特発性門脈圧亢進症

Anti-centromere antibody (ACA) positivity in primary biliary cholangitis (PBC) has been associated with a portal hypertension-dominant clinical trajectory, even without advanced fibrosis. This study investigated whether porto-sinusoidal vascular disorder (PSVD)-spectrum portal venopathy may underlie this phenotype. This retrospective case-series study included three ACA-positive PBC-spectrum patients who underwent liver biopsy. The clinical characteristics, imaging findings, liver and spleen stiffness, hepatic venography, and hepatic venous pressure gradient (HVPG) were evaluated. Liver specimens were assessed for PSVD-spectrum portal venopathy and PBC-related bile duct injury. All patients presented with PSVD-spectrum portal venopathy on liver biopsy; PBC-defining bile duct lesions were present in two patients, and the presence of ductular reaction was suggestive of a biliary disease but not diagnostic for PBC in one patient. All patients had manifestations of portal hypertension with preserved liver synthetic function and noncirrhotic liver stiffness. The portal sandwich sign was present in all patients. When available, spleen stiffness was markedly elevated, indicating spleen-liver stiffness dissociation. HVPG was heterogeneous, with normal values in two patients and marked elevation in one patient. In summary, PSVD-spectrum portal venopathy may contribute to disproportionate portal hypertension in ACA-positive PBC-spectrum patients but constitutes a diagnostic gray zone because PBC is defined as exclusionary in PSVD definitions.

Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare venous malformation disorder causing recurrent gastrointestinal bleeding and severe anemia. We report three children illustrating endoscopic, surgical, and medical management. All presented with melena and transfusion-requiring anemia. Case 1 developed gastric perforation after endoscopic band ligation and underwent emergency primary repair with bowel-preserving excision of accessible lesions, followed by recovery. Case 2 had more than 100 gastrointestinal lesions and underwent lesion-directed wedge resections, achieving transfusion independence and hemoglobin normalization at 1 year. Case 3 had idiopathic extrahepatic portal vein obstruction, portal hypertension, and diffuse mesenteric disease that was not safely resectable; sirolimus was initiated, but intermittent bleeding and transfusion dependence persisted. Management should be individualized according to lesion burden, anatomical extent, procedural risk, and feasibility of bowel-preserving treatment. Surgery remains important for complications and resectable disease, whereas systemic therapy may be required for diffuse unresectable involvement.

BACKGROUND & AIMS: Transjugular Intrahepatic Portosystemic Shunt (TIPS) is an established treatment for portal hypertension (PH) with expanding indications. Hepatic encephalopathy (HE) is the main complication, affecting up to 50% of patients. Accurate HE-risk prediction is necessary for patient selection. Hence, we aimed to provide an overview of prediction models for post-TIPS HE. METHODS: We conducted a systematic review of studies developing or validating prediction models for post-TIPS HE, which included ≥ 100 patients. The search, conducted up to December 2025, covered databases such as Embase and Medline. Outcomes assessed included AUCs/C-indices and bias risk, evaluated using PROBAST. RESULTS: Twenty-seven studies were included, with 24 developing prediction models. All used retrospective cohorts of cirrhotic patients (population size: 106-621), with 25 cohorts from Asia (93%). Models varied in HE severity and prediction time frame, with 11 studies (41%) not reporting any. Discrimination was variable across studies. Child-Pugh score (CPS) and Model for End-Stage Liver Disease, evaluated in nine studies, showed moderate performance (AUCs/C-indices: 0.60-0.75). Models that included imaging predictors (42%) reported higher discrimination than clinical models alone (0.73-0.97 vs. 0.61-0.86). Common clinical predictors were age (68%), CPS (40%), and creatinine (28%). Models scored a high bias risk in PROBAST analysis (96%). Methodological concerns included inappropriate patient exclusion (63%), low event-per-variable rate (81%), inadequate validation (79%), and neglecting competing risks (93%). Applicability concerns involved using highly selected populations (44%) and specialized imaging predictors (33%). CONCLUSIONS: Current prediction models for post-TIPS HE, despite strong discrimination, cannot support clinical decision-making due to methodological and applicability concerns. Future research should prioritize models with accessible parameters, broad patient representation, competing risks, and rigorous validation to address overfitting. IMPACT AND IMPLICATIONS: Despite numerous studies, there remains no consensus on whether prediction models for post-TIPS HE can be integrated into clinical practice. This paper provides a comprehensive overview of existing models by highlighting their strengths and limitations. In addition, it identifies recurrent predictors across models and proposes a framework for future research aimed at improving the prediction of this challenging complication.

