15,842 results match your criteria Primary Biliary Cirrhosis

Diagnostic criteria for acute-on-chronic liver failure and related disease conditions in Japan.

Hepatol Res 2022 May;52(5):417-421

Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.

The Intractable Hepato-Biliary Disease Study Group of Japan, sponsored by the Ministry of Health, Labor and Wealth, proposed in 2018 that patients with cirrhosis and a Child-Pugh score of 5-9 should be diagnosed as having acute-on-chronic liver failure (ACLF) when a deterioration of liver function ("serum bilirubin level of 5.0 mg/dl or more" and "prothrombin time value of 40% or less of the standardized values and/or international normalization rates of 1.5 or more") caused by severe liver damage develops within 28 days after an acute insult, including alcohol abuse, bacterial infection, gastrointestinal bleeding, and the exacerbation of underlying liver diseases. Read More

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Osteosarcopenia in autoimmune cholestatic liver diseases: Causes, management, and challenges.

World J Gastroenterol 2022 Apr;28(14):1430-1443

Department of Biomedical Sciences, Humanitas University, Pieve Emanuele 20090, Milan, Italy.

Primary biliary cholangitis and primary sclerosing cholangitis (PSC) are the most common cholestatic liver diseases (CLD) in adults and are both characterized by an immune pathogenesis. While primary biliary cholangitis is a model autoimmune disease, with over 90% of patients presenting very specific autoantibodies against mitochondrial antigens, PSC is considered an immune mediated disease. Osteoporosis is the most common bone disease in CLD, resulting in frequent fractures and leading to significant morbidity. Read More

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What Makes Cirrhosis Irreversible?-Consideration on Structural Changes.

Front Med (Lausanne) 2022 27;9:876293. Epub 2022 Apr 27.

Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary.

Several studies have shown that liver fibrosis, and even cirrhosis can be reversed, disproving the old "dogma" that cirrhosis is irreversible. In addition to scaring, vascular alterations appear to be critically important in the progression of chronic liver diseases. To overcome the "tipping-point" of cirrhosis, we need to understand in depth what might make it irreversible in some cases. Read More

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MMP2/9 downregulation is responsible for hepatic function recovery in cirrhotic rats following associating liver partition and portal vein ligation for staged hepatectomy.

Ann Transl Med 2022 Apr;10(8):468

Department of Hepatobiliary, Pancreatic Surgical Oncology, The First Medical Center of Chinese PLA General Hospital, Beijing, China.

Background: Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) was introduced in 2007. The current study explored the mechanisms of rapid liver hypertrophy after ALPPS in cirrhotic rats.

Methods: A cirrhotic rat model was constructed and portal vein ligation (PVL) or ALPPS treatments were administered. Read More

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Partial External Biliary Diversion for Severe Diarrhea After Liver Transplant in Patients with Progressive Familial Intrahepatic Cholestasis Type 1.

Exp Clin Transplant 2022 May;20(Suppl 3):81-84

From the Department of Pediatric Gastroenterology, Gazi University, Ankara, Turkey.

Progressive familial intrahepatic cholestasis is a heterogeneous group of autosomal recessive disorders, and liver transplant is the only curative treatment. A biliary diversion operation for disruption of enterohepatic circulation in patients with progressive familial intrahepatic cholestasis type 1 without cirrhosis is another option. We present a pediatric patient with progressive familial intrahepatic cholestasis type 1 who underwent liver transplant due to end-stage liver disease. Read More

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MR elastography in primary sclerosing cholangitis: a pictorial review.

Abdom Radiol (NY) 2022 May 14. Epub 2022 May 14.

Department of Radiology, Mayo Clinic, Rochester, MN, USA.

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by biliary ductal inflammation and fibrosis causing both intrahepatic and extrahepatic biliary strictures and dilatation. There is currently no effective medical treatment and the disease leads to cirrhosis and liver failure, with patients often requiring liver transplantation in end-stage disease. Liver fibrosis is one of the most important factors in determining patient outcome in PSC, and the diagnosis and monitoring of fibrosis are vital to patient care. Read More

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The Effects of Taurocholic Acid on Biliary Damage and Liver Fibrosis Are Mediated by Calcitonin-Gene-Related Peptide Signaling.

