Publications by authors named "Garcia-Pagan J"

Background:  V617F-mutated myeloproliferative neoplasms (MPN) exhibit abnormal proliferation of bone marrow progenitors and increased risk of thrombosis, specifically in splanchnic veins (SVT). The contribution of the endothelium to the development of the prothrombotic phenotype was explored.

Material And Methods:  Plasma and serum samples from V617F MPN patients with (n=26) or without (n=7) thrombotic debut and different treatments, were obtained (n=33).

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Background And Aims: Data on the effectiveness of classical non-selective beta-blockers (cNSBB, i.e., propranolol and nadolol) versus carvedilol in patients with cirrhosis are scarce.

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Article Synopsis
  • The study focused on identifying unique metabolomic signatures in patients with porto-sinusoidal vascular disorder (PSVD) and cirrhosis to improve diagnosis.
  • Serum samples from healthy volunteers and patients with PSVD or cirrhosis were analyzed using advanced techniques like liquid chromatography-mass spectrometry, identifying significant metabolic changes linked to PSVD.
  • Machine learning models were developed to distinguish PSVD from cirrhosis and healthy controls; key metabolites like taurocholic acid showed strong potential for non-invasive diagnostic use.
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Background & Aims: Patients with vascular liver diseases (VLD) are at higher risk of both severe courses of COVID-19 disease and thromboembolic events. The impact of SARS-CoV-2 vaccination in patients with VLD has not been described and represents the aim of our study.

Methods: International, multicenter, prospective observational study in patients with VLD analyzing the incidence of COVID-19 infection after vaccination, severity of side effects, occurrence of thromboembolic events and hepatic decompensation.

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Background: Cardiovascular disease (CVD) remains the most common cause of mortality in chronic kidney disease (CKD) patients. Several studies suggest that the Mediterranean diet reduces the risk of CVD due to its influence on endothelial function, inflammation, lipid profile, and blood pressure. Integrating metabolomic and proteomic analyses of CKD could provide insights into the pathways involved in uremia-induced CVD and those pathways modifiable by the Mediterranean diet.

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Portal vein thrombosis (PVT) is a frequent event among patients with advanced liver disease, with a prevalence reaching up to 26% in those awaiting liver transplantation (LT). Extensive thrombosis affecting the mesenteric vein confluence correlates with increased morbidity and mortality post-LT, particularly when it impedes physiological anastomosis or contraindicates the LT. Current guidelines advocate for routine PVT screening in all potential liver transplant candidates and prompt treatment upon detection.

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  • This study investigates the relationship between hepatic venous pressure gradient (HVPG) and direct portal pressure (DPP) in cirrhosis patients who still have esophageal varices (EV) after treatment to remove the underlying cause, even when HVPG is low (<10 mmHg).
  • Ten patients with hepatitis C virus (HCV) or alcohol-related cirrhosis were examined, showing that HVPG correlates well with portal pressure measurements but doesn't fully explain the persistence of varices post-treatment.
  • The research suggests that while HVPG reflects overall portal pressure accurately, the presence of varices after treatment needs further exploration, indicating a gap in understanding the benefits of treatment for patients with EV but low HVPG.
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Acute variceal bleeding in cirrhosis represents a critical clinical event that significantly impacts patient prognosis, with mortality rates increasing further after a second episode. This underscores the need for immediate intervention and optimal prophylaxis. The creation of a transjugular intrahepatic portosystemic shunt (TIPS) has been proven to be highly effective for managing esophageal variceal bleeding.

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  • Researchers aimed to evaluate spleen stiffness measurement (SSM) as a standalone non-invasive test for clinically significant portal hypertension (CSPH) in patients with compensated advanced chronic liver disease (cACLD) and compare it to the existing ANTICIPATE±NASH model.
  • The study involved 407 patients recruited from 16 expert centers in Europe, utilizing various non-invasive tests alongside hepatic venous pressure gradient measurements to assess CSPH probability.
  • The findings indicated the potential for SSM to enhance diagnostic capabilities, as models were created to evaluate its effectiveness and discriminative ability compared to existing methods using binary logistic regression analysis.
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Background & Aims: Whether non-invasive tests (NITs) can accurately select patients with cirrhosis requiring non-selective beta-blockers (NSBBs) for clinically significant portal hypertension (CSPH) and prevention of decompensation is unclear. Our aim was to test the performance of NIT-based algorithms for CSPH diagnosis using the prospective PREDESCI cohort. We investigated whether a new algorithm combining NITs with endoscopy could improve performance.

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Article Synopsis
  • - The study aimed to understand the natural history and prognostic factors of porto-sinusoidal vascular disorder (PSVD) by analyzing a large cohort of 587 patients across 27 centers, finding that the majority were asymptomatic at diagnosis, but many experienced complications related to portal hypertension.
  • - Over a median follow-up of 68 months, 8.5% of patients underwent liver transplantation, while 19% died, highlighting significant risks like portal hypertension-related bleeding and ascites, as well as the impact of age and liver function on prognosis.
  • - The findings indicate that the severity of underlying conditions and liver/renal function significantly influence survival chances, leading to the development of a nomogram for more accurate prognosis prediction in
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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.

