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We measured intestinal fatty acid-binding protein (I-FABP) levels, a useful marker of small intestinal mucosal injury, in patients with cirrhosis to determine their relationship with liver function and complications. This cross-sectional study included 71 patients with cirrhosis admitted for treatment of cirrhotic complications or hepatocellular carcinoma (cohort A) and 104 patients with cirrhosis who received direct-acting antiviral therapy for HCV (cohort B). I-FABP levels, measured by ELISA, were evaluated relative to hepatic reserve and compared with non-invasive scoring systems for diagnostic performance in cirrhotic complications. The median I-FABP level in both cohorts were significantly elevated in patients with reduced hepatic reserve (CTP grade A/BC cohort A, 2.33/3.17 ng/mL, p = 0.032; cohort B, 2.46/3.64 ng/mL, p = 0.008) and complications with gastroesophageal varices (GEV; GEV (-)/(+) cohort A, 1.66/3.67 ng/mL, p < 0.001; cohort B, 2.32/3.36 ng/mL; p = 0.003). Further, multiple logistic regression analysis identified I-FABP as the only factor contributing to GEV presence in both cohorts, which outperformed non-invasive scoring systems for GEV diagnosis (sensitivity 84.6%; specificity 84.2%; sensitivity 69.6%; specificity 63.8%, respectively). In conclusion, elevated small-intestinal mucosal injury in patients with cirrhosis was related to reduced hepatic reserve and GEV presence. I-FABP levels reflect portal hypertension and may be useful in cirrhosis management., (© 2024. The Author(s).)

Background: In patients with biliary atresia (BA), severe portal hypertension (HTN) develops even with successful bile flow restoration, suggesting an intrinsic factor driving portal HTN independent from bile obstruction. We hypothesize that patients with BA have abnormal portal vein (PV) development, leading to PV hypoplasia., Methods: In this observational cohort study, we enrolled patients who were referred to a tertiary center from 2017 to 2021 to rule out BA. Newborns who underwent computed tomography angiogram as a clinical routine before intraoperative cholangiogram, and laparoscopic Kasai hepatoportoenterostomy. The diameter of the PV and hepatic artery (HA) were compared to the degree of liver fibrosis in the wedge biopsies. The jaundice clearance, native liver survival, and clinical portal hypertensive events, including ascites development and intestinal bleeding, were assessed., Results: 47 newborns with cholestasis were included in the cohort; 35 were diagnosed with BA. The patients with BA had a smaller median PV diameter (4.3 vs. 5.1 mm; p < 0.001) and larger median HA diameter (1.4 vs. 1.2 mm; p < 0.05) compared to the patients with other forms of cholestasis. The median PV and HA diameter did not correlate with the degree of liver fibrosis. Among 35 patients with BA, 29 patients (82.9%) achieved jaundice clearance, and 23 patients (65.7%) were alive with their native liver at two years of age. Seven patients (20%) developed intestinal bleeding, and seven patients (20%) developed ascites, with one overlapping patient., Conclusion: PV hypoplasia is present in patients with BA independent of liver fibrosis at the time of diagnosis., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background and Aims: The placement of Transjugular intrahepatic portosystemic shunt (TIPS) results in a sudden increase in central circulating blood volume, which requires proper regulation of the cardiovascular system. We aimed to investigate the impact of TIPS on cirrhotic cardiomyopathy (CCM)., Method: A consecutive case series of patients with cirrhosis who underwent TIPS were evaluated by echocardiography and pressure measurements before, immediately after TIPS and 2-4 days later (delayed). Furthermore, all patients underwent a one-year follow-up., Results: In this study, 107 patients were enrolled, 38 (35.5%) with CCM. Echocardiography revealed an increase in postoperative left ventricular filling pressure accompanied by an elevation in left ventricular ejection fraction (LVEF). However, patients in the CCM group exhibited lower LVEF and mean arterial pressure (MAP) compared to the non-CCM group. Post-TIPS, CCM patients showed increased right atrium pressure (RAP) that normalized within 2-4 days, whereas non-CCM patients had lower RAP than baseline. Compared to patient without CCM, CCM patients revealed lower immediate (16.7 ± 4.4 vs. 18.9 ± 4.8, p = 0.022) and delayed 15.9 ± 3.7 vs. 17.7 ± 5.3, p = 0.044) portal vein pressures (PVP) and portal pressure gradients (PPG) (7.7 ± 3.4 vs. 9.2 ± 3.6, p = 0.032 and 10.1 ± 3.1 vs. 12.3 ± 4.9, p = 0.013). The 1-year mortality rates were 13.2% for CCM patients and 4.3% for non-CCM patients (log-rank test, p = 0.093), with MELD score, and preoperative RAP significantly associated with the mortality., Conclusion: Cirrhotic patients with CCM exhibit lower PVP and PPG immediately after TIPS and 2-4 days later, without significantly impacting one-year survival outcomes., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Portal hypertensive gastropathy (PHG) is a serious complication and the most common gastric mucosal injury amongst patients afflicted with cirrhotic or non-cirrhotic portal hypertension (PHT). The pathogenesis of PHG is not completely understood and is likely to be complex. The roles of portal hypertension pressure, parenchymal liver disease, Child-Pugh classification, variceal pressure and Helicobacter pylori infection in the development of PHG are controversial. Splanchnic blood flow, the distribution of mucosal blood, vascular ectasia, local disturbances, inflammatory cell infiltration and increased cytokine production have also been examined to elucidate the underlying mechanisms of PHG. Moreover, various other elements, including prostaglandin E 2 (PGE 2 ), endothelin-1 (ET-1), tumour necrosis factor-α (TNF-α), Fas ligand (FasL)/Fas, nitric oxide (NO), oxygen free radicals and vascular endothelial growth factor (VEGF), have also been revealed to participate in the pathogenesis of PHG. This review provides an overview of the risk factors, classification and potential molecular processes involved in PHG, followed by a concise summary of our and other studies. This review aims to integrate information to deepen our understanding of the interplay between different signalling pathways involved the pathogenesis of PHG and provides insights into how these signalling pathways are regulated to control the development of PHG., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Background/aims: The histological characteristics and natural history of precirrhotic primary biliary cholangitis (PBC) with portal hypertension (PH) are unclear. Our aim was to clarify the prevalence, risk factors, and histological characteristics of precirrhotic PBC patients with PH., Methods: This retrospective study compared the clinical features, histological characteristics, and response to ursodeoxycholic acid (UDCA) between the PH and non-PH groups of precirrhotic PBC patients., Results: Out of 165 precirrhotic PBC patients, 40 (24.2%) also had PH. According to histological stage 1, 2 and 3 disease, 5.3% (1/19), 17.3% (17/98), and 45.8% (22/48) of patients also had PH, respectively. Precirrhotic PBC with PH was significantly positively correlated with bile duct loss, degree of cytokeratin 7 positivity, and degree of fibrosis in the portal area, but significantly negatively correlated with lymphoid follicular aggregation. Compared to the non-PH group, patients in the PH group showed a higher prevalence of obliterative portal venopathy, incomplete septal fibrosis, portal tract abnormalities and non-zonal sinusoidal dilatation (p<0.05). In addition, patients with PH were more likely to present with symptoms of jaundice, ascites, epigastric discomfort, a poorer response to UDCA, and more decompensation events (p<0.05). High alkaline phosphatase levels, low white blood cell counts, high Mayo scores, and high FIB-4 index values were risk factors for precirrhotic PBC with PH., Conclusions: Approximately 24.2% of precirrhotic PBC patients have PH, which is histologically related to the injury of bile ducts. High alkaline phosphatase levels, low white blood cell counts, high Mayo scores, and high FIB-4 index values are associated with increased risk of precirrhotic PBC with PH.