Cells 2022 May 9;11(9). Epub 2022 May 9.

Department of Anatomical, Histological, Forensic Medicine and Orthopedics Sciences, Sapienza University of Rome, 00161 Rome, Italy.

Background & Aims: Cholangiocytes are the target cells of liver diseases that are characterized by biliary senescence (evidenced by enhanced levels of senescence-associated secretory phenotype, SASP, e.g., TGF-β1), and liver inflammation and fibrosis accompanied by altered bile acid (BA) homeostasis. Read More

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The Role of Epigenetics in Primary Biliary Cholangitis.

Int J Mol Sci 2022 Apr 28;23(9). Epub 2022 Apr 28.

Division of Gastroenterology, Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milano-Bicocca, 20900 Monza, Italy.

Primary Biliary Cholangitis (PBC) is a rare autoimmune disease of the liver, affecting mostly females. There is evidence that epigenetic changes have a pathogenic role in PBC. Epigenetic modifications are related to methylation of CpG DNA islands, post-translational modifications of histone proteins, and non-coding RNAs. Read More

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Assessment of Three-Phasic CT Scan Findings of Cirrhosis Due to Primary Sclerosing Cholangitis Versus Cryptogenic Cirrhosis.

Cureus 2022 Apr 8;14(4):e23956. Epub 2022 Apr 8.

Department of Radiology, Medical Imaging Research Center, Shiraz University of Medical Sciences, Shiraz, IRN.

Background: The CT findings of cirrhosis caused by primary sclerosing cholangitis (PSC) differ from cryptogenic cirrhosis. PSC could become complicated with biliary cirrhosis and cholangiocarcinoma. This study aimed at augmenting the information on the role of the three-phasic-abdominopelvic CT scan in PSC. Read More

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[Study of clinical characteristics in patients with gp210 antibody-positive primary biliary cholangitis].

Zhonghua Gan Zang Bing Za Zhi 2022 Apr;30(4):419-425

Department of Critical Hepatology, Beijing You'an Hospital Affiliated to Capital Medical University, Beijing 100069, China.

To analyze the clinical characteristics and prognostic value of liver function in a large samples of patients with anti-glycoprotein 210 (gp210 antibody) positive primary biliary cholangitis (PBC). A retrospective study was performed on 931 PBC cases in Beijing You'an Hospital affiliated to Capital Medical University from 2010 to 2019. According to the detection of gp210 antibody, 318 cases were divided into gp210 antibody positive group (positive group) and 613 cases were divided into gp210 antibody negative group (negative group). Read More

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Evaluation of the Integrative Weaning Index for Predicting the Outcome of Spontaneous Breathing Trial in Patients with Cirrhosis on Mechanical Ventilation: A Pilot Study.

Turk J Anaesthesiol Reanim 2022 Apr;50(2):107-113

Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.

Objective: Studies on mechanical ventilation in patients with cirrhosis have focused mainly on survival, as the disease is considered to carry a poor prognosis. The process of weaning in these patients has never been studied. With improving survival, it would be ideal to study the weaning indices that could add experience in clinical management. Read More

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Efficacy and safety of an open lung ventilation strategy with staircase recruitment followed by comparison on two different modes of ventilation, in moderate ARDS in cirrhosis: A pilot randomized trial.

Can J Respir Ther 2021 20;57:105-112. Epub 2021 Jul 20.

Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.

Background: Mechanical ventilation in cirrhosis with acute respiratory distress syndrome (ARDS) is not widely studied. We aimed to study the effect of the staircase recruitment manoeuvre followed by two different modes of ventilation.

Methods: Thirty patients with cirrhosis with moderate ARDS underwent the staircase recruitment manoeuvre followed by randomisation to volume control or pressure control group. Read More

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Hepatic Regeneration in Cirrhosis.

J Clin Exp Hepatol 2022 Mar-Apr;12(2):603-616. Epub 2021 Sep 4.

Department of Research, Institute of Liver and Biliary Sciences, New Delhi 110070, India.

