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

N/ANot Yet RecruitingNCT07744048

Beijing Municipal Administration of Hospitals150 enrolled

Overview

This retrospective-prospective cohort study investigates portal sinusoidal vascular disorder (PSVD), a liver vascular disease definitively diagnosed via liver biopsy. Addressing PSVD's poorly defined natural history, unclear prognostic determinants and the scarcity of large-scale systematic research in China, the study enrolls histopathologically confirmed patients, collects baseline clinicopathological, laboratory and imaging data, tracks endpoints including transplant-free survival, liver-related events and portal vein thrombosis changes, and conducts prognostic analyses using Cox regression, Kaplan-Meier method and Fine-Gray model, aiming to characterize disease features, identify key prognostic factors, verify high-risk subgroups with inferior outcomes, and support precise clinical management while filling relevant domestic research gaps.

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.

Study Type

OBSERVATIONAL

Enrollment

150

Conditions

Porto-Sinusoidal Vascular Diseases

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

Inclusion Criteria: 1. Patients histopathologically diagnosed with porto-sinusoidal vascular disorder (PSVD) via liver needle biopsy; 2. Liver biopsy specimen length ≥ 10 mm (or assessed as diagnostically adequate by a pathologist), with no histological evidence of cirrhosis; 3. Meeting the established diagnostic criteria for PSVD. Exclusion Criteria: 1. Concomitant Budd-Chiari syndrome or other hepatic venous outflow obstruction diseases; 2. Concomitant congenital hepatic fibrosis, hepatic sarcoidosis, or sinusoidal obstruction syndrome; 3. Concomitant hepatocellular carcinoma or other hepatic malignancies; 4. Age \< 18 years; 5. Severe deficiency of clinical data that precludes valid analysis.

Outcomes

Primary Outcomes

Transplant-free survival

Time from study enrollment to all-cause death or liver transplantation, whichever occurs first.

Time frame: From baseline through Month 36, with outcome status assessed at Months 6, 12, 18, 24, 30, and 36, or until all-cause death or liver transplantation, whichever occurs first.

Secondary Outcomes

Liver-related composite events

Occurrence of variceal bleeding, refractory ascites, hepatic encephalopathy, PVT progression or hepatic decompensation.