- ICH GCP
- US Clinical Trials Registry
- Clinical Trial NCT06041906
International Registry of Congenital Portosystemic Shunt (IRCPSS) (IRCPSS)
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
Conditions
Intervention / Treatment
Study Type
Enrollment (Estimated)
Contacts and Locations
Study Locations
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Victoria
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Parkville, Victoria, Australia, 3052
- Royal Children's Hospital Melbourne
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Brussels, Belgium, 1200
- Universitary Clinics Saint-Luc, UCL
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Ontario
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Toronto, Ontario, Canada, M5G 1X8
- The Hospital for Sick Children
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Clichy, France, 92100
- Beaujon University Hospital
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Le Kremlin-Bicêtre, France, 94275
- Hôpital Bicêtre-Hôpitaux Universitaires Paris-Sud- Assistance Publique Hôpitaux de Paris.
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Paris, France, 75743
- Hopital Necker
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Munich, Germany, 80337
- Haunersche Kinderklinik - LMU Munich
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Tuebingen, Germany, 72076
- University of Tuebingen
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Athens, Greece, 11527
- "Agia Sofia" Children's Hospital
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Chaidari, Greece, 12462
- " ATTIKON " University Hospital
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Budapest, Hungary, 1083
- First Department of Pediatrics, Semmelweis University
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New Delhi, India, 110070
- Department of Pediatric Hepatology Institute of Liver and Biliary Sciences
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Petach Tikvah, Israel, 49202
- Schneider Children's Medical Center of Israel
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Naples, Italy, 80129
- Children's Hospital Santobono
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Palermo, Italy, 90127
- Istituto di Ricovero e Cura a Carattere Scientifico (ISMETT)
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Tokyo, Japan, 157-8535
- National Center for Child Health and Development
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Amsterdam, Netherlands, 1105
- Academic Medical Centre
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Groningen, Netherlands, 9700 RB
- University Medical Center Groningen
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Barcelona, Spain, 08036
- Hospital Clínic de Barcelona
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GE
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Geneva, GE, Switzerland, 1205
- Hôpitaux universitaires de Genève (HUG)
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Istanbul, Turkey, 34010
- Koc University School of Medicine
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Ankara
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Yenimahalle, Ankara, Turkey, 06560
- Gazi University, Faculty of Medicine
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Birmingham, United Kingdom, B4 6NH
- Birmingham Children's Hospital
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Edinburgh, United Kingdom, EH16 4TJ
- Royal Hospital for Children and Young People
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Leeds, United Kingdom, LS1 3EX
- Leeds General Infirmary
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London, United Kingdom, NW3 2QG
- Royal Free London NHS Foundation Trust
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London, United Kingdom, SE5 9RS
- Kings College Hospital
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Connecticut
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New Haven, Connecticut, United States, 06520
- Yale University School of Medicine
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Participation Criteria
Eligibility Criteria
Ages Eligible for Study
- Child
- Adult
- Older Adult
Accepts Healthy Volunteers
Sampling Method
Study Population
Description
Inclusion Criteria:
Individual with CPSS
Exclusion Criteria:
Secondary shunt without evidence of congenital shunt
Study Plan
How is the study designed?
Design Details
Cohorts and Interventions
Group / Cohort |
Intervention / Treatment |
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Neonate, Children, Adult with CPSS
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Consist in spontaneous, surgical or interventional closure of the shunt.
Depending on context, surgical intervention may also be transplantation.
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What is the study measuring?
Primary Outcome Measures
Outcome Measure |
Time Frame |
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Numbers of patients with spontaneous shunt closure
Time Frame: 3; 6; 12; 24 months
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3; 6; 12; 24 months
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Number of patients developing one or more complications
Time Frame: 5; 10; 20 years
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5; 10; 20 years
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Number of patients undergoing preemptive closure
Time Frame: 6; 12; 24 months
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6; 12; 24 months
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Collaborators and Investigators
Sponsor
Collaborators
Investigators
- Study Director: Valérie A Mc Lin, Prof. Dr. med., University Hospital, Geneva
Publications and helpful links
General Publications
- 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.
Study record dates
Study Major Dates
Study Start (Actual)
Primary Completion (Estimated)
Study Completion (Estimated)
Study Registration Dates
First Submitted
First Submitted That Met QC Criteria
First Posted (Actual)
Study Record Updates
Last Update Posted (Actual)
Last Update Submitted That Met QC Criteria
Last Verified
More Information
Terms related to this study
Keywords
- Insulin resistance
- hypoglycemia
- rare disease
- liver
- Liver Neoplasms
- registry
- hyperandrogenism
- shunt
- Hepatic Encephalopathy
- Hepatopulmonary Syndrome
- Hypertension, Pulmonary
- hyperinsulinism
- vascular malformation
- Hyperammonemia
- portosystemic
- portasystemic
- porto-systemic
- porta-systemic
- Abernethy malformation
- hyperinsulinemic
Other Study ID Numbers
- 2018-00413
Plan for Individual participant data (IPD)
Plan to Share Individual Participant Data (IPD)?
IPD Plan Description
Drug and device information, study documents
Studies a U.S. FDA-regulated drug product
Studies a U.S. FDA-regulated device product
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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