsis
  • Obeticholic acid (OCA) is a medicine used for treating a liver disease called primary biliary cirrhosis.
  • Analyzing OCA in the body is tricky because it breaks down into different forms, which makes measuring it complicated.
  • Researchers created a new testing method to measure OCA and its two main breakdown products in blood, and it worked really well during studies on healthy people taking OCA tablets.
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Article Synopsis
  • A study was conducted to compare robotic minor liver resections (RMLR) with laparoscopic minor liver resections (LMLR) in patients undergoing surgery on the anterolateral liver segments.
  • The analysis included over 10,000 patients and employed propensity score matching to balance the groups for accuracy in comparisons.
  • Results indicated RMLR had benefits like less blood loss, lower major morbidity, and shorter hospital stays than LMLR, although the difference in 30-day readmission rates suggested RMLR may have some drawbacks.
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Background: Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease or portal hypertension, impacting the patient's quality of life and survival. There are still many gaps in the literature on this topic, especially in pediatrics, with practices frequently based on extrapolation of data obtained from adults.

Objective: Provide a synthesis of the current knowledge about HPS in children.

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Real-world data on the management of patients with primary biliary cholangitis (PBC) are so far scarce in Germany. Therefore, we aimed to establish a nationwide registry and describe the clinical characteristics and therapy of PBC patients.Three different cohorts defined as ursodeoxycholic acid (UDCA) responders, as inadequate responders according to Paris II criteria, and as newly diagnosed patients were prospectively recruited.

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Adeno-associated virus-based gene therapy (valoctocogene roxaparvovec) is an attractive treatment for hemophilia A. Careful clinical management is required to minimize the risk of hepatotoxicity, including assessment of baseline liver condition to determine treatment eligibility and monitoring liver function after gene therapy. This article describes recommendations (developed by a group of hemophilia experts) on hepatic function monitoring before and after gene therapy.

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Primary biliary cholangitis.

Lancet

September 2024

Department of Medicine and Surgery, Section of Gastroenterology, Baylor College of Medicine, Houston, TX, USA; Hepatology, and Division of Abdominal Transplantation, Baylor College of Medicine, Houston, TX, USA.

Article Synopsis
  • - Primary biliary cholangitis (PBC) is a chronic autoimmune disease primarily affecting women aged 40-70, and its incidence is rising globally while the gender ratio is shifting.
  • - The disease is linked to genetic predisposition and environmental triggers, such as certain chemicals, smoking, and changes in gut microbiome diversity, leading to complex interactions of immune cells and biliary epithelial cells.
  • - New treatments, including recently approved drugs and ongoing clinical trials, aim for better management of PBC symptoms, with future strategies focusing on achieving normal biochemical markers and improving quality of life for patients.
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Primary Biliary Cholangitis (PBC) is a chronic cholestatic liver disease typically diagnosed by elevated cholestatic liver enzymes and a positive anti-mitochondrial antibody (AMA) test. The clinical importance of AMA positivity in patients with normal cholestatic liver enzymes is unclear. The aim of this study was to determine the relationship between PBC and AMA positivity detected in individuals with normal cholestatic enzyme levels.

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Article Synopsis
  • The study investigates the causal relationship between primary biliary cholangitis (PBC) and systemic rheumatic diseases (SRDs) using Mendelian randomization analysis, to clarify previously observed associations.
  • Researchers utilized genetic data from established databases to identify genetic variants linked to PBC and assess their effects on several SRDs, including Sjögren syndrome and rheumatoid arthritis.
  • Findings reveal that PBC has significant causal effects on various SRDs, providing new insights into the connection between these conditions, with no evidence of horizontal pleiotropy affecting results.
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Associations between sleep disorders and clinical outcomes of patients with primary biliary cholangitis.

Adv Med Sci

September 2024

Department of Gastroenterology and Hepatology, General Hospital, Tianjin Medical University, Tianjin, China. Electronic address:

Purpose: Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by a range of symptoms, including sleep disturbances. The present study aimed to investigate the prevalence of sleep disorders and the associations between sleep disorders and clinical outcomes in PBC.

Patients And Methods: We enrolled 177 patients with PBC and 165 healthy controls (age- and sex-matched).

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Liver fibrosis is a chronic liver disease with progressive wound healing reaction caused by liver injury. Currently, there is no FDA approved drugs for liver fibrosis. Human adipose mesenchymal stem cells (hADSCs) have shown remarkable therapeutic effects in liver diseases.

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Functional liver imaging score (FLIS) can predict adverse events in HCC patients.

Eur J Radiol

November 2024

Department of Diagnostic Radiology, Fondazione IRCCS San Gerardo dei Tintori, Via Pergolesi 33, 20900 Monza, MB, Italy; Department of Medicine and Surgery, University of Milano Bicocca, Via Cadore 33, 20090 Monza, MB, Italy.

Article Synopsis
  • This study evaluated the effectiveness of the FLIS (Functional Liver Imaging Score) in predicting post-hepatectomy liver failure (PHLF) and death in patients who had liver surgery for cancer.
  • It involved 150 patients, with a high reliability in assessment results between radiologists, indicating solid agreement in the evaluation processes used.
  • The findings suggest that FLIS, along with factors like blood transfusions and major resections, may help identify patients at risk for adverse outcomes after surgery.
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Background: Chronic liver disease (CLD) in children, often leads to cirrhosis and end-stage liver disease (ESLD). CLD poses significant challenges in management and prognosis. Assessing body composition, including sarcopenia, is increasingly recognized as important in understanding outcomes in this population.

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Evaluation of Factors Affecting Rehospitalization and Survival After Living Donor Liver Transplantation.

Transplant Proc

September 2024

İstanbul Aydın University, Medikalpark Florya Hospital, Organ Transplantation Center, Küçükçekmece, İstanbul. Electronic address:

Background: Complications and comorbidities that may develop after living donor liver transplantation may necessitate rehospitalization after discharge. We aimed to investigate the demographic and clinical factors affecting rehospitalization after discharge.

Methods: Two hundred seventy patients who underwent living-donor liver transplantation (LDLT) for end-stage liver cirrhosis were included in the study.

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