BACKGROUND: Idiopathic portal hypertension (IPH) is a rare non-cirrhotic cause of non-cirrhotic portal hypertension, characterized by splenomegaly, hypersplenism, and recurrent variceal bleeding. Transfusion-associated hemolytic anemia with autoimmune features following red blood cell transfusion is an uncommon but serious complication, and its occurrence in IPH patients undergoing invasive procedures has rarely been reported. This study aims to report a rare case of idiopathic portal hypertension complicated by transfusion-associated hemolytic anemia and to describe the clinical outcomes of covered stent transjugular intrahepatic portosystemic shunt followed by glucocorticoid therapy. CASE DESCRIPTION: A 20-year-old woman with a 3-year history of recurrent hematemesis and melena presented with acute upper gastrointestinal bleeding. She had chronic anemia (hemoglobin 56 g/L), severe splenomegaly crossing the anterior midline, and hypersplenism (white blood cell 1.53×109/L, platelets 35×109/L). Liver function and coagulation were preserved, and contrast-enhanced computed tomography (CT) showed portal hypertension without cirrhosis. Comprehensive workup excluded viral hepatitis, autoimmune liver disease, Budd-Chiari syndrome, and myeloproliferative neoplasms, leading to a diagnosis of IPH. She underwent covered stent transjugular intrahepatic portosystemic shunt (TIPS) placement, with portal pressure decreasing from 41 to 30 cmH2O. On postoperative day one, after transfusion of washed red blood cells, she developed acute jaundice, dark urine, and worsening anemia (hemoglobin 46 g/L). Laboratory findings revealed indirect hyperbilirubinemia (indirect bilirubin 72.0 µmol/L) and positive direct/indirect anti-human globulin tests, confirming transfusion-associated hemolytic anemia with autoimmune features. She was treated with methylprednisolone (40 mg/day) followed by oral prednisone tapered over 3 months, along with urinary alkalinization and choleretic agents. Hemolysis resolved within 3 days, and no recurrent bleeding occurred despite recent variceal hemorrhage. At 12-month follow-up, the TIPS remained patent, gastroscopy confirmed absence of esophageal varices, and hypersplenism parameters normalized without need for splenectomy. CONCLUSIONS: Covered stent TIPS may effectively control portal hypertension and improve hypersplenism in select IPH patients. However, transfusion-associated hemolytic anemia is a potential complication in alloimmunized patients receiving transfusions during TIPS, requiring prompt diagnosis and glucocorticoid therapy. Multidisciplinary collaboration among hepatologists, hematologists, and interventional radiologists is essential for optimal outcomes.

BACKGROUND & AIMS: Sinusoidal obstruction syndrome (SOS) is a potentially severe complication of conditioning regimens in hematopoietic stem cell transplantation (HSCT). Its long-term outcome is unknown. The aim of this study was to investigate the long-term liver outcome of patients with SOS. METHODS: We retrospectively analyzed the outcome of all patients with histologically proven SOS related to HSCT conditioning who survived 6 months or more after HSCT. 22 patients with SOS were included. RESULTS: Median follow-up after SOS was 6.1 years. During this period, 10 patients (45%) developed signs of portal hypertension and 8 (36%) of them liver-related complications (ascites, n=8; spontaneous bacterial peritonitis, n=1; hepatic encephalopathy, n=3; variceal bleeding, n=3). Cumulative incidence of signs of portal hypertension was 27% at 3 years. Patients who developed signs of portal hypertension were less likely to have received defibrotide (hazard ratio:0.096; p<0.001) and had lower mean arterial pressure than those who did not. Of the 10 patients who developed signs of portal hypertension during follow-up, 3 underwent a second liver biopsy; all biopsies showed features of porto-sinusoidal vascular disorder. Cumulative incidence of death, considering non-liver-related death as a competing event, was significantly higher in patients who developed signs of portal hypertension than in those who did not [hazard ratio: 10.7 (1.27-89.9)]. CONCLUSION: Development of signs of portal hypertension and liver-related complications are common in the long-term after SOS following HSCT, especially when patients did not receive defibrotide, and this affects patients' survival. Features consistent with a porto-sinusoidal vascular disorder may be seen in liver histology in a subset of patients at the long-term follow-up after SOS. IMPACT AND IMPLICATIONS: Long-term liver outcome of sinusoidal obstruction syndrome (SOS) following conditioning regimens in haematopoietic stem cell transplantation (HSCT) is unknown.This study demonstrates that signs of portal hypertension and liver-related complications are common during long-term follow-up after SOS following HSCT. Treatment of SOS with defibrotide seems to be associated with a lower risk of developing portal hypertension. Liver histology at the long-term follow-up after SOS may show features consistent with PSVD.SOS following HSCT might thus be removed from the PSVD exclusion criteria.