Clinical Diagnosis and Pathological Spectrum of Porto-sinusoidal Vascular Disease in India

NCT ID: NCT06054451

Last Updated: 2024-04-25

Basic Information

• Recruitment Status: RECRUITING
• Total Enrollment: 210 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2023-08-01
• Study Completion Date: 2024-06-30

Brief Summary

There is a need to re-evaluate the patients classified as NCPH and determine whether the new histological classification proposed by the VALDIG applies to the Indian scenario. We intend to identify the patient cohorts who have been diagnosed as NCPH, NCPF, EHPVO, hepatic venous outlet tract obstruction (HVOTO), Veno-occlusive disease (VOD) and sinusoidal obstruction syndrome (SOS) based on their liver biopsy, endoscopy, HVPG, and radiology reports. These patients will be screened to find the patients who fit the diagnosis of PSVD. It is important to establish whether the new definition of PSVD is relevant to the Indian population and establish the usefulness of invasive tests like liver biopsy in diagnosing the disease. The patient cohorts meeting diagnosis of INCPH will be compared with those meeting the new diagnosis of PSVD. The investigators will describe the clinical (demographic, clinical risk factors, socioeconomic status), etiological (associated conditions, coagulation disorders medication use, genetic risk factors), imaging (based on ultrasound Doppler imaging or cross- sectional imaging), endoscopic, fibrosis tests (using non-invasive tests), and the histopathology of the patients who fulfil the criteria of PSVD.

Detailed Description

Portal hypertension is clinically characterized by a portal venous pressure gradient between the inferior vena cava and the portal vein is more than 5 mm. Liver cirrhosis remains the leading cause of portal hypertension in the western world. However, there are conditions where portal hypertension can even occur without liver cirrhosis, referred to as Noncirrhotic portal hypertension (NCPH). The term NCPH has been derived from a variety of histopathological entities such as hepatoportal sclerosis, noncirrhotic portal fibrosis, nodular regenerative hyperplasia, or incomplete septal fibrosis. In the absence of other causes of cirrhosis and portal venous thrombosis, the condition is termed idiopathic noncirrhotic portal hypertension. The histopathological findings of INCPH are not entirely specific to portal hypertension, similar changes can be seen in patients without portal hypertension. Therefore the term Porto-sinusoidal vascular disorder(PSVD) has been recently introduced to define a group of liver vessel disorders affecting the portal venules and sinusoids irrespective of the absence or presence of liver cirrhosis. This disease entity can have a varied clinical presentation and histological findings and has been associated with several immunological and systemic disorders.

Porto-sinusoidal vascular disease - Currently, for clinical practice, the VALDIG group has given a working definition for PSVD.

"Liver biopsy ≥20mm without cirrhosis with either 1 specific sign of portal hypertension OR 1 specific histological finding for PSVD" OR "Liver biopsy ≥20mm without cirrhosis with 1 sign not specific for portal hypertension AND 1 sign not specific for PSVD"

Conditions

Portal Hypertension; Budd-Chiari Syndrome; Non-Cirrhotic Portal Hypertension; Porto-Sinusoidal Vascular Diseases; Sinusoidal Obstruction Syndrome; Veno Occlusive Disease, Hepatic; Non-Cirrhotic Portal Fibrosis

Study Design

• Observational Model Type: CASE_ONLY
• Study Time Perspective: PROSPECTIVE

Eligibility Criteria

Inclusion Criteria

• Patients of 12-70 years, either gender with clinical, pathological radiological diagnosis of NCPH

Exclusion Criteria

• Any patients having Cirrhosis based on clinical, pathological, or radiological diagnosis
• Any patients having active malignancy

• Minimum Eligible Age: 12 Years
• Maximum Eligible Age: 70 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Post Graduate Institute of Medical Education and Research, Chandigarh

OTHER

Sponsor Role: lead

Responsible Party

Madhumita Premkumar

ASSOCIATE PROFESSOR

Responsibility Role: PRINCIPAL_INVESTIGATOR

Principal Investigators

Dr Madhumita Premkumar, DM

Role: PRINCIPAL_INVESTIGATOR

Post Graduate Institute of Medical Education and Research, Chandigarh

Locations

Dr. Madhumita Premkumar

Sector-12, Chandigarh, India

Site Status: RECRUITING

Countries

India

Central Contacts

Dr Madhumita Premkumar, DM

Role: CONTACT

drmadhumitap@gmail.com

7087003409

Facility Contacts

Dr. Madhumita Premkumar, MD

Role: primary

drmadhumitap@gmail.com

Other Identifiers

IEC-INT/2023/MD-1081

Identifier Type: -

Identifier Source: org_study_id