Abstract:
:Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease, characterized by multiple strictures and dilatations of the intra- and extrahepatic bile ducts, leading to progressive liver fibrosis, in 10-15% cholangiocarcinoma, and ultimately end-stage liver disease. The pathogenesis is poorly understood, but (epi-)genetic factors, mechanisms of innate and adaptive immunity, toxic effects of hydrophobic bile acids, and possibly intestinal dysbiosis appear to be involved. The strong link with inflammatory bowel disease (IBD) is associated with a markedly enhanced risk of colorectal cancer which next to cholangiocarcinoma represents the most serious diagnostic challenge in long-term PSC management. Despite extensive research, no medical treatment has been proven so far to prolong the time to liver transplantation (LTx), which remains the effective treatment in late-stage disease. Recurrence of PSC after LTx is observed in up to 20% of patients. Here, we briefly summarize actual views on PSC pathogenesis and provide an algorithmic approach to diagnostic procedures and recommendations for the management of PSC and its complications. We describe promising treatment options subject to current clinical trials.
journal_name
Hepatol Intjournal_title
Hepatology internationalauthors
Prokopič M,Beuers Udoi
10.1007/s12072-020-10118-xsubject
Has Abstractpub_date
2020-12-30 00:00:00eissn
1936-0533issn
1936-0541pii
10.1007/s12072-020-10118-xpub_type
杂志文章,评审abstract::Portal hypertension develops as a result of increased intrahepatic vascular resistance often caused by chronic liver disease that leads to structural distortion by fibrosis, microvascular thrombosis, dysfunction of liver sinusoidal endothelial cells (LSECs), and hepatic stellate cell (HSC) activation. While the basic ...
journal_title:Hepatology international
pub_type: 杂志文章,评审
doi:10.1007/s12072-017-9826-x
更新日期:2018-02-01 00:00:00
abstract:BACKGROUND AND AIMS:T cell-mediated immune injury plays a critical role in the pathogenesis of hepatitis B virus-related acute-on-chronic liver failure (HBV-ACLF). Given the high short-term mortality and crucial role of T cells in the disease progression, it is necessary to investigate the dynamics of T cell clones dur...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-019-10008-x
更新日期:2020-01-01 00:00:00
abstract::Chronic hepatitis B virus (HBV) infection is endemic in the Asian-Pacific region, and reactivation of HBV post-cancer chemotherapy has become an emerging clinical challenge. Patients with detectable serum HBV DNA before chemotherapy and those receiving intensive chemotherapy are particularly at a risk of HBV reactivat...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-011-9279-6
更新日期:2013-06-01 00:00:00
abstract:PURPOSE:Transcriptional silencing of tumor suppressor genes associated with DNA hypermethylation has been known as a hallmark of human cancer. In this study, we revealed that a local anesthetic, procaine (PCA), possessed growth-inhibitory and demethylating effect on human hepatoma cells in vitro and in vivo. METHODS:T...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-007-9014-5
更新日期:2007-09-01 00:00:00
abstract:OBJECTIVES:The aim of this study was to investigate the association of the Laennec staging system with degree of cirrhosis, clinical stage and liver function. METHODS:Liver biopsy was performed for 30 patients with hepatitis B cirrhosis to test the content of hydroxyproline in hepatic tissue, judge the degree of cirrh...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-015-9648-7
更新日期:2015-10-01 00:00:00
abstract:BACKGROUND:L-Asparaginase is a bacterial enzyme used in the treatment of acute lymphoblastic leukemia. In the ongoing U.S. Drug-Induced Liver Injury Network (DILIN) prospective study, standard and pegylated asparaginase were the most frequent cause of liver injury with jaundice among anti-cancer agents (8 of 40: 20%). ...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-019-09971-2
更新日期:2019-09-01 00:00:00
abstract::The prognosis of advanced hepatocellular carcinoma (HCC) remains poor. For patients with advanced HCC, the multikinase inhibitor sorafenib is recommended as the current standard of care. In contrast, hepatic arterial infusion chemotherapy (HAIC) is one of the recommended treatments in Japan. However, in Japan, the use...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-013-9515-3
