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| Name | Class |
|---|---|
| European Society of Pediatric Gastroenterology, Hepatology and Nutrition | OTHER |
| European Association for the Study of the Liver (EASL) | UNKNOWN |
| Fondation Andrea Ferrari | UNKNOWN |
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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Neonate, Children, Adult with CPSS |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Shunt Closure | Procedure | Consist in spontaneous, surgical or interventional closure of the shunt. Depending on context, surgical intervention may also be transplantation. |
|
| Measure | Description | Time Frame |
|---|---|---|
| Numbers of patients with spontaneous shunt closure | 3; 6; 12; 24 months | |
| Number of patients developing one or more complications | 5; 10; 20 years | |
| Number of patients undergoing preemptive closure | 6; 12; 24 months |
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Inclusion Criteria:
Individual with CPSS
Exclusion Criteria:
Secondary shunt without evidence of congenital shunt
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neonates; children; adults
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| Name | Affiliation | Role |
|---|---|---|
| Valérie A Mc Lin, Prof. Dr. med. | University Hospital, Geneva | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Yale University School of Medicine | New Haven | Connecticut | 06520 | United States | ||
| Lurie Children's Hospital |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 28386718 | Background | 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. | |
| 23936100 | Background |
| Label | URL |
|---|---|
| International Registry of Congenital Portosystemic Shunts website, for patients and professionals | View source |
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undecided
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| Chicago |
| Illinois |
| 60611 |
| United States |
| Royal Children's Hospital Melbourne | Parkville | Victoria | 3052 | Australia |
| Universitary Clinics Saint-Luc, UCL | Brussels | 1200 | Belgium |
| The Hospital for Sick Children | Toronto | Ontario | M5G 1X8 | Canada |
| Beaujon University Hospital | Clichy | 92100 | France |
| Hôpital Bicêtre-Hôpitaux Universitaires Paris-Sud- Assistance Publique Hôpitaux de Paris. | Le Kremlin-Bicêtre | 94275 | France |
| Hôpital Necker | Paris | 75743 | France |
| Hannover Medical School | Hanover | 30625 | Germany |
| Haunersche Kinderklinik - LMU Munich | Munich | 80337 | Germany |
| University of Tuebingen | Tübingen | 72076 | Germany |
| First Department of Pediatrics, Semmelweis University | Budapest | 1083 | Hungary |
| Amrita institute of Medical Sciences | Kochi | Kerala | 682041 | India |
| Department of Pediatric Hepatology Institute of Liver and Biliary Sciences | New Delhi | 110070 | India |
| Schneider Children's Medical Center of Israel | Petach Tikvah | 49202 | Israel |
| Papa Giovanni XXIII Hospital | Bergamo | 24127 | Italy |
| Children's Hospital Santobono | Naples | 80129 | Italy |
| Istituto di Ricovero e Cura a Carattere Scientifico (ISMETT) | Palermo | 90127 | Italy |
| National Center for Child Health and Development | Tokyo | 157-8535 | Japan |
| Academic Medical Centre | Amsterdam | 1105 | Netherlands |
| University Medical Center Groningen | Groningen | 9700 RB | Netherlands |
| Hospital Clínic de Barcelona | Barcelona | 08036 | Spain |
| Hôpitaux Universitaires de Geneve (HUG) | Geneva | Canton of Geneva | 1205 | Switzerland |
| Gazi University, Faculty of Medicine | Yenimahalle | Ankara | 06560 | Turkey (Türkiye) |
| Birmingham Children's Hospital | Birmingham | B4 6NH | United Kingdom |
| Royal Hospital for Children and Young People | Edinburgh | EH16 4TJ | United Kingdom |
| 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. |
| 33276028 | Background | 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. |
| 33749116 | Background | 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. |
| 35854389 | Background | 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. |
| 33281106 | Background | 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. |
| 35104444 | Background | 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. |
| 30628988 | Background | 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. |
| 35204963 | Background | 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. |
| 32279979 | Background | 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. |
| 33605662 | Background | 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. |
| ID | Term |
|---|---|
| D000098526 | Congenital Portosystemic Shunt |
| D035583 | Rare Diseases |
| D054079 | Vascular Malformations |
| D006976 | Hypertension, Pulmonary |
| D020065 | Hepatopulmonary Syndrome |
| D008113 | Liver Neoplasms |
| D007003 | Hypoglycemia |
| D006946 | Hyperinsulinism |
| D007333 | Insulin Resistance |
| D017588 | Hyperandrogenism |
| D006501 | Hepatic Encephalopathy |
| D022124 | Hyperammonemia |
| ID | Term |
|---|---|
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D020969 | Disease Attributes |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
| D008107 | Liver Diseases |
| D004066 | Digestive System Diseases |
| D004067 | Digestive System Neoplasms |
| D009371 | Neoplasms by Site |
| D009369 | Neoplasms |
| D044882 | Glucose Metabolism Disorders |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D058489 | 46, XX Disorders of Sex Development |
| D012734 | Disorders of Sex Development |
| D014564 | Urogenital Abnormalities |
| D052776 | Female Urogenital Diseases |
| D005261 | Female Urogenital Diseases and Pregnancy Complications |
| D000091642 | Urogenital Diseases |
| D047808 | Adrenogenital Syndrome |
| D052801 | Male Urogenital Diseases |
| D006058 | Gonadal Disorders |
| D004700 | Endocrine System Diseases |
| D017093 | Liver Failure |
| D048550 | Hepatic Insufficiency |
| D001928 | Brain Diseases, Metabolic |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
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