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., Methods: The research was carried out through narrative review. The databases used for the search include Medline, Embase, Elsevier, Lilacs and Scielo. The keywords used were "hepatopulmonary syndrome" AND child, children, infant, preschool, pediatric., Results: In cirrhotic children, the prevalence of HPS can reach up to 42.5%, and it is even more common in those whose underlying condition is biliary atresia, reaching up to 63%. Screening with pulse oximetry (O2 saturation <96%), unlike in adults, has low sensitivity in the pediatric age group. Management involves supportive care with oxygen therapy; liver transplantation is the only definitive treatment to reverse the condition and HPS is considered an exceptional criterion for waitlist. The waitlist mortality is similar among children listed by HPS as a special criterion when compared to those listed for other reasons. The reported rates of complete resolution of hypo-xemia after liver transplantation are close to 100% in children. The post-liver transplantation survival is similar or slightly lower in children with HPS when compared to those without HPS. Contrary to findings from adults, no differences were found in post- liver transplantation mortality between children of different hypoxemia ranges, although longer mechanical ventilation time and hospital stay were observed in children with PaO2 <50 mmHg., Conclusion: HPS is not an uncommon complication of cirrhosis in children and adolescents, particularly when biliary atresia is the underlying condition. There are still many gaps to be filled regarding the condition, and this article demonstrates that not all data obtained in studies with adults reflects the disease's behavior in pediatrics, especially concerning prognosis.

Background and Aims: Transjugular intrahepatic portosystemic shunt (TIPS) is an established procedure for the treatment of several complications of portal hypertension (PH), including non-neoplastic portal vein thrombosis (PVT). Selection criteria for TIPS in PVT are not yet well established. Despite anecdotal, cases of thromboembolic events from paradoxical embolism due to the presence of patent foramen ovale (PFO) after TIPS placement have been reported in the literature. Therefore, we aimed at describing our experience in patients with non-neoplastic splanchnic vein thrombosis (SVT) who underwent TIPS following PFO screening., Methods: We conducted a single-centre retrospective study, including consecutive patients who underwent TIPS for the complications of cirrhotic and non-cirrhotic portal hypertension (NCPH) and having SVT., Results: Of 100 TIPS placed in patients with SVT, 85 patients were screened for PFO by bubble-contrast transthoracic echocardiography (TTE) with PFO being detected in 22 (26%) cases. PFO was more frequently detected in patients with non-cirrhotic portal hypertension (NCPH) (23% in the PFO group vs. 6% in those without PFO, p = .04) and cavernomatosis (46% in the PFO group vs. 19% in those without PFO, p = .008). Percutaneous closure was effectively performed in 11 (50%) after multidisciplinary evaluation of anatomical and clinical features. No major complications were observed following closure., Conclusions: PFO screening and treatment may be considered feasible for patients with SVT who undergo TIPS placement., (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background and Aims: Impact of type 2 diabetes mellitus (T2DM) in patients with end-stage liver disease (ESLD) awaiting liver transplantation (LT) remains poorly defined. The objective of the present study is to evaluate the relationship between T2DM and clinical outcomes among patients with LT waitlist registrants. We hypothesize that the presence of T2DM will be associated with worse clinical outcomes., Methods: 593 patients adult (age 18 years or older) who were registered for LT between 1/2010 and 1/2017 were included in this retrospective analysis. The impact of T2DM on liver-associated clinical events (LACE), survival, hospitalizations, need for renal replacement therapy, and likelihood of receiving LT were evaluated over a 12-month period. LACE was defined as variceal hemorrhage, hepatic encephalopathy, and ascites. Kaplan-Meier and Cox regression analysis were used to determine the association between T2DM and clinical outcomes., Results: The baseline prevalence of T2DM was 32% (n = 191) and patients with T2DM were more likely to have esophageal varices (61% vs. 47%, p = 0.002) and history of variceal hemorrhage (23% vs. 16%, p = 0.03). The presence of T2DM was associated with increased risk of incident ascites (HR 1.91, 95% CI 1.11, 3.28, p = 0.019). Patients with T2DM were more likely to require hospitalizations (56% vs. 49%, p = 0.06), hospitalized with portal hypertension-related complications (22% vs. 14%; p = 0.026), and require renal replacement therapy during their hospitalization. Patients with T2DM were less likely to receive a LT (37% vs. 45%; p = 0.03). Regarding MELD labs, patients with T2DM had significantly lower bilirubin at each follow-up; however, no differences in INR and creatinine were noted., Conclusion: Patients with T2DM are at increased risk of clinical outcomes. This risk is not captured in MELD score, which may potentially negatively affect their likelihood of receiving LT., (© 2024. The Author(s).)

Introduction and Objectives: Laparoscopic splenectomy and esophagogastric devascularization (LSED) are minimally invasive, effective, and safe in treating esophageal-fundic variceal bleeding with portal hypertension (PHT). The study aimed to assess the learning curve of LSED by cumulative summation (CUSUM) analysis. The 10-year follow-up data for LSED and open surgery were also examined., Patients and Methods: Five hundred and ninety-four patients were retrospectively analyzed. Operation time, intraoperative blood loss, open operation conversion, and postoperative complications were selected as the evaluation indicators of surgical ability. The learning curve of LESD was assessed by the CUSUM approach. Patient features, perioperative indices, and 10-year follow-up data were examined., Results: Totally 236 patients underwent open surgery, and 358 underwent LSED. Patient characteristics were similar between groups. The LSED patients experienced less intraoperative blood loss, fewer complications, and faster recovery compared to the open surgery cohort. The learning curve of LESD was maximal for a case number of 50. Preoperative general characteristics were comparable for both stages. But the skilled stage had decreased operation time, reduced blood loss, less postoperative complications, and better recovery compared to the learning stage. The LSED group had higher recurrent hemorrhage-free survival rate and increased overall survival in comparison with cases administered open surgery in the 10-year follow-up. Free-liver cancer rates were similar between two groups., Conclusions: About 50 cases are needed to master the LSED procedure. Compared to open surgery, LSED is a safer, feasible, and safe procedure for PHT patients, correlating with decreased rebleeding rate and better overall survival., (© 2024. The Author(s).)

Background: Extrahepatic, abdominal surgery in patients with cirrhosis is associated with high morbidity and mortality. This systematic review presents the current evidence available on the utility of a preoperative transjugular intrahepatic portosystemic shunt (TIPS), assessed by its effect on surgical candidacy and postoperative mortality and morbidity in patients with cirrhosis undergoing extrahepatic, abdominal surgery., Methods: MEDLINE, EMBASE, Cochrane Library and Web of Science databases were searched till 2022 to identify studies. Studies that reported characteristics and outcomes of participants with cirrhosis that had a TIPS inserted in preparation for extrahepatic, abdominal surgery, were included., Results: Twenty-one studies (292 patients) were included, of which three were comparative studies and the remaining case series or case reports. A TIPS was inserted in 190 patients prior to surgery. At least one clinical sign of portal hypertension identified by ascites, varices, and/or hepatic encephalopathy were present in all patients except one patient. Fifty eight percent had decompensated cirrhosis. TIPS insertion was successful in all patients. Eighty-nine percent of patients underwent surgery. The cumulative 30-day postoperative mortality was 2% (3/148). There were 97 complications reported in 168 patients (57%). In the three comparative studies, there was no difference in mortality or morbidity among patients who underwent TIPS prior to surgery compared to those who did not undergo TIPS prior to surgery., Conclusion: Preoperative TIPS has been used to improve surgical candidacy in patients with cirrhosis undergoing extrahepatic, abdominal surgery, while reducing complications of portal hypertension. However, there is not enough evidence to support that TIPS insertion prior to extrahepatic, abdominal surgery significantly improves surgical outcomes in patients with cirrhosis and further studies are needed., (Copyright © 2024 Copyright: © 2024 Saudi Journal of Gastroenterology.)