更新日期:2014-09-01 00:00:00
abstract:BACKGROUND AND AIMS:Acute-on-chronic liver failure (ACLF) is associated with a high mortality rate in the absence of liver transplantation. There is limited data on predictors of survival in ACLF in children. Therefore, we prospectively studied the predictors of outcome of ACLF in children. METHODS:A prospective evalu...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-010-9217-z
更新日期:2011-06-01 00:00:00
abstract:AIMS:Relationships of renal function and liver disease are described in acute and chronic liver failure. The aim of the study is to investigate which relationship, if any, is present between severity of non-alcoholic fatty liver disease (NAFLD), assessed by bright liver score (BLS) versus mild-moderate renal insufficie...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-011-9254-2
更新日期:2011-09-01 00:00:00
abstract:BACKGROUND:The aim of our study was to explore how C1QTNF1-AS1 regulated miR-221-3p/SOCS3 axis in human hepatocellular carcinoma (HCC). METHODS:Differentially expressed lncRNAs and genes were examined via RNA-seq. GO analysis and KEGG pathway enrichment analysis were carried out based on the function of dys-regulated ...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-019-09944-5
更新日期:2019-05-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:Cytokines have been reported to be involved in the cirrhosis and hepatic encephalopathy (HE). Many aspects on the correlation between minimal HE (MHE) and cytokine levels were still unclear. METHODS:Two hundred eighty-nine HBV-infected cirrhotic patients were grouped: non MHE (n = 156), MHE (n...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-015-9610-8
更新日期:2015-04-01 00:00:00
abstract:BACKGROUND AND AIMS:Recently, Sofosbuvir was launched in India at affordable cost. We conducted a real-life study to determine the efficacy and safety of Sofosbuvir plus Ribavirin, with and without peginterferon-alfa 2a, in patients with chronic hepatitis C (CHC) genotype 3, the commonest genotype in South Asia. METHO...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-017-9794-1
更新日期:2017-05-01 00:00:00
abstract:PURPOSE:The diagnostic potential of hepatic stellate cells (HSCs) and γδT cells for patients with hepatocellular carcinoma (HCC) and their synergistic contributions to the prognosis of these patients have not yet been investigated. The aim of this study was to elucidate the prognostic value of these cells in HCC. METH...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-019-09969-w
更新日期:2019-09-01 00:00:00
abstract:BACKGROUND/PURPOSE:In Japan, there is a growing population of patients with chronic hepatitis C virus (HCV) infection who failed a direct-acting antiviral (DAA)-based regimen. In this Phase 3 study, we evaluated sofosbuvir-velpatasvir plus ribavirin in Japanese patients with genotype 1 or 2 HCV infection who previously...
journal_title:Hepatology international
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s12072-018-9878-6
更新日期:2018-07-01 00:00:00
abstract:BACKGROUND:Variceal bleeding is a major complication of portal hypertension, which is associated with significant mortality. Moreover, patients surviving a variceal bleeding episode have very high risk of rebleeding, which is associated with mortality as high as that of the first bleed. Because of this, prevention of b...
journal_title:Hepatology international
pub_type: 杂志文章,评审
doi:10.1007/s12072-017-9833-y
更新日期:2018-02-01 00:00:00
abstract::Actinomycotic hepatic abscess was diagnosed in a 46-year-old male driver from Ukraine presenting with the symptoms of malaise, loss of appetite, upper right quadrant pain, weight loss, and night sweats which had been present for last 2 months. Computed tomography (CT) of the abdomen revealed a hypodense mass in the le...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-007-9032-3
更新日期:2008-03-01 00:00:00
abstract::Non-alcoholic fatty liver disease (NAFLD) is currently one of the leading forms of chronic liver disease, and its rising frequency worldwide has reached epidemic proportions. NAFLD, particularly its progressive variant NASH (non-alcoholic steatohepatitis), can lead to advanced fibrosis, cirrhosis, and HCC. The pathoph...