End-stage liver disease is characterized by massive hepatocyte death resulting in clinical decompensation and organ failures. Clinical consequences in cirrhosis are the results of the loss of functional hepatocytes and excessive scarring. The only curative therapy in advanced cirrhosis is orthotropic liver transplantation, but the clinical demand outweighs the availability of acceptable donor organs. Read More

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September 2021

Giant Cell Hepatitis in Copper Toxicosis.

J Clin Exp Hepatol 2022 Mar-Apr;12(2):719-720. Epub 2021 Jul 16.

PedGastro, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, India.

Cholestatic jaundice in new born and infants results from biliary obstruction or hepatocellular dysfunction. Biliary atresia (BA) and Idiopathic neonatal hepatitis comprises the major aetiology. Cholestasis due to toxins is rare in infants. Read More

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Saroglitazar for Nonalcoholic Fatty Liver Disease: A Single Centre Experience in 91 Patients.

J Clin Exp Hepatol 2022 Mar-Apr;12(2):435-439. Epub 2021 Jun 19.

Institute of Liver, Gastroenterology, and Pancreatico-Biliary Sciences, Sir Ganga Ram Hospital, New Delhi, India.

Background: Saroglitazar is a novel, dual peroxisome proliferator-activated receptors-α/γ agonist and is being investigated for the treatment of nonalcoholic fatty liver disease (NAFLD).

Patients And Methods: Consecutive overweight (body mass index [BMI] >23 kg/m) patients of NAFLD, diagnosed based on controlled attenuation parameter (CAP) >248 dB/m, and attending the outpatient department of a tertiary care centre in New Delhi, were enrolled. Patients with cirrhosis (liver stiffness measurement [LSM] >13. Read More

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Progressive Familial Intrahepatic Cholestasis: Need for Genetic Analysis Before Liver Transplantation.

J Clin Exp Hepatol 2022 Mar-Apr;12(2):686-688. Epub 2021 Jun 18.

Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi 110070, India.

The clinical course after liver transplantation (LT) in progressive familial intrahepatic cholestasis type 1 (PFIC1) is complicated by intractable diarrhoea, growth failure, graft steatosis and cirrhosis. Recent evidence from Japan suggests the role of genotype to predict outcome after LT. We report a case with pathogenic frameshift mutation who had failed partial external biliary diversion, underwent LT and his post-LT course has been complicated by intractable diarrhoea, growth failure, steatosis and fibrosis. Read More

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Progressive Familial Intrahepatic Cholestasis: A Study in Children From a Liver Transplant Center in India.

J Clin Exp Hepatol 2022 Mar-Apr;12(2):454-460. Epub 2021 Jun 13.

Department of Pediatric Gastroenterology and Hepatology, Indraprastha Apollo Hospital, New Delhi, India.

Background/aims: This study aimed to delineate the clinical profile of children diagnosed with progressive familial intrahepatic cholestasis (PFIC).

Methods: This study was a retrospective analysis of case records of children in the tertiary care hospital, with the diagnosis of PFIC from January 2017 to January 2020. The diagnosis was made using clinical and laboratory parameters and with genetic testing when available. Read More

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Clinical Outcomes of Transcatheter Arterial Embolization Using N-butyl-2-cyanoacrylate (NBCA) in Cirrhotic Patients.

J Clin Exp Hepatol 2022 Mar-Apr;12(2):353-361. Epub 2021 Aug 26.

Department of Hepatology, Institute of Liver and Biliary Sciences, D-1 Vasant Kunj, New Delhi, 110070, India.

Purpose: To evaluate the clinical outcomes of transcatheter arterial embolization (TAE) with n-butyl-2-cyanoacrylate (NBCA) for treatment of bleeding in cirrhotic patients.

Materials And Methods: A total of 35 cirrhotic patients (26 men, 9 women; mean age, 48.4 ± 11. Read More

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Concentration of Serum Matrix Metalloproteinase-3 in Patients With Primary Biliary Cholangitis.

Front Immunol 2022 22;13:885229. Epub 2022 Apr 22.

Department of Gastroenterology, Hepatology and Clinical Oncology Centre of Postgraduate Medical Education, Warsaw, Poland.