Background: Ascites in children is multifactorial and serum ascites albumin gradient (SAAG) ≥1.1 helps differentiate portal hypertension (PHTN) related from non-PHTN ascites., Aims: We evaluated the aetiology and diagnostic accuracy of SAAG in children with ascites., Methods: Children with ascites were retrospectively evaluated. Etiological diagnosis was based on clinical presentation and investigations. All cases with ascitic fluid analysis and a definite diagnosis were included for calculating the utility of SAAG., Results: We enrolled 878 children (568[64.7%] boys). Majority were PHTN related (638[72.7%]) and secondary to acute viral hepatitis (98,15.4%), acute liver failure (185,29%), chronic liver disease (276,43.3%) and Budd-Chiari syndrome (79,12.4%). Other causes included tubercular (46,5.2%), pancreatic (32,3.6%), chylous (20,2.3%), biliary (12,1.4%), pseudoascites (16,1.8%), infections (46,5.2%), nephrotic (26,2.9%), malignancy (23,2.6%), cardiac (9,1.0%) and others (10,1%). SAAG (n = 305) correctly differentiated PHTN and non-PHTN ascites in 272 (89.2%) cases, with a high sensitivity (97%), specificity (93%) and diagnostic accuracy (95.8%). Reasons for inaccurate SAAG included mixed ascites (n = 9), different day serum and ascitic fluid albumin estimation (n = 5), serum albumin ≤1.1 g/dL (n = 2), chylous ascites (n = 3), hypergammaglobulinemia (n = 1), albumin infusions (n = 1) and unexplained (n = 12)., Conclusions: Nearly 27% children had non-PHTN related ascites. SAAG differentiates PHTN from non-PHTN ascites with a diagnostic accuracy of 95%., Competing Interests: Declaration of competing interest None of the authors have any conflict of interest to be disclosed, (Copyright © 2024 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Portal vein thrombosis (PVT) refers to the development of a non-malignant obstruction of the portal vein, its branches, its radicles, or a combination. This Review first provides a comprehensive overview of all aspects of PVT, namely the specifics of the portal venous system, the risk factors for PVT, the pathophysiology of portal hypertension in PVT, the interest in non-invasive tests, as well as therapeutic approaches including the effect of treating risk factors for PVT or cause of cirrhosis, anticoagulation, portal vein recanalisation by interventional radiology, and prevention and management of variceal bleeding in patients with PVT. Specific issues are also addressed including portal cholangiopathy, mesenteric ischaemia and intestinal necrosis, quality of life, fertility, contraception and pregnancy, and PVT in children. This Review will then present endpoints for future clinical studies in PVT, both in patients with and without cirrhosis, agreed by a large panel of experts through a Delphi consensus process. These endpoints include classification of portal vein thrombus extension, classification of PVT evolution, timing of assessment of PVT, and global endpoints for studies on PVT including clinical outcomes. These endpoints will help homogenise studies on PVT and thus facilitate reporting, comparison between studies, and validation of future studies and trials on PVT., Competing Interests: Declaration of interests VHG has received speaker fees from Gore Medical and Cook Medical. ADG has received consulting fees from Astrazeneca and Swedish Orphan Biovitrum. MR serves as consultant from Quantum Surgical (fees paid to institution) and has received speaker fees from Servier, Guerbet, GE Healthcare, Ipsen, Angiodynamics, and AstraZeneca. FN has received speaker fees from Advanz and Permanyer. VML serves as consultant from AstraZaneca and has received speaker fees and travel support from Albireo Ipsen. AB serves as consultant for Boehringer Ingelheim and has received speaker fees from GE Healthcare and Hologic. AA has served as a lecturer for Boehringer-Ingelheim and Gore Medical. AN has received research funding from MSD-Avenir and consulting fees from Abbvie and Janssen. MM serves as a lecturer for AbbVie, Ipsen, Echosens, Gilead Sciences, and Gore Medical, and has received travel support from AbbVie and Gilead Sciences. DP has served as lecturer for Boehringer-Ingelheim and Gore Medical. DT has received research grants from Boehringer-Ingelheim and Novartis and has served as lecturer for Gore Medical. JCGP has received speaker fees from Gore Medical and research grants from Mallinkrodt and AstraZeneca. All other authors declare no competing interests., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Background and Aims: The fully-covered self-expanding metal stent (SEMS) has a role in the management of refractory acute variceal haemorrhage. The aim of this study was to evaluate its effectiveness and complications in real-world practice., Patients and Methods: An observational, descriptive, multicenter study was carried out. Eight patients with clinically significant portal hypertension who underwent a SEMS were included., Results: SEMS placement controlled acute bleeding in 7 patients with technical success. Stents were removed after a median of 8 days. Rescue transjugular intrahepatic portosystemic shunt was performed around 48 hours after SEMS placement. Four patients survived after successful SEMS removal. The most common adverse event was stent loop in 2 patients., Conclusions: In our experience, SEMS was highly effective in controlling acute refractory variceal bleeding. Bleeding-related mortality rate was probably due to impossibility of TIPS implantation. Stent loop was a common limiting factor.

Background: Patients with hepatocellular carcinoma (HCC) and cirrhosis can present with features of severe portal hypertension, which can be worsened further by portal vein tumoral thrombosis (PVTT). Due to the technical difficulties and short survival of these patients, HCC was traditionally considered a relative contra-indication for transjugular intrahepatic portosystemic shunt (TIPS). However, there is an increasing body of evidence, mainly from China, supporting the use of TIPS in HCC. The present study aimed at analyzing the efficacy and safety of TIPS in patients with HCC., Methods: From 2000 through May 2023, MEDLINE, Embase and Scopus were searched for studies analyzing the outcome of TIPS in HCC. Technical and clinical success, adverse events (AE) and mortality were the main outcomes assessed. With the use of a random effects model, the event rates were combined., Results: Total 19 studies with 1498 patients were included in the final analysis. The pooled technical and clinical success rates with TIPS in HCC were 98.8% (98.0-99.7) and 94.1% (91.2-97.0), respectively. After TIPS, ascites was controlled in 89.2% (85.1-93.3) of the cases, while rebleeding was observed in 17.2% (9.4-25.0) of cases on follow-up. The pooled incidence of overall AE, serious AE and post-TIPS hepatic encephalopathy (HE) was 5.2% (2.5-7.9), 0.1% (0.0-0.4) and 25.1% (18.7-31.5), respectively. On follow-up, 11.9% (7.8-15.9) of the patients developed shunt dysfunction requiring re-intervention., Conclusion: The present analysis supports the feasibility, safety and efficacy of TIPS in the management of portal hypertension in patients with HCC., (© 2024. Indian Society of Gastroenterology.)

