Porto-Sinusoidal Vascular Diseases
Conditions
Keywords
Porto-sinusoidal vascular disorder, PSVD, INCPH
Brief summary
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.
Detailed description
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.
Interventions
None listed
Sponsors
Beijing Municipal Administration of Hospitals
Study design
Observational model
COHORT
Time perspective
OTHER
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Transplant-free survival | 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. | Time from study enrollment to all-cause death or liver transplantation, whichever occurs first. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Liver-related composite events | 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. | Occurrence of variceal bleeding, refractory ascites, hepatic encephalopathy, PVT progression or hepatic decompensation. |
Countries
China
Contacts
CONTACTMinghui Li, Dr
CONTACTWeihua Cao, Dr
Outcome results
None listed