Study on Clinicopathological Features, Influencing Factors and Clinical Outcomes in Patients With PSVD

The study aims to include approximately 100-150 patients with histopathologically confirmed PSVD by liver biopsy, covering subgroups with/without definite etiology, with/without PVT, with/without portal hypertension, etc. Collect demographic, clinical, laboratory, imaging and pathological data of enrolled patients. Follow up endpoints including transplant-free survival, liver-related events and dynamic changes of PVT. Apply Cox regression, Kaplan-Meier method and Fine-Gray model to identify independent prognostic factors and compare prognosis differences among subgroups, so as to provide reference for clinical diagnosis and treatment of PSVD.

Inclusion criteria

• Patients histopathologically diagnosed with porto-sinusoidal vascular disorder (PSVD) via liver needle biopsy;
• Liver biopsy specimen length ≥ 10 mm (or assessed as diagnostically adequate by a pathologist), with no histological evidence of cirrhosis;
• Meeting the established diagnostic criteria for PSVD.

Exclusion criteria

• Concomitant Budd-Chiari syndrome or other hepatic venous outflow obstruction diseases;
• Concomitant congenital hepatic fibrosis, hepatic sarcoidosis, or sinusoidal obstruction syndrome;
• Concomitant hepatocellular carcinoma or other hepatic malignancies;
• Age < 18 years;
• Severe deficiency of clinical data that precludes valid analysis.