International Registry of Congenital Portosystemic Shunt (IRCPSS) (IRCPSS)

June 13, 2025 updated by: Prof. Valérie Mc Lin

International Registry of Congenital Portosystemic Shunts (IRCPSS) - A Multi-centre, Retrospective and Prospective Registry of Neonates, Children and Adults With Congenital Portosystemic Shunts

Congenital Portosystemic Shunt (CPSS) is a rare condition important by the multiplicity and severity of associated complications.

CPSS is venous anomaly in which blood coming from the intestines only partially passes through the liver.

This leads to the accumulation of potentially toxic factors that cause systemic effects.

Complications vary among the individuals, and currently, it is challenging to predict which individuals will develop severe complications.

The IRCPSS registry is established with the aim of centralizing detailed clinical follow-up and biological information from participants around the world who suffer from Congenital Portosystemic Shunt (CPSS). A multidisciplinary consortium of experts is collaborating to enhance our understanding of the prevalence, natural history, individual risks, and physiopathology of the disease through the IRCPSS registry.

Study Overview

Status

Enrolling by invitation

Conditions

Intervention / Treatment

Study Type

Observational

Enrollment (Estimated)

500

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

  • Australia
    • Victoria
      • Parkville, Victoria, Australia, 3052
        • Royal Children's Hospital Melbourne
  • Belgium
      • Brussels, Belgium, 1200
        • Universitary Clinics Saint-Luc, UCL
  • Canada
    • Ontario
      • Toronto, Ontario, Canada, M5G 1X8
        • The Hospital for Sick Children
  • France
      • Clichy, France, 92100
        • Beaujon University Hospital
      • Le Kremlin-Bicêtre, France, 94275
        • Hôpital Bicêtre-Hôpitaux Universitaires Paris-Sud- Assistance Publique Hôpitaux de Paris.
      • Paris, France, 75743
        • Hôpital Necker
  • Germany
      • Hannover, Germany, 30625
        • Hannover Medical School
      • Munich, Germany, 80337
        • Haunersche Kinderklinik - LMU Munich
      • Tuebingen, Germany, 72076
        • University of Tuebingen
  • Hungary
      • Budapest, Hungary, 1083
        • First Department of Pediatrics, Semmelweis University
  • India
      • New Delhi, India, 110070
        • Department of Pediatric Hepatology Institute of Liver and Biliary Sciences
    • Kerala
      • Kochi, Kerala, India, 682041
        • Amrita Institute of Medical Sciences
  • Israel
      • Petach Tikvah, Israel, 49202
        • Schneider Children's Medical Center of Israel
  • Italy
      • Bergamo, Italy, 24127
        • Papa Giovanni XXIII Hospital
      • Naples, Italy, 80129
        • Children's Hospital Santobono
      • Palermo, Italy, 90127
        • Istituto di Ricovero e Cura a Carattere Scientifico (ISMETT)
  • Japan
      • Tokyo, Japan, 157-8535
        • National Center for Child Health and Development
  • Netherlands
      • Amsterdam, Netherlands, 1105
        • Academic Medical Centre
      • Groningen, Netherlands, 9700 RB
        • University Medical Center Groningen
  • Spain
      • Barcelona, Spain, 08036
        • Hospital Clinic de Barcelona
  • Switzerland
    • GE
      • Geneva, GE, Switzerland, 1205
        • Hopitaux Universitaires de Geneve (HUG)
  • Turkey
    • Ankara
      • Yenimahalle, Ankara, Turkey, 06560
        • Gazi University, Faculty of Medicine
  • United Kingdom
      • Birmingham, United Kingdom, B4 6NH
        • Birmingham Children's Hospital
      • Edinburgh, United Kingdom, EH16 4TJ
        • Royal Hospital for Children and Young People
  • United States
    • Connecticut
      • New Haven, Connecticut, United States, 06520
        • Yale University School of Medicine
    • Illinois
      • Chicago, Illinois, United States, 60611
        • Lurie Children's Hospital

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

  • Child
  • Adult
  • Older Adult

Accepts Healthy Volunteers

No

Sampling Method

Non-Probability Sample

Study Population

neonates; children; adults

Description

Inclusion Criteria:

Individual with CPSS

Exclusion Criteria:

Secondary shunt without evidence of congenital shunt

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

Cohorts and Interventions

Group / Cohort
Intervention / Treatment
Neonate, Children, Adult with CPSS
Procedure: Shunt Closure
Consist in spontaneous, surgical or interventional closure of the shunt. Depending on context, surgical intervention may also be transplantation.

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Time Frame
Numbers of patients with spontaneous shunt closure
Time Frame: 3; 6; 12; 24 months
3; 6; 12; 24 months
Number of patients developing one or more complications
Time Frame: 5; 10; 20 years
5; 10; 20 years
Number of patients undergoing preemptive closure
Time Frame: 6; 12; 24 months
6; 12; 24 months

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Prof. Valérie Mc Lin

Collaborators

European Society of Pediatric Gastroenterology, Hepatology and Nutrition
European Association for the Study of the Liver (EASL)
Fondation Andrea Ferrari

Investigators

  • Study Director: Valérie A Mc Lin, Prof. Dr. med., University Hospital, Geneva

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Helpful Links

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

April 26, 2018

Primary Completion (Estimated)

October 26, 2026

Study Completion (Estimated)

December 31, 2028

Study Registration Dates

First Submitted

September 11, 2023

First Submitted That Met QC Criteria

September 11, 2023

First Posted (Actual)

September 18, 2023

Study Record Updates

Last Update Posted (Actual)

June 18, 2025

Last Update Submitted That Met QC Criteria

June 13, 2025

Last Verified

December 1, 2024

More Information

Terms related to this study

Keywords

Other Study ID Numbers

  • 2018-00413

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

UNDECIDED

IPD Plan Description

undecided

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

product manufactured in and exported from the U.S.

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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