Background And Aims: Metalloproteinases (MMPs) are involved in many distinct processes in the liver. Matrix metalloproteinase-3 (MMP-3) plays an important role in connective tissue remodeling, degradation of collagen (types II, III, IV, IX, and X), proteoglycans, fibronectin, laminin, and elastin. In addition, MMP-3 can also activate other MMPs such as MMP-1, MMP-7, and MMP-9. Read More

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TRPM8 deficiency attenuates liver fibrosis through S100A9-HNF4α signaling.

Cell Biosci 2022 May 7;12(1):58. Epub 2022 May 7.

Hunan Provincial Key Laboratory of Hepatobiliary Disease Research, Division of Hepato-Biliary-Pancreatic Surgery, Department of Surgery, The Second Xiangya Hospital, Central South University, 139 Renminzhong Road, Changsha, 410011, People's Republic of China.

Background: Liver fibrosis represent a major global health care burden. Data emerging from recent advances suggest TRPM8, a member of the transient receptor potential (TRP) family of ion channels, plays an essential role in various chronic inflammatory diseases. However, its role in liver fibrosis remains unknown. Read More

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Gadoxetic Acid-enhanced MRI-derived Functional Liver Imaging Score (FLIS) and Spleen Diameter Predict Outcomes in ACLD.

J Hepatol 2022 May 4. Epub 2022 May 4.

Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical, Medical University of Vienna, Vienna, Austria.

Background & Aims: Functional Liver Imaging Score (FLIS) derived from gadoxetic acid (GA)-enhanced MRI correlates with liver function in chronic liver disease (CLD) patients and independently predicts liver-related mortality. Splenic craniocaudal diameter (SCCD) is a marker of portal hypertension, a key driver of disease progression. The aim of this study was to investigate the accuracy of a combination of FLIS & SCCD for predicting hepatic decompensation, acute-on-chronic liver failure (ACLF), and mortality in advanced CLD (ACLD) patients. Read More

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Magnetic resonance imaging for the diagnosis of hepatocellular carcinoma in adults with chronic liver disease.

Cochrane Database Syst Rev 2022 05 6;5:CD014798. Epub 2022 May 6.

Department of Radiology , Clinical Hospital Centre Rijeka, Rijeka, Croatia.

Background: Hepatocellular carcinoma occurs mostly in people with chronic liver disease and ranks sixth in terms of global incidence of cancer, and third in terms of cancer deaths. In clinical practice, magnetic resonance imaging (MRI) is used as a second-line diagnostic imaging modality to confirm the presence of focal liver lesions suspected as hepatocellular carcinoma on prior diagnostic test such as abdominal ultrasound or alpha-fetoprotein, or both, either in surveillance programmes or in clinical settings. According to current guidelines, a single contrast-enhanced imaging study (computed tomography (CT) or MRI) showing typical hallmarks of hepatocellular carcinoma in people with cirrhosis is considered valid to diagnose hepatocellular carcinoma. Read More

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Single-Cell Characterization of Hepatic CD8 T Cells in a Murine Model of Primary Biliary Cholangitis.

Front Immunol 2022 20;13:860311. Epub 2022 Apr 20.

Medical Research Center, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China.

Primary biliary cholangitis (PBC), an organ-specific autoimmune disease, is characterized by injury to small bile ducts, inflammatory cell infiltrates within the liver, progressive cholestasis, and in some cases, cirrhosis with unclear pathogenesis. We aimed to clarify the importance role of hepatic immunce cells in the pathogenesis of human and experimental PBC.The dominant-negative TGFβ receptor type II transgenic (dnTGFβRII) mice, a well-studied and established murine model of PBC were used to identify changes of immune cells, especially the pathogenic CD8 T cells. Read More

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Proton pump inhibitor increasing the risk of infection and mortality in cirrhosis- need closer look!

Gastroenterology 2022 May 2. Epub 2022 May 2.

Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India. Electronic address:

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Risk Factors For Bleeding Adverse Events In Percutaneous Transhepatic Biliary Drainage: A Prospective Multicenter Study (BleeCom Study).