Background: Portal hypertensive enteropathy (PHE) is a small-bowel lesion observed in patients with portal hypertension. The clinical significance of endoscopic findings in PHE remains unclear. We aimed to clarify the clinical significance and predictive factors of capsule endoscopic findings in patients with PHE based on long-term outcomes., Methods: This retrospective study enrolled 55 patients with PHE (33 males and 22 females; median age, 64 years; range, 23-87) followed for > 3 years using capsule endoscopy (CE) between February 2009 and May 2023. We evaluated the clinical factors affecting PHE exacerbations and the effects of PHE exacerbations on gastrointestinal bleeding by comparing exacerbated and unchanged PHE groups., Results: Overall, 3 (5%) patients showed improvement, 33 (60%) remained unchanged, and 19 (35%) showed exacerbation on follow-up CE. In the exacerbated group, the rates of worsened fibrosis-4 index, exacerbated esophageal varices, and exacerbated portal hypertensive gastropathy were significantly higher than those in the unchanged group (21%, 32%, and 42% vs. 3%, 6%, and 12%, respectively; P < 0.05), and the rate of splenectomy was significantly lower in the exacerbated group than in the unchanged group (5% vs. 39%, respectively; P < 0.01). In multivariate analysis, exacerbation of esophageal varices and absence of splenectomy were significantly associated with PHE exacerbation. The rate of gastrointestinal bleeding after follow-up CE was significantly high in the exacerbated group (log-rank, P = 0.037)., Conclusions: Exacerbation of esophageal varices and splenectomy were significantly associated with exacerbation of PHE. Exacerbated PHE requires specific attention to prevent gastrointestinal bleeding., (© 2024. The Author(s).)

Competing Interests: Intérêts concurrents :: Aucun déclaré.

Background: Sinistral portal hypertension (SPH) is the only type of portal hypertension that is entirely curable. However, it can easily cause varicose veins in the esophagus and/or stomach, as well as upper gastrointestinal hemorrhage. This study aimed to investigate the clinical characteristics and treatments of sinistral portal hypertension., Methods: All patients with pancreatic disease were included in this retrospective cohort study at the Affiliated Hospital of Southwest Medical University (Luzhou, China) from September 2019 to September 2021. The required information including the patient's demographics, serum laboratory indicators, imaging and endoscopy examinations, and clinical features were gathered and evaluated. The results were expressed as numbers and percentages., Results: Out of the 830 patients with pancreatic diseases, 61 (7.3%) developed SPH. The most common cause of SPH was acute pancreatitis (80.3%), followed by chronic pancreatitis (11.5%). The splenic vein was the most frequently affected vein in patients (45/61, 73.8%). The findings of the contrast-enhanced computed tomography (CECT) indicated that 51 cases (83.6%) had gastric fundal-body varices, and three cases had combined gastric and esophageal varices. In the perigastric collateral channel formation, gastroepiploic varices (43/61, 70.5%) most frequently occurred in patients with SPH. Splenomegaly was a prevalent manifestation in SPH patients (45.9%). Five cases had gastrointestinal variceal hemorrhage., Conclusion: SPH was associated with the patency of the splenic vein and the formation of distinctive perigastric collateral veins. Surgery and/or endoscopic treatment were recommended, particularly for patients who have experienced a significant amount of gastrointestinal bleeding and have failed conservative treatment., Competing Interests: None declared., (Copyright: © Iranian Journal of Medical Sciences.)

Porto-sinusoidal vascular disease (PSVD) is the medical diagnosis for a patient who has portal hypertension in the absence of cirrhosis on liver biopsy. There are several specific histologic findings for PSVD, including obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal fibrosis. Epidemiologic reports vary widely among regions; PSVD comprises less than 10% of causes of portal hypertension in Western countries but incidence has been found to be as high as 48% in India. There is an expansive list of etiologies that have been reported to cause PSVD., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Portopulmonary hypertension (POPH), hepatopulmonary syndrome, and hepatic hydrothorax constitute significant complications of portal hypertension, with important implications for management and liver transplantation (LT) candidacy. POPH is characterized by obstruction and remodeling of the pulmonary resistance arterial bed. Hepatopulmonary syndrome is the most common pulmonary vascular disorder, characterized by intrapulmonary vascular dilatations causing impaired gas exchange. LT may improve prognosis in select patients with POPH. LT is the only effective treatment of hepatopulmonary syndrome. Hepatic hydrothorax is defined as transudative pleural fluid accumulation that is not explained by primary cardiopulmonary or pleural disease. LT is the definitive cure for hepatic hydrothorax., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Acute kidney injury (AKI) is a common complication among patients with decompensated cirrhosis and its development is associated with worse prognosis in terms of survival. Patients with decompensated cirrhosis may develop a unique type of AKI, known as hepatorenal syndrome (HRS-AKI), characterized by marked impairment of kidney function due to haemodynamic changes that occur in late stages of liver cirrhosis. Besides, patients with cirrhosis also may develop chronic alterations of kidney function (chronic kidney disease, CKD), the incidence of which is increasing markedly and may be associated with clinical complications. The aim of this review is to provide the reader with an update of the most relevant aspects of alterations of kidney function in patients with cirrhossi that may be useful for theri clinical practice., Competing Interests: Disclosure P. Ginès has received research funding from Gilead, United States, Mallinckrodt, Grifols, Spain, Ferring, Switzerland and has consulted or attended advisory boards for Grifols SA, Ferring Pharmaceuticals, Gilead, Intercept, Martin Pharmaceuticals, Promethera, Sequana, RallyBio, Behring, Ocelot Bio, SeaBe Life, Merck, Sharp and Dohme, and Boehringer-Ingelheim. Authors’ contributions: A. Juanola, A.T. Ma, J. Gratacós-Ginès, A. Soria, C. Solé, E. Pose and P. Ginès: review and interpretation of data, drafting of the manuscript, and critical revision of the manuscript., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: Hepatic venovenous communications (HVVC) is detectable in more than one-third of cirrhotic patients, where portal hypertension (PHT) tends to present more severely. We aimed to explore the prognostic implications of HVVC in patients with sinusoidal PHT treated by transjugular intrahepatic portosystemic shunt (TIPS)., Method: The multicenter data of patients (2020-2022) undergoing balloon-occluded hepatic venography during TIPS were retrospectively analyzed. Pre-TIPS total bile acids (TBA) levels in portal, hepatic and peripheral veins were compared between groups. The primary endpoint was the development of overt hepatic encephalopathy (HE) within one year after TIPS., Results: 183 patients were eligible and classified by the presence (n = 69, 37.7 %) or absence (n = 114, 62.3 %) of HVVC. The agreement between wedged hepatic venous pressure and portal venous pressure was poor in HVVC group (intraclass correlation coefficients [ICC]: 0.141, difference: 13.4 mmHg, p < 0.001), but almost perfect in non-HVVC group (ICC: 0.877, difference: 0.4 mmHg, p = 0.152). At baseline, patients with HVVC had lower Model for end-stage liver disease scores (p < 0.001), blood ammonia levels (p < 0.001), TBA concentrations in the hepatic (p = 0.011) and peripheral veins (p = 0.049) rather than in the portal veins (p = 0.516), and a higher portosystemic pressure gradient (p = 0.035), suggesting more effective intrahepatic perfusion in this group. Within 1-year post-TIPS, HVVC group had a lower incidence of overt HE (11.7 % vs. 30.5 %, p = 0.004, HR: 0.34, 95 % CI: 0.16-0.74, absolute risk difference [ARD]: -17.4) and an improved liver transplantation-free survival rate (97.1 % vs. 86.8 %, p = 0.021, HR: 0.16, 95 % CI: 0.05-0.91, ARD: -10.3)., Conclusions: For patients with sinusoidal PHT treated by TIPS, the presence of HVVC was associated with a reduced risk of overt HE and a potential survival benefit., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier B.V.)

