Porto-Sinosoidal Vascular Disorder, PSVD
Conditions
Interventions
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: Patients aged 18 years or older who meet 1 and 2, or 1 and 4, or 1, 3, and 5 can be included: 1. Have already undergone liver puncture examination, and liver biopsy excludes cirrhosis; 2. Clinically present with specific signs of portal hypertension (any of the following); (1) Esophagogastric varices or ectopic varices; (2) Portal hypertension-related bleeding; (3) Formation of portal venous collaterals; 3. Clinically present with non-specific signs of portal hypertension (any of the following); (1) Ascites (non-malignant); (2) Spleen longest axis greater than 13 cm; (3) PLT < 150 × 10^9/L; 4. Histological features of specific PSVD (any of the following); (1) Obliterative portal venopathy (thickening of portal vein walls, luminal obstruction, and disappearance of portal veins); (2) Nodular regenerative hyperplasia; (3) Incomplete septal fibrosis (also called incomplete septal cirrhosis); 5. Histological features of non-specific changes (any of the following); (1) Portal tract abnormalities (increased arteries, dilated vessels, formation of perivascular collateral pathways, and abnormal vessels); (2) Structural disorganization: irregular distribution of portal tracts and central veins; (3) Non-zonal sinusoidal dilatation; (4) Mild perisinusoidal fibrosis.
Exclusion criteria
Exclusion criteria: 1. Budd-Chiari syndrome; 2. Congenital hepatic fibrosis; 3. Sarcoidosis; 4. Sinusoidal obstruction syndrome (SOS); 5. Sarcoma; 6. Schistosomiasis; 7. Primary biliary cholangitis (PBC); 8. Primary sclerosing cholangitis (PSC); 9. Participants enrolled in other clinical studies; 10. Patients deemed unsuitable for this study by the investigator.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Routine clinical test indicators;Incidence of liver decompensation (ascites, hepatic encephalopathy, bleeding) and improvement status;Liver-related mortality;Incidence of liver cancer;Rebleeding rate;Shunt success rate; | — |
Countries
China
Contacts
Huashan Hospital, Fudan University