CENTER-SPECIFIC DATA FROM THE INTERNATIONAL MULTICENTER PEDIATRIC PORTAL HYPERTENSION REGISTRY (IMPPHR) – INITIAL ANALYSES OF 23 INTERNATIONAL SITES

<div><p><strong><b>Background:</strong> </b>There are very limited high-quality data from which to derive therapeutic approaches to portal hypertension (PHT) in children. Management of varices, in particular, is quite controversial in pediatrics. IMPPHR was developed to derive large-scale international data, thereby enhancing our knowledge of PHT.

ELUCIDATING THE MECHANISMS OF KIF12-MEDIATED HIGH GGT CHOLESTASIS

<div><p><b>Background: </b>We and others have shown that rare bi-allelic loss-of-function<strong> </strong>mutations in Kinesin family member 12 (<em>KIF12</em>) cause pediatric high-GGT cholestasis as well as fibrosis, ductular reaction, and bile duct loss. KIF12 has putatively been defined as a microtubule-associated motor protein, but little is known about its function and even less about its role in the pathogenesis of cholestasis.</p>

CARVEDILOL PLUS NUCS TO PREVENT THE PROGRESSION OF ESOPHAGEAL VARICES IN VIROLOGICAL SUPPRESSED HBV-CIRRHOSIS PATIENTS: A RANDOMISED, OPEN-LABEL TRIAL.

<div><p><b>Background:</p> </b><p>Portal hypertension progression can be relieved after controlling the etiology of liver cirrhosis. Whether beta-blockers could additionally enhance the effects during treatment, particularly for mild esophageal varices (EVs), was unclear. This study aims to assess the efficacy of add-on carvedilol to delay EVs progression during anti-HBV treatment in HBV-related cirrhosis.</p>

CIRCULATING CLASSICAL MONOCYTE-DERIVED IL-1β PRODUCTION IS ASSOCIATED WITH THE REDUCTION OF SERUM HBsAg LEVELS IN CHRONIC HEPATITIS B

<div><p><strong>Background</strong>: Although nucleotide analogs (NA) prevent viral replication in chronic hepatitis B (CHB) patients, it rarely achieve HBsAg seroclearance. While the reduction of HBsAg levels is associated with HBsAg seroclearance, the factors regulating HBsAg levels are not well understood. To aim for “Functional Cure”, we explored the host immunodynamics involved in the reduction of HBsAg levels.

ACTIVATION OF TFEB PROTECTS AGAINST ACETAMINOPHEN-INDUCED LIVER INJURY IN MICE

<div><p><b>Background:<span> </b>Macroautophagy (referred to as autophagy hereafter) is a major intracellular lysosomal degradation pathway that is responsible for the degradation of misfolded/damaged proteins and organelles. Previous studies showed that autophagy protects against acetaminophen (APAP)-induced injury (AILI) via selective removal of damaged mitochondria and APAP protein adducts.

INTESTINAL EPITHELIAL CELL OSTEOPONTIN PROTECTS FROM MASH BY CHANGING THE COMPOSITION OF THE GUT MICROBIOME AND BILE ACIDS

<div><p><strong><b>Background:</strong> </b>osteopontin (OPN, encoded by <em>SPP1</em>) is involved in chronic liver disease. Previous studies reported that intestinal OPN regulates the gut microbiota, however the role of intestinal epithelial cell (IEC)-derived OPN in metabolic dysfunction-associated steatohepatitis (MASH) is unknown.</p>

HEPATOCYTE-SPECIFIC HIF-1α DELETION ACCELERATES NON-ALCOHOLIC STEATOHEPATITIS PROGRESSION TO LIVER FIBROSIS

<div><p><b>Background:</p> </b><p>Non-alcoholic steatohepatitis (NASH) is prevalent, yet lacks for effective treatments to hinder rapid fibrosis progression, which is the main factor that limits patient prognosis. The role of Hypoxia-inducible factor 1α (HIF-1α) in NASH progression remains unclear. This study investigates the impact and mechanism of hepatocyte-specific HIF-1α deletion on NASH fibrosis, aiming to uncover a novel therapeutic target.</p>

MOLECULAR SIGNATURE OF PERIPHERAL CIRCULATING TUMOR CELLS IN CANCER PROGRESSION OF HEPATOCELLULAR CARCINOMA PATIENTS TREATED BY ATEZOLIZUMAB PLUS BEVACIZUMAB

<div><p><b>Background:</p> </b><p>The mechanisms leading to cancer progression of hepatocellular carcinoma (HCC) such as acquired resistance to immunotherapies and metastatic progression are not clear. Circulating tumor cells (CTCs) have been defined as cancer cells released into the blood circulation from primary tumor. CTCs have notable advantages in that they are noninvasive and real-time biomarker that provide information about metastatic process.

ESTABLISHMENT OF PFIC 3 MOUSE MODEL CARRYING HUMAN-LIKE BILE ACID COMPOSITION BY IN VIVO LIVER-SPECIFIC GENE DELETION USING ADENO-ASSOCIATED VIRUS AND CRISPR/Cas9 SYSTEM

<div><p><strong><span><b>Background:</span> </b></strong><span>Progressive familial intrahepatic cholestasis (PFIC) 3 is a life-threatening hereditary disease caused by adenosine triphosphate-binding cassette subfamily B member 4 (ABCB4) gene mutations. The murine bile acid composition is different from that of humans. Mice are less likely to develop cholestasis due to the predominance of hydrophilic bile acids such as muricholic acids, which are synthesized by enzymes including Cyp2c70.

CHRONIC ETHANOL INSULT INCREASES HSD17beta13 ON HEPATOCELLULAR LIPID DROPLETS VIA A LOSS OF AN ASSOCIATED SEGREGASE p97/VCP TO PROMOTE HEPATIC STEATOSIS

<div><p><b>Background: </b>Chronic EtOH insult is known to lead to an accumulation of lipid droplets (LDs) in hepatocytes resulting in hepatic steatosis. Our previous study reported that EtOH resulted in a 10-fold reduction of the LD-associated segregase VCP/p97, a protein implicated in LD protein clearance. Concomitant with this decrease was a 6-fold increase in the association of the hydroxysteroid enzyme HSD17β13 known to play an important role in hepatic steatosis.

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