Portal hypertension has cerebral consequences via its causes and complications, namely hepatic encephalopathy (HE), a common and devastating brain disturbance caused by liver insufficiency and portosystemic shunting. The pathogenesis involves hyperammonemia and systemic inflammation. Symptoms are disturbed personality and reduced attention. HE is minimal or grades I to IV (coma). Bouts of HE are episodic and often recurrent. Initial treatment is of events that precipitated the episode and exclusion of nonhepatic causes. Specific anti-HE treatment is lactulose. By recurrence, rifaximin is add-on. Anti-HE treatment is efficacious also for prophylaxis, but emergence of HE marks advanced liver disease and a dismal prognosis., Competing Interests: Disclosure All authors have nothing to disclose. This research was generously supported by grants from The Foundation of Manufacturer Vilhelm Pedersen and Wife, and the Novo Nordisk Foundation, Denmark (NFF19OC0055039 and NNF20OC0059717)., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background/aims: Transjugular intrahepatic portosystemic shunt (TIPS) is a commonly performed procedure in patients with liver cirrhosis to treat portal hypertension-related conditions, including variceal bleeding and refractory ascites. However, while the increased risk of hepatic encephalopathy (HE) after TIPS is important to consider when determining whether a patient is a good candidate for TIPS, currently there is no widely used method to predict the development of post-TIPS HE, although the model for end-stage liver disease (MELD) score is used to predict post-TIPS mortality. We conducted a systematic review and meta-analysis to evaluate sarcopenia as a risk factor for HE and mortality in patients undergoing TIPS., Methods: A comprehensive search strategy was used to identify reports of post-TIPS HE and mortality in sarcopenia vs. non-sarcopenia patients with liver cirrhosis who received TIPS in March 2023. Open Meta Analyst was used to compute the results., Results: Twelve studies with 2056 patients met inclusion criteria and were included in the final meta-analysis. Sarcopenia was associated with a significantly higher post-TIPS HE rate than non-sarcopenia (risk ratio [RR]: 1.68, 95% CI: 1.48-1.92, p < 0.00001, I 2  = 65%), as well as a significantly higher post-TIPS mortality rate (RR: 1.73, 95% CI: 1.14-2.64, p < 0.00001, I 2  = 87%)., Conclusion: Patients with sarcopenia have a significantly increased risk of post-TIPS HE and mortality. Presence of sarcopenia should be considered when weighing the risks and benefits of performing TIPS in patients with cirrhosis. Further studies are needed to determine the clinical utility of important risk factors such as sarcopenia on post-TIPS outcomes., (© 2023. Indian Society of Gastroenterology.)

Patients with cirrhosis and clinically significant portal hypertension are at high risk of developing bacterial infections (BIs) that are the most common trigger of acute decompensation and acute-on-chronic liver failure. Furthermore, after decompensation, the risk of developing BIs further increases in an ominous vicious circle. BIs may be subtle, and they should be ruled out in all patients at admission and in case of deterioration. Timely administration of adequate empirical antibiotics is the cornerstone of treatment. Herein, we reviewed current evidences about pathogenesis, clinical implications and management of BIs in patients with cirrhosis and portal hypertension., Competing Interests: Disclosure PA held a patent with Biovie, received advisory board fees from Biovie, Biomarin, and GenFit and speaking fees from Grifols, Kedrion, and Bhering. SP received consulting fees from Plasma Protein Therapeutics Association and speaking fees from Grifols and Medscape. SI has nothing to disclose regarding the work under consideration for publication. No specific funding supported this study. Authors’ contribution: S. Incicco, P. Angeli, and S. Piano reviewed the literature and drafted the article., (Copyright © 2024 Elsevier Inc. All rights reserved.)

In portal hypertension, acute variceal bleed is the cause of 2/3rd of all upper gastrointestinal bleeding episodes. It is a life-threatening emergency in patients with cirrhosis. Nonselective beta-blockers by decreasing the hepatic venous pressure gradient are the mainstay of medical therapy for the prevention of variceal bleeding and rebleeding. Evaluation of the severity of bleed, hemodynamic resuscitation, prophylactic antibiotic, and intravenous splanchnic vasoconstrictors should precede the endoscopy procedure. Endoscopic band ligation is the recommended endotherapy. Rescue transjugular intrahepatic port-systemic shunt (TIPS) is recommended for variceal bleed refractory to endotherapy. In patients with a high risk of failure of combined pharmacologic and endoscopic therapy, pre-emptive TIPS may improve the outcome. For gastric varices, "Sarin classification" is universally applied as it is simple and has therapeutic implication. For IGV1 and GOV2, injection cyanoacrylate glue is considered the endotherapy of choice. Endoscopic ultrasound is a useful modality in the management of gastric varices., Competing Interests: Disclosures None., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Competing Interests: Conflict of interest The authors declare that they have no conflicts of interest.

Ectopic varices account for 5% of variceal bleedings and occur outside the gastro-esophageal region. This review evaluates the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) for ectopic variceal management. A comprehensive search through PubMed, Scopus, Web of Science, and Embase was conducted until January 16, 2023, using relevant keywords. Case reports and case series with fewer than 10 patients on TIPS for ectopic variceal management were included. The quality assessment followed the Joanna Briggs Institute checklist for case reports. This systematic review evaluated 43 studies involving 50 patients with ectopic varices undergoing TIPS. Patients had a mean age of 54.3 years, half were female, and two were pregnant. Alcoholic liver disease (48%) and hepatitis C infection (26%) were common causes of portal hypertension. Ascites and splenomegaly were reported in 32% and 28% of the patients, respectively. Rectal, oral, and stomal variceal bleeding accounted for 62%, 16%, and 22% of the patients, respectively. Ectopic varices were mainly located in the duodenum (28%) and rectum (26%) regions. Complications affected 42% of the patients, re-bleeding in eleven and hepatic encephalopathy in seven. The follow-up lasted 12 months on average, and finally, 5 received a liver transplant. Mortality post-TIPS was 18%. Despite complications and a notable mortality rate, favorable outcomes were observed in almost half of the patients with ectopic variceal bleeding managed with TIPS. Further research is warranted to refine strategies and improve patient outcomes., (© 2024. The Author(s), under exclusive licence to American Society of Emergency Radiology (ASER).)

Ectopic varices are rare but potentially life-threatening conditions usually resulting from a combination of global portal hypertension and local occlusive components. As imaging, innovative devices, and interventional radiologic techniques evolve and are more widely adopted, interventional radiology is becoming essential in the management of ectopic varices. The interventional radiologist starts by diagnosing the underlying causes of portal hypertension and evaluating the afferent and efferent veins of ectopic varices with CT. If decompensated portal hypertension is causing ectopic varices, placement of a transjugular intrahepatic portosystemic shunt is considered the first-line treatment, although this treatment alone may not be effective in managing ectopic variceal bleeding because it may not sufficiently resolve focal mesenteric venous obstruction causing ectopic varices. Therefore, additional variceal embolization should be considered after placement of a transjugular intrahepatic portosystemic shunt. Retrograde transvenous obliteration can serve as a definitive treatment when the efferent vein connected to the systemic vein is accessible. Antegrade transvenous obliteration is a vital component of interventional radiologic management of ectopic varices because ectopic varices often exhibit complex anatomy and commonly lack catheterizable portosystemic shunts. Superficial veins of the portal venous system such as recanalized umbilical veins may provide safe access for antegrade transvenous obliteration. Given the absence of consensus and guidelines, a multidisciplinary team approach is essential for the individualized management of ectopic varices. Interventional radiologists must be knowledgeable about the anatomy and hemodynamic characteristics of ectopic varices based on CT images and be prepared to consider appropriate options for each specific situation. © RSNA, 2024 Supplemental material is available for this article.

