International Registry of Congenital Portosystemic Shunt (IRCPSS)
NCT ID: NCT06041906
Last Updated: 2025-06-18
Study Results
The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.
Basic Information
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ENROLLING_BY_INVITATION
500 participants
OBSERVATIONAL
2018-04-26
2028-12-31
Brief Summary
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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.
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Detailed Description
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Conditions
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Study Design
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COHORT
OTHER
Study Groups
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Neonate, Children, Adult with CPSS
Shunt Closure
Consist in spontaneous, surgical or interventional closure of the shunt. Depending on context, surgical intervention may also be transplantation.
Interventions
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Shunt Closure
Consist in spontaneous, surgical or interventional closure of the shunt. Depending on context, surgical intervention may also be transplantation.
Eligibility Criteria
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Inclusion Criteria
Exclusion Criteria
1 Day
ALL
No
Sponsors
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European Society of Pediatric Gastroenterology, Hepatology and Nutrition
OTHER
European Association for the Study of the Liver (EASL)
UNKNOWN
Fondation Andrea Ferrari
UNKNOWN
Prof. Valérie Mc Lin
OTHER
Responsible Party
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Prof. Valérie Mc Lin
Prof. Dr. med.
Principal Investigators
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Valérie A Mc Lin, Prof. Dr. med.
Role: STUDY_DIRECTOR
University Hospital, Geneva
Locations
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Yale University School of Medicine
New Haven, Connecticut, United States
Lurie Children's Hospital
Chicago, Illinois, United States
Royal Children's Hospital Melbourne
Parkville, Victoria, Australia
Universitary Clinics Saint-Luc, UCL
Brussels, , Belgium
The Hospital for Sick Children
Toronto, Ontario, Canada
Beaujon University Hospital
Clichy, , France
Hôpital Bicêtre-Hôpitaux Universitaires Paris-Sud- Assistance Publique Hôpitaux de Paris.
Le Kremlin-Bicêtre, , France
Hôpital Necker
Paris, , France
Hannover Medical School
Hanover, , Germany
Haunersche Kinderklinik - LMU Munich
Munich, , Germany
University of Tuebingen
Tübingen, , Germany
First Department of Pediatrics, Semmelweis University
Budapest, , Hungary
Amrita institute of Medical Sciences
Kochi, Kerala, India
Department of Pediatric Hepatology Institute of Liver and Biliary Sciences
New Delhi, , India
Schneider Children's Medical Center of Israel
Petach Tikvah, , Israel
Papa Giovanni XXIII Hospital
Bergamo, , Italy
Children's Hospital Santobono
Naples, , Italy
Istituto di Ricovero e Cura a Carattere Scientifico (ISMETT)
Palermo, , Italy
National Center for Child Health and Development
Tokyo, , Japan
Academic Medical Centre
Amsterdam, , Netherlands
University Medical Center Groningen
Groningen, , Netherlands
Hospital Clínic de Barcelona
Barcelona, , Spain
Hôpitaux Universitaires de Geneve (HUG)
Geneva, Canton of Geneva, Switzerland
Gazi University, Faculty of Medicine
Yenimahalle, Ankara, Turkey (Türkiye)
Birmingham Children's Hospital
Birmingham, , United Kingdom
Royal Hospital for Children and Young People
Edinburgh, , United Kingdom
Countries
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References
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Hanquinet S, Morice C, Courvoisier DS, Cousin V, Anooshiravani M, Merlini L, McLin VA. Globus pallidus MR signal abnormalities in children with chronic liver disease and/or porto-systemic shunting. Eur Radiol. 2017 Oct;27(10):4064-4071. doi: 10.1007/s00330-017-4808-x. Epub 2017 Apr 6.
Cudalbu C, McLin VA, Lei H, Duarte JM, Rougemont AL, Oldani G, Terraz S, Toso C, Gruetter R. The C57BL/6J mouse exhibits sporadic congenital portosystemic shunts. PLoS One. 2013 Jul 23;8(7):e69782. doi: 10.1371/journal.pone.0069782. Print 2013.
Joye R, Lador F, Aggoun Y, Farhat N, Wacker J, Wildhaber BE, Vallee JP, Hachulla AL, McLin VA, Beghetti M. Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients. J Hepatol. 2021 Mar;74(3):742-747. doi: 10.1016/j.jhep.2020.11.039. Epub 2020 Dec 1.
McLin V, Beghetti M, D'Antiga L, Franchi-Abella S; International Registry of Congenital Porto-Systemic Shunts (IRCPSS). Current Quandaries in the Management of Congenital Portosystemic Shunts. Liver Transpl. 2021 Aug;27(8):1212-1213. doi: 10.1002/lt.26055. Epub 2021 Jul 20. No abstract available.
Korff S, Mostaguir K, Beghetti M, D'Antiga L, Debray D, Franchi-Abella S, Gonzales E, Guerin F, Hachulla AL, Lambert V, Makrythanasis P, Roduit N, Savale L, Senat MV, Spaltenstein J, van Steenbeek F, Wildhaber BE, Zwahlen M, McLin VA. International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts. Orphanet J Rare Dis. 2022 Jul 19;17(1):284. doi: 10.1186/s13023-022-02412-8.
Lambert V, Ladarre D, Fortas F, Durand P, Herve P, Gonzales E, Guerin F, Savale L, McLin VA, Ackermann O, Franchi-Abella S; International Registry of Congenital Portosystemic Shunts (IRCPSS). Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre. Arch Cardiovasc Dis. 2021 Mar;114(3):221-231. doi: 10.1016/j.acvd.2020.10.003. Epub 2020 Dec 3.
McLin VA, D'Antiga L. The current pediatric perspective on type B and C hepatic encephalopathy. Anal Biochem. 2022 Apr 15;643:114576. doi: 10.1016/j.ab.2022.114576. Epub 2022 Jan 29.
McLin VA, Franchi Abella S, Debray D, Guerin F, Beghetti M, Savale L, Wildhaber BE, Gonzales E; Members of the International Registry of Congenital Porto-Systemic Shunts. Congenital Portosystemic Shunts: Current Diagnosis and Management. J Pediatr Gastroenterol Nutr. 2019 May;68(5):615-622. doi: 10.1097/MPG.0000000000002263.
Bahadori A, Kuhlmann B, Debray D, Franchi-Abella S, Wacker J, Beghetti M, Wildhaber BE, McLin VA, On Behalf Of The Ircpss. Presentation of Congenital Portosystemic Shunts in Children. Children (Basel). 2022 Feb 11;9(2):243. doi: 10.3390/children9020243.
Guerin F, Franchi Abella S, McLin V, Ackermann O, Girard M, Cervoni JP, Savale L, Hernandez-Gea V, Valla D, Hillaire S, Dutheil D, Bureau C, Gonzales E, Plessier A. Congenital portosystemic shunts: Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver. Clin Res Hepatol Gastroenterol. 2020 Sep;44(4):452-459. doi: 10.1016/j.clinre.2020.03.004. Epub 2020 Apr 9. No abstract available.
Rock NM, Beghetti M, Tissot C, Willi JP, Bouhabib M, McLin VA, Maggio ABR. Reliable Detection of Intrapulmonary Shunts Using Contrast-Enhanced Echocardiography in Children With Portal Hypertension or Portosystemic Shunt. J Pediatr Gastroenterol Nutr. 2021 Jul 1;73(1):73-79. doi: 10.1097/MPG.0000000000003079.
Related Links
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International Registry of Congenital Portosystemic Shunts website, for patients and professionals
Other Identifiers
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2018-00413
Identifier Type: -
Identifier Source: org_study_id
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