J Vasc Interv Radiol 2022 Apr 30. Epub 2022 Apr 30.

DAICIM Foundation, Buenos Aires, Argentina; University of Buenos Aires, Argentina; Percutaneous Surgery, IHU IRCAD, University of Strasbourg, France.

Purpose: To estimate percutaneous transhepatic biliary drainage (PTBD) risk factors (RF) for bleeding adverse events (BAE) and develop a risk assessment model.

Materials And Methods: Multicenter, prospective, observational, cross-sectional, and comparative study between 2015 and 2020. Adults with an indication for PTBD were included. Read More

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DNA methylation profile of liver tissue in end-stage cholestatic liver disease.

Epigenomics 2022 Apr 27;14(8):481-497. Epub 2022 Apr 27.

Division of Gastroenterology & Hepatology, Mayo Clinic, Rochester, MN 55905, USA.

In this methylome-wide association study of cholestatic liver diseases (primary sclerosing cholangitis and primary biliary cholangitis), the authors aimed to elucidate changes in methylome and pathway enrichment to identify candidate genes Reduced representation bisulfite sequencing was performed on liver tissue from 58 patients with primary sclerosing cholangitis (n = 13), primary biliary cholangitis (n = 20), alcoholic liver disease (n = 21) and live liver donors (n = 4). Pathway enrichment and network analysis were used to explore key genes/pathways. Both cholestatic liver diseases were characterized by global hypomethylation, with pathway enrichment demonstrating distinct genes and pathways associated with the methylome. Read More

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Methylation of the HCBP6 promoter is associated with primary biliary cholangitis pathogenesis.

Biochem Biophys Res Commun 2022 Jun 14;610:176-181. Epub 2022 Apr 14.

Department of General Medicine, Beijing Ditan Hospital, Capital Medical University, No.8, Jingshun East Street, Chaoyang District, Beijing, 100015, China. Electronic address:

Hepatitis C virus core-binding protein 6 (HCBP6), first characterized in our laboratory and also referred to as FUNDC2, is involved in lipid and glucose metabolism, platelet activation, and platelet survival. Here we demonstrate that hypermethylation of mitochondrial HCBP6 is linked Primary Biliary Cholangitis (PBC) pathogenesis. Serum and liver levels of HCBP6 in PBC patients were reduced in comparison with controls. Read More

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[Guidelines on the diagnosis and management of primary biliary cholangitis (2021)].


Zhonghua Gan Zang Bing Za Zhi 2022 Mar;30(3):264-275

In 2015, the Chinese Society of Hepatology and Chinese Society of Gastroenterology issued a consensus on the diagnosis and management of primary biliary cholangitis (PBC). In the past years, more clinical studies have been reported in the field of PBC. To provide guidance to the clinical diagnosis and management of patients with PBC, the Chinese Society of Hepatology invited a panel of experts to assess the new clinical evidence and formulated the current guidelines which comprises 26 clinical recommendations. Read More

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A randomized-controlled trial comparing 20% albumin to plasmalyte in patients with cirrhosis and sepsis-induced hypotension [ALPS trial].

J Hepatol 2022 Apr 20. Epub 2022 Apr 20.

Department of Hepatology. Electronic address:

Background And Aim: The choice of resuscitation fluid in cirrhosis patients with sepsis-induced hypotension (SIH) is unclear. 5% albumin has been superior to normal saline in the FRISC study. We compared the efficacy and safety of 20% albumin, which has greater oncotic properties with plasmalyte in reversing SIH. Read More

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Liquid chromatography coupled to tandem mass spectrometry methods for the selective and sensitive determination of 24S-hydroxycholesterol, its sulfate, and/or glucuronide conjugates in plasma.

J Mass Spectrom 2022 May;57(5):e4827

Laboratory of Molecular Pharmacology, Endocrinology and Nephrology Axis, CHU de Québec Research Centre and the Faculty of Pharmacy, Laval University, Québec City, Quebec, Canada.

24S-hydroxycholesterol (i.e., cerebrosterol, 24S-OH-Chol) is the main form of cholesterol elimination from the brain. Read More

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