Background and Aims: Performing a Transjugular intrahepatic portal system shunt (TIPS) in patients with portal vein cavernous transformation (CTPV) poses significant challenges. As an alternative, transjugular extrahepatic portal vein shunt (TEPS) may offer a potential solution for these patients. Nonetheless, the effectiveness and safety of TEPS remain uncertain. This case series study aimed to evaluate the efficacy and safety of TEPS in treating patients with CTPV portal hypertension complications., Methods: The study encompassed a cohort of 22 patients diagnosed with CTPV who underwent TEPS procedures. Of these, 13 patients manifested recurrent hemorrhagic episodes subsequent to conventional therapies, 8 patients grappled with recurrent or refractory ascites, and 1 patient experienced acute bleeding but refused endoscopic treatment. Comprehensive postoperative monitoring was conducted for all patients to rigorously evaluate both the technical and clinical efficacy of the intervention, as well as long-term outcomes., Results: The overall procedural success rate among the 22 patients was 95.5% (21/22).During the TEPS procedure, nine patients were guided by percutaneous splenic access, three patients were guided by percutaneous hepatic access, five patients were guided by transmesenteric vein access from the abdomen, and two patients were guided by catheter marking from the hepatic artery. Additionally, guidance for three patients was facilitated by pre-existing TIPS stents. The postoperative portal pressure gradient following TEPS demonstrated a statistically significant decrease compared to preoperative values (24.95 ± 3.19 mmHg vs. 11.48 ± 1.74 mmHg, p < 0.01).Although three patients encountered perioperative complications, their conditions ameliorated following symptomatic treatment, and no procedure-related fatalities occurred. During a median follow-up period of 14 months, spanning a range of 5 to 39 months, we observed four fatalities. Specifically, one death was attributed to hepatocellular carcinoma, while the remaining three were ascribed to chronic liver failure. During the follow-up period, no instances of shunt dysfunction were observed., Conclusions: Precision-guided TEPS appears to be a safe and efficacious intervention for the management of CTPV., (© 2024. The Author(s).)

Background: Spleen stiffness measurement (SSM) is a recently developed non-invasive method for predicting clinically significant portal hypertension and esophageal varices in compensated advanced liver disease or cirrhosis. This study aims to validate the accuracy of SSM for screening high-risk esophageal varices among cirrhotic patients in Indonesia., Methods: This is a single-center, cross-sectional study. Patients with liver cirrhosis who underwent endoscopy at Cipto Mangunkusumo Hospital, Jakarta were included. Clinical data and data from laboratory tests, endoscopy, liver, and spleen stiffness measurement by transient elastography (TE) were collected. A 100 Hz spleen-dedicated TE probe was used for SSM., Results: Of 86 patients, 52 had high-risk esophageal varices. The median (IQR) value of SSM were significantly higher in patients with high-risk varices [36.1 kPa (IQR 21.5-59.1) vs. 70.3 kPa (IQR (52.2-86.0); p<0.001). SSM with a low cutoff value of 20 kPa had sensitivity and negative predictive value of 98.1% and 87.5%, respectively. The high cutoff value of 70 kPa had specificity and positive predictive value of 82.4% and 81.3%, respectively., Conclusion: SSM is useful for screening high-risk esophageal varices. Furthermore, the new dual cutoff value can help rule-in and rule-out high-risk esophageal varices among cirrhotic patients in Indonesia.

Early identification of clinically significant portal hypertension (CSPH) is crucial in cirrhosis, as it is the primary underlying mechanism of cirrhosis complications. One such complication is esophageal varices, which are associated with high morbidity and mortality. Hepatic venous pressure gradient (HVPG) measurements and endoscopy, considered the gold standard for these conditions, are invasive methods that are limited, costly, and inconvenient. To address this challenge, a combination of non-invasive tests can be employed to predict CSPH and esophageal varices. A validated approach involves combining liver stiffness measurement (LSM) by transient elastography (TE) with platelet count. Additionally, spleen stiffness measurement (SSM) has emerged as a promising method for evaluating high-risk varices, as reported by Nababan et al. Therefore, non-invasive methods can be utilized to identify patients who need invasive procedures or potentially replace invasive procedures altogether.

Portal cavernoma thrombosis is a complication of portal cavernoma. We describe the case of a 74-year-old patient who presented to the emergency department with abdominal pain. The computed tomography scan showed a mass from the head of the pancreas to the hepatic hilum not enhanced after injection of iodinated contrast. There was no dilatation of the bile ducts. Abdominal magnetic resonance ruled out a tumour and confirmed a portal cavernoma thrombosis. In 50 % of cases the etiology of the portal cavernoma is unknown. It is often asymptomatic. It may be discovered in case of complications of portal hypertension. In rare cases the portal cavernoma can compress the bile ducts. To our knowledge, portal cavernoma thrombosis has only been described in one article. It is important to search for a thrombophilic disorder when such a complication is found. We share this case report in order to raise awareness in the medical community about this rare complication.

Competing Interests: Declaration of competing interest The authors declare that they have no competing interests.

Background and Aims: Wilson's disease may progress to cirrhosis and clinically significant portal hypertension (CSPH). We aimed to assess the prevalence and prognostic impact of CSPH-related features on hepatic decompensation and transplant-free survival in patients with Wilson's disease., Methods and Results: About 137 patients with Wilson's disease (Leipzig score ≥4), followed for a median observation period of 9.0 (3.9-17.7) years at the Vienna General Hospital, were included in this retrospective study. Overall, 49 (35.8%) developed features of CSPH: 14 (10.2%) varices, 40 (29.2%) splenomegaly, 20 (14.6%) ascites, 18 (13.1%) hepatic encephalopathy and 3 (2.2%) experienced acute variceal bleeding. Overall, 8 (5.8%) patients died, including three deaths caused by CSPH-related complications. Within 10 years, compensated patients with features of CSPH developed more decompensation events (8.3% vs. 1.5% in patients without CSPH, p = 0.3) and had worse transplant-free-survival (91.7% vs. 98.6%), which further declined in patients with hepatic decompensation (26.7%, log-rank: p < 0.0001). Patients with liver stiffness <15 kPa and normal platelets (≥150 G/L) were less likely to decompensate within 10 years (2.6% vs. 8.4%, p = 0.002) and had a better 10-year transplant-free-survival (97.7% vs. 83.9%, p = 0.006)., Conclusions: Patients with Wilson's disease developing features of CSPH are at an increased risk for hepatic decompensation and liver-related mortality, warranting for regular screening and timely initiation of effective CSPH-directed treatments., (© 2024 The Author(s). Alimentary Pharmacology & Therapeutics published by John Wiley & Sons Ltd.)