journal_title:Hepatology international
pub_type: 杂志文章,评审
doi:10.1007/s12072-018-9854-1
更新日期:2018-03-01 00:00:00
abstract:PURPOSE:To analyze the prognostic factors for the patients with lung metastases from hepatocellular carcinoma (HCC). METHODS AND MATERIALS:One hundred and five patients with lung metastases from HCC were analyzed retrospectively. We analyzed the impact factors, including the gender, age, liver function, serum AFP and ...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-008-9052-7
更新日期:2008-06-01 00:00:00
abstract:BACKGROUND/AIMS:[corrected] The risk of hepatocellular carcinoma (HCC) increased with progression of hepatic fibrosis as assessed by liver stiffness measurement (LSM). This study used LSM to assess the risk of HCC presence in patients with chronic hepatitis. METHODS:The patients with liver tumor or chronic hepatitis i...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-010-9223-1
更新日期:2010-10-08 00:00:00
abstract:BACKGROUND:There is much controversy surrounding the natural history of hepatitis C virus (HCV) infection. AIMS:The aim of this review was to review the natural history of HCV infection. METHODS:Published English literature was searched via pubmed and then reviewed. RESULTS:Approximately, 75-85% of HCV-infected pers...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-012-9355-6
更新日期:2012-10-01 00:00:00
abstract:PURPOSE:Microvascular invasion of hepatocellular carcinoma (HCC) is considered a poor prognostic factor of liver resection (LR) and liver transplantation (LT), but its significance for lesions within the up-to-7 criteria is unclear. This study investigated the survival benefit of primary LT against LR for HCC with micr...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-011-9318-3
更新日期:2011-10-21 00:00:00
abstract::The bone marrow contains stem cells that have the potential to differentiate into a variety of organ-specific mature cells, including the liver and the pancreas. Recently, the origin of hepatic progenitors and hepatocytes was identified to be the bone marrow. However, evidence that describes which cells, among all bon...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-007-9031-4
更新日期:2008-03-01 00:00:00
abstract:PURPOSE:To elucidate the efficiency of contrast-enhanced ultrasonography alone and in combination with other noninvasive models for grading hepatic fibrosis. METHODS:This prospective study included 74 patients with four grades (F1-F4) of chronic liver disease (17, 20, 18, and 19 patients, respectively). Diagnostic per...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-012-9370-7
更新日期:2013-06-01 00:00:00
abstract:BACKGROUND AND AIMS:The progression of HBsAg-positive chronic hepatitis is insidious and unpredictable. Identification of factors leading to either a benign or more serious clinical outcome may assist in decision making for antiviral therapy. METHODS:From 1989 to 1998, 130 untreated patients with chronic hepatitis wer...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-015-9651-z
更新日期:2015-10-01 00:00:00
abstract::Hepatitis B virus (HBV) reactivation with hepatic decompensation leading to acute on chronic liver failure is not uncommon. It is associated with high mortality of up to 30-70%. Prognostic factors for mortality include high bilirubin level, more prolonged prothrombin time, low platelet count and presence of pre-existi...
journal_title:Hepatology international
pub_type: 杂志文章,评审
doi:10.1007/s12072-014-9569-x
更新日期:2015-07-01 00:00:00
abstract:PURPOSE:To assess the accuracy of a formula derived from 159 living liver donors to estimate the liver size of a normal subject: standard liver weight (g) = 218 + body weight (kg) × 12.3 + 51 (if male). Standard liver volume (SLV) is attained by a conversion factor of 1.19 mL/g. METHODS:The total liver volume (TLV) of...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-011-9264-0
更新日期:2011-03-26 00:00:00
abstract::In a country such as Japan with the average age of patients with chronic hepatitis C treated with antivirals sometimes well above 60 years, the standard combination therapy is not well tolerated. In this randomized, prospective, controlled trial, we investigated the efficacy of 24-week peginterferon α monotherapy for ...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-009-9134-1
更新日期:2009-09-01 00:00:00
abstract::Hepatitis C virus (HCV) treatment in HIV/HCV co-infected individuals has renewed relevance given the ongoing opioid crisis and rise of new HIV and HCV infections associated with injection drug use. Patients co-infected with HIV and HCV demonstrate increased rates of hepatic fibrosis, progression to liver failure, and ...
journal_title:Hepatology international
pub_type: 杂志文章,评审
doi:10.1007/s12072-018-9896-4
更新日期:2018-11-01 00:00:00
abstract:BACKGROUND/PURPOSE:Gut microbiota has been associated with liver cirrhosis and, possibly, hepatic encephalopathy. However, only a few studies have examined the link between mucosa-associated microbiota (MAM) and minimal hepatic encephalopathy (MHE). Our aim was to investigate this relationship. METHODS:Twenty-four pat...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-019-09963-2
更新日期:2019-07-01 00:00:00
abstract:PURPOSE:We investigated whether the combined presence and evolution of hepatitis B virus (HBV) mutant strains in the hepatitis B e antigen (HBeAg)-positive status can predict clinical outcomes after HBeAg seroconversion. METHODS:One hundred and eighty-six patients with spontaneous HBeAg seroconversion were enrolled in...
journal_title:Hepatology international
pub_type: 杂志文章
doi:10.1007/s12072-012-9381-4
更新日期:2013-06-01 00:00:00