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Transjugular intrahepatic portosystemic shunt (TIPS), a highly effective procedure reducing portal hypertension, has been in use for over seven decades and is now a cornerstone in managing portal hypertension-related complications such as variceal bleeding and ascites. Historically, TIPS has dealt with two main challenges: ensuring stent patency and preventing post-TIPS hepatic encephalopathy. The introduction of PTFE-coated stents markedly reduced the risk of TIPS dysfunction and stent patency is no longer a major concern.

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Background & Aims: Metabolic dysfunction-associated steatotic liver disease (MASLD) is a leading cause of advanced chronic liver disease (ACLD). Portal hypertension drives hepatic decompensation and is best diagnosed by hepatic venous pressure gradient (HVPG) measurement. Here, we investigate the prognostic value of HVPG in MASLD-related compensated ACLD (MASLD-cACLD).

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Introduction: The incidence of hepatocellular carcinoma (HCC) in Budd-Chiari syndrome (BCS) is unknown and there is no validated diagnostic work-up to define the liver nodules with arterial phase hyperenhancement (APHE), suggesting malignancy. This prospective study evaluates HCC incidence in a Western cohort of patients with BCS and assesses the performance of MRI with hepatobiliary contrast (HB-MRI) for nodule characterization.

Methods: Patients with BCS followed in our hospital were prospectively evaluated by MRI with extracellular contrast (EC-MRI).

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  • Oxaliplatin (OX) is linked to a condition called porto-sinusoidal vascular disorder (PSVD) in patients with colon cancer, and the study aimed to understand its natural progression and identify risk factors.
  • A multicenter study compared patients with PSVD due to OX treatment to controls without PSVD, looking at various data points and genetic markers.
  • The study found that an increase in spleen diameter was the strongest predictor of PSVD, and patients with low platelet counts a year after treatment faced a higher risk, suggesting they should be monitored for related complications.
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  • A study examined the risk of new thrombotic events in patients with non-cirrhotic portal vein thrombosis (NCPVT) linked to local factors after stopping anticoagulation therapy.
  • Out of 154 patients assessed, a significant portion had high-risk prothrombotic factors, with new thrombotic events occurring in 17 patients during a median follow-up of 52 months.
  • The results suggest that high-risk factors increase the likelihood of new thrombosis, while continuous anticoagulation treatment may reduce these risks effectively.
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Background & Aims: Porto-sinusoidal vascular disorder (PSVD) encompasses a group of liver diseases with vascular abnormalities that can cause portal hypertension in the absence of cirrhosis. The new diagnostic criteria allow for coexistence with other liver diseases, however its relationship with chronic hepatitis B (CHB) remains unclear. This study aimed to assess HBV prevalence in a PSVD cohort and evaluate its clinical impact.

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Background And Aims: Since the introduction of SARS-CoV-2 vaccines, several cases of vaccine-induced immune thrombocytopenia and thrombosis (VITT) have been described, especially cerebral vein thrombosis. We aimed to retrospectively collect all new cases of acute onset first or recurrent splanchnic vein thrombosis (SVT) following a recent SARS-CoV-2 vaccination within the Vascular Liver Disease Group network.

Approach And Results: New cases of SVT were identified from April 2021 to April 2022; follow-up was completed on December 31, 2022.

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  • This study examined the impact of splanchnic vein thrombosis (SVT) on prognosis and survival in patients with polycythemia vera (PV) or essential thrombocythemia (ET), comparing those with SVT at diagnosis to a matched control group.
  • Findings revealed that a majority of patients with SVT at diagnosis had a specific genetic mutation (JAK2), but this group also showed a significantly higher risk of death and lower event-free survival compared to controls.
  • The research indicated that the timing of SVT development (at diagnosis vs. follow-up) influences molecular risk factors, highlighting the importance of genetic testing for better assessing thrombotic risks and disease progression in younger patients with PV/ET.
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Over the past decade, significant advancements in pharmacological, endoscopic, and radiographic treatments have emerged in the management of patients with cirrhosis and esophagogastric varices or variceal hemorrhage. These advances have been in several areas, including the role of screening and primary prophylaxis (preventing an initial variceal bleed), evaluation and management of acute esophagogastric variceal hemorrhage, and in preventing variceal rebleeding. Therefore, we believe there is a need for an updated, evidence-based "narrative review" on this important clinical topic that will be relevant for internists, hospitalists, intensive care unit physicians, and those in training.

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Background: Porto-sinusoidal vascular disorder (PSVD) involves a group of rare vascular liver diseases of unknown aetiology that may lead to the development of portal hypertension and its life-threatening complications. Its pathophysiology is not well understood, and animal models described to date do not fully recapitulate human disease.

Methods: We developed three different PSVD rat models by either immunosensitization (repetitive intraportal LPS or intramuscular spleen extract injections) or toxic (Selfox: combination of FOLFOX and a selenium-enriched diet) treatment and characterized them at haemodynamic, histological, biochemical and transcriptional levels.

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Fontan-type surgery is the final step in the sequential palliative surgical treatment of infants born with a univentricular heart. The resulting long-term haemodynamic changes promote liver damage, leading to Fontan-associated liver disease (FALD), in virtually all patients with Fontan circulation. Owing to the lack of a uniform definition of FALD and the competitive risk of other complications developed by Fontan patients, the impact of FALD on the prognosis of these patients is currently debatable.

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