Some patients with refractory esophagogastric varices require surgery, such as gastric devascularization and splenectomy (Hassab's procedure). However, these patients are at risk of perioperative morbidities when undergoing devascularization to develop collateral vessels. We performed a more simplified procedure, splenectomy, and en bloc gastropancreatic fold division (GPFD) with hand-assisted laparoscopic surgery. Four patients with refractory esophagogastric varices and portal hypertension underwent splenectomy and GPFD. We reviewed patients' perioperative laboratory and morphological data, operative variables, and postoperative outcomes. Esophagogastric varices improved in 3 (75%) of the 4 patients. In one patient, esophageal varices (F1RC0) were observed 3 years after surgery, but they required no treatment and only received follow-up. Treatment with splenectomy and GPFD is not only less invasive than Hassab's procedure but also provides effective outcomes for refractory esophagogastric varices., (© 2023. The Author(s) under exclusive licence to Springer Nature Singapore Pte Ltd.)

Mean arterial blood pressure (MAP), which decreases as portal hypertension progresses, may be a modifiable risk factor among patients with cirrhosis. We included adults enrolled in the Functional Assessment in Liver Transplantation study. We completed latent class trajectory analyses to define MAP trajectories. We completed time-dependent Cox-regression analyses to test the association between outpatient MAP and 3 cirrhosis-related outcomes: (1) stage 2 acute kidney injury (AKI), defined as a ≥200% increase in serum creatinine from baseline; (2) a 5-point increase in the MELD-Na score, defined as the incidence of increase from initial MELD-Na; (3) waitlist mortality, defined as death on the waitlist. For each outcome, we defined MAP cut points by determining the maximally selected Log-rank statistic after univariable Cox-regression analyses. Among the 1786 patients included in this analysis, our latent class trajectory analyses identified 3 specific outpatient MAP trajectories: "stable-low," "stable-high," and "increasing-to-decreasing." However, >80% of patients were in a "stable-low" trajectory. We found in adjusted analyses that outpatient MAP was associated with each of our outcomes: Stage 2 AKI (adjusted hazard ratio 0.88 per 10 mm Hg increase in MAP [95% CI: 0.79-0.99]); 5-point increase in MELD-Na (adjusted hazard ratio: 0.91 [95% CI: 0.86-0.96]; waitlist mortality (adjusted hazard ratio: 0.89 [95% CI: 0.81-0.96]). For each outcome, we found that an outpatient MAP of 82 mm Hg was most associated with outcomes ( p <0.05 for all). Our study informs the association between outpatient MAP and cirrhosis-related outcomes. These findings, coupled with the identification of specific thresholds, lay the foundation for the trial of targeted outpatient MAP modulation in patients with cirrhosis., (Copyright © 2024 American Association for the Study of Liver Diseases.)

A 52-year-old male diagnosed with severe acute pancreatitis developed persistent abdominal distension and intermittent emesis six weeks after treatment. Computed tomography (CT) showed that the shape of the pancreas was not clear, and a pseudocyst (11.2*6.6 cm) existed in the tail of the pancreas, which compressed the portal vein and led to local portal hypertension. A transpapillary pancreatic duct stent (7F*9 cm) was successfully placed for drainage of fluid collection despite pancreatic duct obstruction, with one end into the pseudocyst cavity and the other into the duodenum. Duodenoscope observed outflow of the fluid collection immediately. Four days after the surgery, CT scan validated the location of the pancreatic duct stent, and the pancreatic pseudocyst was smaller than before. Another week later, the pancreatic pseudocyst nearly disappeared. No significant esophagogastric varix was observed by gastroscope, indicating a regression of local portal hypertension. The patient resumed eating normally, did not complain of vomiting, and was discharged from our hospital.

Objective: Examine portal hypertension (PHT) impact on postoperative and survival outcomes in hepatocellular carcinoma (HCC) patients after liver resection (LR), specifically exploring distinctions between indirect signs and invasive measurements of PHT., Background: PHT has historically discouraged LR in individuals with HCC due to the elevated risk of morbidity, including liver decompensation (LD)., Methods: A systematic review was conducted using 3 databases to identify prospective-controlled and matched cohort studies until December 28, 2022. Focus on comparing postoperative outcomes (mortality, morbidity, and liver-related complications) and overall survival in HCC patients with and without PHT undergoing LR. Three meta-analysis models were utilized: for aggregated data (fixed-effects inverse variance model), for patient-level survival data (one-stage frequentist meta-analysis with gamma-shared frailty Cox proportional hazards model), and for pooled data (Freeman-Tukey exact and double arcsine method)., Results: Nine studies involving 1124 patients were analyzed. Indirect signs of PHT were not significantly associated with higher mortality, overall complications, PHLF or LD. However, LR in patients with hepatic venous pressure gradient (HVPG) ≥10 mm Hg significantly increased the risk of overall complications, PHLF, and LD. Despite elevated risks, the procedure resulted in a 5-year overall survival rate of 55.2%. Open LR significantly increased the risk of overall complications, PHLF, and LD. Conversely, PHT did not show a significant association with worse postoperative outcomes in minimally invasive LR., Conclusions: LR in the presence of indirect signs of PHT poses no increased risk of complications. Yet, in HVPG ≥10 mm Hg patients, LR increases overall morbidity and liver-related complications risk. Transjugular HVPG assessment is crucial for LR decisions. Minimally invasive approach seems to be vital for favorable outcomes, especially in HVPG ≥10 mm Hg patients., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Transjugular intrahepatic portosystemic shunt (TIPS) reduces portal hypertension complications. Its impact on hepatocellular carcinoma (HCC) remains unclear. We evaluated 42,843 liver transplant candidates with HCC from the Scientific Registry of Transplant Recipients (2002-2022). 4,484 patients with and without TIPS were propensity score-matched 1:3. Analysing wait-list changes in total tumor volume, HCC count, and alpha-fetoprotein levels, and assessing survival from listing and transplantation; TIPS correlated with a decreased nodule count (-0.24 vs. 0.04, p = 0.028) over a median wait period of 284 days (IQR 195-493) and better overall survival from listing (95.6% vs. 91.5% at 1 year, p < 0.0001). It was not associated with changes in tumor volume (0.28 vs. 0.11 cm³/month, p = 0.58) and AFP (14.37 vs. 20.67 ng/mL, p = 0.42). Post-transplant survival rates (91.8% vs. 91.7% at 1 year, p = 0.25) and HCC recurrence (5.1% vs. 5.9% at 5 years, p = 0.14) were similar, with a median follow-up of 4.98 years (IQR 2.5-8.08). While TIPS was associated with a reduced nodule count and improved waitlist survival, it did not significantly impact HCC growth or aggressiveness. These findings suggest potential benefits of TIPS in HCC management, but further studies need to confirm TIPS safety., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 El Hajji, Lacotte, Moeckli, Cauchy, Compagnon and Toso.)

