World Journal of Hepatology最新文献

Background: Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive liver disease, causing episodic cholestasis with intense pruritus. This case report highlights the effectiveness of early plasmapheresis as a therapeutic option for BRIC type 2, offering rapid symptom relief and early termination of cholestatic episodes. It contributes to the limited evidence supporting plasmapheresis as a treatment for BRIC flares resistant to conventional therapies.

Case summary: A 43-year-old male with BRIC type 2 presented with fatigue, jaundice, and severe pruritus, triggered by a recent mild severe acute respiratory syndrome coronavirus 2 infection. Laboratory results confirmed cholestasis with elevated bilirubin and alkaline phosphatase. First-line pharmacological treatments, including cholestyramine and rifampicin, failed. Endoscopic nasobiliary drainage was ineffective, prompting initiation of plasmapheresis. This intervention rapidly relieved pruritus, with complete biochemical normalisation after 11 sessions. Two years later, a similar episode occurred, and early reinitiation of plasmapheresis led to symptom resolution within two sessions and biochemical recovery within two weeks. The patient tolerated the procedure well, with no adverse effects observed. Follow-up showed no signs of cholestasis recurrence.

Conclusion: Plasmapheresis is a safe and effective option for therapy-refractory BRIC type 2, particularly when initiated early in cholestasis.

Background: Hepatitis B virus (HBV) evades the innate immunity and leads to persistent chronic infection, but the molecular mechanism is still not well known.

Aim: To investigate whether HBV-miR-3 is involved in HBV immune evasion.

Methods: HBV-miR-3 agomir and antagomir were employed to verify the effectiveness of HBV-miR-3 on cGAS-Sting-IFN pathway through the experiments on relative luciferase activity, cGAS protein expression, Sting phosphorylation and interferon (IFN) production.

Results: HBV-miR-3 down-regulates cGAS protein expression post-transcriptionally by inhibition of cGAS 3'-untranslated region (3'-UTR) activity, which results in lower Sting phosphorylation and IFN production. HBV-miR-3 antagomir rescued cGAS protein expression, Sting phosphorylation and IFN-β production.

Conclusion: HBV-miR-3 plays an important role in HBV immunity evasion by targeting cGAS 3'-UTR and interfering with cGAS-Sting-IFN pathway.

Background: Heterozygous familial hypobetalipoproteinemia (FHBL) is a semi-autosomal disorder that is caused mainly by an APOB variant. It is usually asymptomatic and rarely leads to non-alcoholic steatohepatitis (NASH).

Case summary: A 12-year-old boy was referred to our hospital after prolonged elevation of liver enzymes was observed during health checkups in Kagawa Prefecture. Abdominal ultrasound showed a bright liver, and laboratory investigations revealed low low-density lipoprotein cholesterol and apolipoprotein B protein levels. His family history included fatty liver and hypolipidemia in his father, which led to a clinical diagnosis of FHBL. A liver biopsy was performed on suspicion of liver fibrosis based on biomarkers. The liver tissue showed fatty steatosis, inflammation, hepatocyte ballooning, and fibrosis, indicating NASH. Genetic testing detected the APOB variant, and the patient was treated successfully with vitamin E.

Conclusion: It is important to assess family history and liver dysfunction severity in non-obese patients with hypolipidemia and fatty liver.

Background: This review evaluated the diagnostic effectiveness of various ultrasound (US) methods compared to liver biopsy.

Aim: To determine the diagnostic accuracy of US techniques in assessing liver fibrosis and steatosis in adults, using the area under the receiver operating characteristic curve (AUROC) as the standard measure.

Methods: The review included original retrospective or prospective studies published in the last three years in peer-reviewed medical journals, that reported AUROC values. Studies were identified through PubMed searches on January 3 and April 30, 2024. Quality was assessed using the QUADAS-2 tool. Results were tabulated according to the diagnostic method and the type of liver pathology.

Results: The review included 52 studies. For liver fibrosis detection, 2D-shear wave elastography (SWE) AUROCs ranged from 0.54 to 0.994, showing better accuracy for advanced stages. Modifications, including 2D-SWE with propagation map guidance and supersonic imagine achieved AUROCs of 0.84 to nearly 1.0. point SWE and classical SWE had AUROCs of 0.741-0.99, and 0.507-0.995, respectively. Transient elastography (TE), visual TE, vibration-controlled TE (VCTE), and FibroTouch reported AUROCs close to 1.0. For steatosis, VCTE with controlled attenuation parameter showed AUROCs up to 0.89 (for ≥ S1), acoustic radiation force impulse ranged from 0.762 to 0.784, US attenuation parameter from 0.88 to 0.93, and normalized local variance measurement from 0.583 to 0.875. Most studies had a low risk of bias across all or most domains, but evidence was limited by variability in study quality and small sample sizes. Innovative SWE variants were evaluated in a single study.

Conclusion: Modern US techniques can serve as effective noninvasive diagnostic tools for liver fibrosis and steatosis, with the potential to reduce the reliance on biopsies.

This letter addresses the study by Jayabalan et al, which underscores the liver outcome score (LOS) and hemoglobin (Hb) as key prognostic markers for patients with autoimmune liver disease overlap syndromes (AILDOS), with particular relevance to the autoimmune hepatitis-primary biliary cholangitis (AIH-PBC) subgroup. The findings indicate that an LOS threshold of 6 achieves high sensitivity and specificity in predicting liver-related mortality among AIH-PBC patients. Moreover, low Hb levels emerge as a significant mortality predictor across all AILDOS cases. These results contribute valuable perspectives on risk stratification in AILDOS, highlighting the promise of non-invasive prognostic tools. Future studies with larger cohorts are needed to substantiate LOS and Hb as robust markers for clinical application.

