International Registry of Congenital Portosystemic Shunt (IRCPSS)

Официальное название

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

Обзор

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.

Вмешательства

• Процедура Shunt Closure
  Consist in spontaneous, surgical or interventional closure of the shunt. Depending on context, surgical intervention may also be transplantation.

Первичные конечные точки

• Numbers of patients with spontaneous shunt closure [Срок оценки: 3; 6; 12; 24 months]
• Number of patients developing one or more complications [Срок оценки: 5; 10; 20 years]
• Number of patients undergoing preemptive closure [Срок оценки: 6; 12; 24 months]

Критерии участия

Критерии включения

Individual with CPSS

Критерии исключения

Secondary shunt without evidence of congenital shunt

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Нет

Дизайн исследования

Модель наблюдения

Когортное

Центры проведения

Франция · 3 центра

• Beaujon University Hospital — Clichy
• Hôpital Bicêtre-Hôpitaux Universitaires Paris-Sud- Assistance Publique Hôpitaux de Paris. — Le Kremlin-Bicêtre
• Hôpital Necker — Paris

Германия · 3 центра

• Hannover Medical School — Hanover
• Haunersche Kinderklinik - LMU Munich — Munich
• University of Tuebingen — Tübingen

Италия · 3 центра

• Papa Giovanni XXIII Hospital — Bergamo
• Children's Hospital Santobono — Naples
• Istituto di Ricovero e Cura a Carattere Scientifico (ISMETT) — Palermo

США · 2 центра

• Yale University School of Medicine — New Haven
• Lurie Children's Hospital — Chicago

Индия · 2 центра

• Amrita institute of Medical Sciences — Kochi
• Department of Pediatric Hepatology Institute of Liver and Biliary Sciences — New Delhi

Нидерланды · 2 центра

• Academic Medical Centre — Amsterdam
• University Medical Center Groningen — Groningen

Великобритания · 2 центра

• Birmingham Children's Hospital — Birmingham
• Royal Hospital for Children and Young People — Edinburgh

Австралия · 1 центр

• Royal Children's Hospital Melbourne — Parkville

Бельгия · 1 центр

• Universitary Clinics Saint-Luc, UCL — Brussels

Канада · 1 центр

• The Hospital for Sick Children — Toronto

Венгрия · 1 центр

• First Department of Pediatrics, Semmelweis University — Budapest

Израиль · 1 центр

• Schneider Children's Medical Center of Israel — Petach Tikvah

Япония · 1 центр

• National Center for Child Health and Development — Tokyo

Испания · 1 центр

• Hospital Clínic de Barcelona — Barcelona

Швейцария · 1 центр

• Hôpitaux Universitaires de Geneve (HUG) — Geneva

Turkey (Türkiye) · 1 центр

• Gazi University, Faculty of Medicine — Yenimahalle

Публикации

• 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. PMID 28386718
• 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. PMID 23936100
• 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. PMID 33276028
• 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. PMID 33749116
• 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 p PMID 35854389
• 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. PMID 33281106
• 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. PMID 35104444
• 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. PMID 30628988

Идентификаторы

NCT: NCT06041906 · 2018-00413

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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