Ectopic varices and associated bleeding, although rare, pose a significant risk to patients with portal hypertension, carrying a relatively high mortality rate. These varices can occur in various anatomical regions, excluding the gastroesophageal region, which is typically associated with portal vein drainage. The limited data available in the literature, derived mostly from case reports and series, make the diagnosis and treatment of ectopic variceal bleeding particularly challenging. Furthermore, it is crucial to recognize that ectopic varices in different sites can exhibit variations in key decision-making factors such as aetiology and vascular anatomy, severity and bleeding risk and hepatic reserve. These factors significantly influence treatment strategies and underscore the importance of adopting individualized management approaches. Therefore, the objective of this review is to provide a comprehensive overview of the fundamental knowledge surrounding ectopic varices and to propose site-oriented, stepwise diagnosis and treatment algorithms for this complex clinical issue. A multidisciplinary treatment approach is strongly recommended in managing ectopic varices. In addition, to enhance clinical reference, we have included typical case reports of ectopic varices in various sites in our review, while being mindful of potential publication bias., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Rational: Congenital hepatic fibrosis (CHF) is a rare autosomal recessive genetic disease, which is often diagnosed in children and young adults. The clinical manifestations of CHF were lack of specificity, mainly including portal hypertension related symptoms and signs, and normal or mildly abnormal liver function. When no obvious varices are indicated under endoscope, it can easily lead to misdiagnosis or missed diagnosis. We report this case in the hope of raising awareness of this disease., Patient Concerns: A 31 years old male patient with major clinical manifestations of unexplained thrombocytopenia for 5 years., Diagnoses: Results of ultrasound, magnetic resonance imaging (MRI) and computed tomography portal venography (CTV) showed that patient had liver cirrhosis with portal hypertension and liver biopsy revealed CHF., Intervention: Patient received ursodeoxycholic acid tablets, fuzheng huayu capsule, ganshuang granule, etc for liver protection treatment., Outcomes: The condition of patient stabilized after symptomatic treatment. Spleen resection will be considered during follow-up., Lessons: This case reminds us that in case of patients with negative endoscopic evaluation, ultrasonic, computed tomography (CT) and MRI examination should be performed at the same time to determine whether patients have portal hypertension. When patients with normal or mildly abnormal liver function had unexplained liver cirrhosis complicated with portal hypertension, the possibility of CHF should be considered., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: There are many options for the reduction of portal hypertension (pH) in cirrhotic patients, but all the current ones have side effects. Probiotics are a new approach for ameliorating the hyperdynamic circulation of cirrhotic patients. The aim of this study is to measure the effect of probiotics on pH in cirrhosis for the first time., Methods: A search was conducted across four electronic databases (PubMed, Scopus, Web of Science, Cochrane) for English-language records evaluating probiotic effects on pH in cirrhotic patients. Quality assessment used the Cochrane Collaboration's tool, employing a random-effects model in statistical analysis with Stata software version 1., Results: A search yielded 1,251 articles, which were narrowed down to 5 through screening. These studies, involving 158 participants across Canada, India, Spain, and Russia, focused on probiotic interventions in cirrhotic patients. Meta-analysis of two RCTs (66 participants) indicated a significant decrease in hepatic venous pressure gradient (HVPG) (SMD: -0.60 [-1.09, -0.12]). In single-arm analysis, four studies (58 participants) showed a substantial reduction in HVPG with probiotic use compared to the control (SMD: -2.55 [-3.42, -1.68])., Conclusion: In summary, it showcased a notable reduction in HVPG compared to the control group, indicating a potential advantage of probiotics in decreasing pH in cirrhotic patients. Further research with larger samples and robust designs is warranted., Competing Interests: Declaration of competing interest There are no conflicts of interest to be disclosed by the authors., (Copyright © 2024. Published by Elsevier Masson SAS.)

A 45-year-old man who was regularly followed up for Crohn's disease (CD) and maintained clinical remission with vedolizumab (VDZ). At 37 years old, he was diagnosed CD from longitudinal ulcers in the distal ileum by balloon-assisted enteroscopy (BAE). During the follow-up, liver enzyme elevation, splenomegaly and thrombocytopenia were in progress. Esophagogastric varices suggested chronic liver disease and portal hypertension. Magnetic resonance elastography (MRE) showed liver stiffness of 3.4 kPa and proton density fat fraction (PDFF) of 1.86%. He was diagnosed with granulomatous hepatitis based on a liver biopsy. The hepatic venous pressure gradient (HVPG) was mildly elevated at 7 mmHg, consistent with the pre-sinusoidal portal hypertension due to granulomatous hepatitis. We report a rare case with granulomatous hepatitis diagnosed from liver injury and portal hypertension, despite the stable intestinal symptoms of CD., (© 2024. Japanese Society of Gastroenterology.)

Background and Aims: Esophageal varices (EVs), irrespective of size, are the most-reliable indicators of clinically significant portal hypertension (CSPH) in patients with compensated advanced chronic liver disease (cACLD). While non-invasive tools (NITs) accurately identify those with varices needing treatment (VNTs), their role in identifying any EVs in patients with cACLD is not known., Methods: Patients with cACLD with reliable liver stiffness measurements (LSM), blood parameters and gastroscopy were retrospectively recruited from a multinational cohort. The performance of Baveno-VI (LSM > 20kPa and platelet count < 150,000/mm 3 ) and expanded Baveno-VI criteria (LSM > 25kPa or platelet count < 110,000/mm 3 ) was assessed to detect VNTs as well as any EVs. This performance was compared with the Baveno-VII possible CSPH criteria (LSM ≥ 15 kPa and platelet ≤ 150,000/mm 3 ) to evaluate its utility in detecting any EVs., Results: Patients with cACLD (n = 1200) of predominantly viral etiology (hepatitis B virus, 269; hepatitis C virus, 564; non-alcoholic fatty liver disease, 145; alcoholic liver disease, 130; other, 92) were included. Any EVs and VNTs were present in 514 (42.8%) and 70 (5.8%) patients, respectively. The Baveno-VI, expanded Baveno-VI and Baveno-VII criteria missed 29/514 (5.6%), 115/514 (22.4%) and 19/514 (3.7%) patients with any EVs, respectively, whereas they misclassified 517/686 (75.4%), 211/686 (30.8%) and 598/686 (87.4%) patients with no EVs as having a high risk of EVs. The Baveno-VI, expanded Baveno-VI and possible CSPH criteria missed 3/70 (4.3%), 15/70 (21.4%) and 0/70 (0%) VNTs, respectively., Conclusion: Both original Baveno-VI and Baveno-VII criteria were highly sensitive in detecting varices in cACLD, albeit with high misclassification rates., (© 2023. Indian Society of Gastroenterology.)

Background: Non-cirrhotic portal hypertension (NCPH) is a spectrum of liver diseases, including porto-sinusoidal vascular disorder, with portal hypertension (PH) in the absence of cirrhosis. The natural history and diagnostic approach to NCPH are not well understood., Aim: We aimed to evaluate disease progression and outcomes in NCPH., Methods: Patients with or at risk for NCPH were enrolled in a single centre prospective study; two groups were formed based on the presence of specific features of PH, such as varices, collaterals, portal hypertensive gastropathy or portal hypertensive bleeding. All participants underwent a baseline liver biopsy. Liver stiffness measurement (LSM), and imaging were repeated every 6-12 months., Results: Fifteen patients without specific features of PH (Group I), and 35 patients with specific features (Group II) were enrolled. The median follow-up time was 50 months. Group II had higher hepatic venous pressure gradients, non-invasive measures of PH and a lower platelet count (PLT) when compared to Group I. Rates of survival and decompensation were similar in both groups. Patients with PLT ≤100 K/mcL had lower survival compared to those with PLT >100 K/mcL. Patients with LSM ≥10 kPa had lower survival and survival without decompensation when compared to patients with LSM <10 kPa., Conclusions: Patients irrespective of specific features of PH had similar survival or survival without decompensation. Patients without specific features are at risk for disease progression and should be monitored closely. Thrombocytopenia and increased LSM are associated with severe forms of liver disease, which are strongly associated with outcomes., (© 2024 John Wiley & Sons Ltd. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA.)