Gilbert's syndrome (GS) is a common hereditary condition characterized by mild increases in serum bilirubin levels due to inherited defects in bilirubin metabolism. This review, based on peer-reviewed articles spanning from 1977 to January 2024 and sourced through the PubMed platform, provides an overview of current knowledge regarding GS. Early studies primarily focused on defining the clinical and genetic characteristics of the syndrome. More recent research has delved into the genetic mechanisms underlying the reduced expression of bilirubin UDP-glucuronosyltransferase, significantly enhancing our understanding of the pathogenesis of GS. Recent studies have also investigated clinical implications of GS, including its association with metabolic associated steatotic liver disease, cardiovascular disease, mental health and mortality risk, highlighting the complex interplay between genetic factors, bilirubin metabolism, and clinical outcomes.

Background: Hepatic alveolar echinococcosis (HAE) is a chronic parasitic disease caused by the larval stage of Echinococcus multilocularis. Although significant research has been conducted on the pathogenesis and immunological aspects of HAE, comprehensive bibliometric analyses in this area are still lacking. This study sought to fill this gap by systematically analyzing the immunological literature on HAE using bibliometric methods.

Aim: To identify research trends, key contributors, and emerging developments and offer insights to guide future research in this field.

Methods: Research articles on HAE published between 1983 and 2023 were retrieved from the Web of Science Core Collection database. A total of 319 articles were selected for bibliometric analysis, which was conducted using the CiteSpace and VOSviewer software. The analysis focused on key variables such as publication volume, authors, journals, countries, references, and keywords.

Results: The analysis revealed a significant increase in research on HAE over the past four decades, particularly after 1995. China and Switzerland emerged as the leading countries in terms of publication volume, with Bruno Gottstein and Vuitton DA identified as the most influential authors in this field. Key research areas include the interaction between the pathogen and the host immune system, as well as advances in disease diagnosis and treatment strategies. The keyword co-occurrence analysis highlighted the primary themes and identified emerging trends within the research landscape.

Conclusion: This study provides a comprehensive framework for understanding HAE immunology and highlights research hotspots, future directions, key contributors, and the importance of international cooperation.

Background: Transjugular intrahepatic portosystemic shunt (TIPS) has an important role in the therapy of complications of portal-hypertension-related ascites. Various guidelines now indicate that TIPS is indicated for refractory ascites (RA), but TIPS for recurrent nonrefractory ascites (RNRA) achieved better clinical results.

Aim: To compare the clinical outcomes of TIPS for RA and RNRA in patients with complications related to portal hypertension.

Methods: There were 863 patients divided into two main categories who underwent TIPS between September 2016 and September 2021. In category 1, patients had ascites without cirrhotic gastrointestinal bleeding. The patients were divided into group A (RNRA, n = 183) and group B (RA, n = 217). In category 2, patients had ascites and cirrhotic gastrointestinal bleeding. The patients were divided into group C (RNRA, n = 328) and group D (RA, n = 135). The clinical outcomes were probability of total hepatic impairment, incidence of hepatic encephalopathy (HE) and mortality.

Results: The symptoms of ascites disappeared or were relieved within 1 week in group A compared with group B (P = 0.032), and in group C compared with group D (P = 0.027). By the end of follow-up, there were significant differences in the rate of RA in group A compared with group B (P = 0.016), and in group C compared with group D (P = 0.012). The probability of total hepatic impairment was significantly different in group A compared with group B (P = 0.024), and in group C compared with group D (P = 0.019). The total incidence of HE was significantly different in group A compared with group B (P = 0.008), and in group C compared with group D (P = 0.004). The 6-month, and 1-, 2- and 3-year survival rates were significantly different between groups A and B (all P < 0.05), and between groups C and D (all P < 0.05).

Conclusion: TIPS has a good therapeutic effect on ascites related to cirrhotic portal hypertension, and early TIPS for RNRA can prolong survival, and prevent progression to RA.

Background: Non-alcoholic fatty liver disease (NAFLD) is a disease of increasing global prevalence and an important risk factor for the development of insulin resistance, type 2 diabetes, non-alcoholic steatohepatitis and hepatocellular carcinoma, but the pathogenesis is not clear. The aim of this study was to explore the role of ILF3 in NAFLD.

Aim: To investigate the molecular processes through which ILF3 facilitates the advancement of NAFLD by inhibiting the expression of p-AMPK. This exploration seeks to provide new insights into the etiology of NAFLD and evaluate the potential of ILF3 as a diagnostic marker and potential treatment focus for future interventions.

Methods: In vitro and in vivo experiments were conducted using HepG2 cells and NAFLD animal models. The effects of ILF3 knockdown on lipid synthesis and triglyceride (TG) secretion were examined by analyzing the expression levels of p-AMPK. Additionally, the roles of ILF3 and the AMPK signaling pathway were verified using techniques such as Western blotting, quantitative reverse transcription PCR, Oil Red O staining, and immunohistochemistry.

Results: Investigations revealed an increase in ILF3 Levels within both HepG2 cells and animal models of NAFLD, concurrently with a decrease in p-AMPK expression. Knocking down ILF3 activated the AMPK pathway, reducing lipid production and TG secretion in hepatocytes, thereby mitigating the advancement of NAFLD.

Conclusion: ILF3 promotes the evolution of NAFLD by inhibiting the expression of p-AMPK. The knockdown of ILF3 activates the AMPK signaling pathway, alleviating the severity of NAFLD. These findings underscore the function of ILF3 in the pathogenesis of NAFLD and demonstrate its viability as a treatment focus and diagnostic indicator.