{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:20:58Z","timestamp":1777594858766,"version":"3.51.4"},"reference-count":34,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2025,7,17]],"date-time":"2025-07-17T00:00:00Z","timestamp":1752710400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background\/Objectives: Portal hypertension (PH) is a common complication in children with chronic liver diseases. Primary and secondary prophylaxis of variceal bleeding in these patients remains controversial. Our study aims to evaluate the management of gastrointestinal (GI) varices in children with PH in Italy. Methods: A questionnaire was sent to 21 major pediatric hepatology centers. It included 34 questions referring to the medical, endoscopic, radiological, and surgical management of GI varices. Results: Out of 21 centers, 16 returned a completed questionnaire (survey response rate 76%) with a high level of completeness. A total of 1206 children with PH were under follow-up. Splenomegaly associated with hypersplenism was the main indication for endoscopic surveillance in all centers (100%). Primary prophylaxis was performed with endoscopy plus non-selective beta-blockers (NSBBs) in 50%, endoscopy alone in 38%, and NSBBs alone in 12%. All centers managed acute variceal bleeding with endoscopy within 24 h, acid suppression, and octreotide infusion. Secondary prophylaxis of variceal bleeding was conducted using endoscopy (100%) and NSBBs (87%). Transjugular intrahepatic portosystemic shunt (TIPS) was considered a good option when endoscopic treatment failed in 94% of centers. Conclusions: In Italy, there is broad consensus among centers regarding the management of gastrointestinal varices in children with portal hypertension. All participating centers endorsed the use of endoscopic screening for children presenting with clinical signs of portal hypertension. Nonetheless, further research is essential to establish evidence-based guidelines and to improve overall quality of care.<\/jats:p>","DOI":"10.3390\/children12070940","type":"journal-article","created":{"date-parts":[[2025,7,17]],"date-time":"2025-07-17T13:00:51Z","timestamp":1752757251000},"page":"940","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Primary and Secondary Prophylaxis of Gastrointestinal Bleeding in Children with Portal Hypertension: A Multicenter National Study by SIGENP"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8799-7266","authenticated-orcid":false,"given":"Naire","family":"Sansotta","sequence":"first","affiliation":[{"name":"Paediatric Hepatology Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, 24127 Bergamo, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"De Angelis","sequence":"additional","affiliation":[{"name":"Gastroenterology and Nutrition Unit, Bambino Ges\u00f9 Children\u2019s Hospital IRCCS, 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniele","family":"Alberti","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, ASST Spedali Civili, 25100 Brescia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9915-8972","authenticated-orcid":false,"given":"Fabiola","family":"Di Dato","sequence":"additional","affiliation":[{"name":"Department of Translational Medical Science, University of Naples Federico II, 80121 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Serena","family":"Arrigo","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Endoscopic Unit, IRCCS Istituto Giannina Gaslini, 16121 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5249-8248","authenticated-orcid":false,"given":"Matteo","family":"Bramuzzo","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health IRCCS \u201cBurlo Garofolo\u201d, 34121 Trieste, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Benedetta","family":"Calcaterra","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Milano\u2014Bicocca, 20100 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mara","family":"Cananzi","sequence":"additional","affiliation":[{"name":"University Hospital of Padova, 35121 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maurizio","family":"Cheli","sequence":"additional","affiliation":[{"name":"Paediatric Surgery, Hospital Papa Giovanni XXIII Bergamo, 24127 Bergamo, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Chiaro","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Endoscopic Unit, IRCCS Istituto Giannina Gaslini, 16121 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Cirillo","sequence":"additional","affiliation":[{"name":"Santobono-Pausilipon Children\u2019s Hospital, 80121 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mara","family":"Colusso","sequence":"additional","affiliation":[{"name":"Paediatric Surgery, Hospital Papa Giovanni XXIII Bergamo, 24127 Bergamo, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6313-4482","authenticated-orcid":false,"given":"Grazia","family":"Di Leo","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health IRCCS \u201cBurlo Garofolo\u201d, 34121 Trieste, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simona","family":"Faraci","sequence":"additional","affiliation":[{"name":"Gastroenterology and Nutrition, Bambino Ges\u00f9 Children\u2019s Hospital IRCCS, 00163 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"Gaio","sequence":"additional","affiliation":[{"name":"University Hospital of Padova, 35121 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppe","family":"Indolfi","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital IRCCS, 50139 Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Silvia","family":"Iuliano","sequence":"additional","affiliation":[{"name":"Gastroenterology and Endoscopy Unit, University Hospital of Parma, 43126 Parma, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0311-5204","authenticated-orcid":false,"given":"Daniela","family":"Liccardo","sequence":"additional","affiliation":[{"name":"Unit of Hepatology and Liver Transplant, Division of Metabolic Diseases and Hepatology, Bambino Ges\u00f9 Children\u2019s Hospital IRCCS, 00163 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4349-3728","authenticated-orcid":false,"given":"Antonio","family":"Marseglia","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Casa Sollievo della Sofferenza, Division of Pediatrics, 71013 San Giovanni Rotondo, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matteo","family":"Motta","sequence":"additional","affiliation":[{"name":"University Hospital Parma, 43126 Parma, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"Nuti","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, 20129 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1486-0234","authenticated-orcid":false,"given":"Filippo","family":"Parolini","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, ASST Spedali Civili, 25100 Brescia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-6094-7983","authenticated-orcid":false,"given":"Sara","family":"Renzo","sequence":"additional","affiliation":[{"name":"Gastroenterology and Nutrition Unit, Meyer Children\u2019s Hospital IRCCS, 50139 Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4606-9145","authenticated-orcid":false,"given":"Francesca","family":"Sbravati","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Unit, Maggiore Hospital, 40133 Bologna, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4092-9071","authenticated-orcid":false,"given":"Marco","family":"Sciveres","sequence":"additional","affiliation":[{"name":"ISMETT, University of Pittsburgh Medical Center Italy, 90133 Palermo, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2425-5449","authenticated-orcid":false,"given":"Claudia","family":"Mandato","sequence":"additional","affiliation":[{"name":"Department of Medicine, Surgery and Dentistry \u201cScuola Medica Salernitana\u201d, Pediatrics Section, University of Salerno, 84131 Baronissi, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angelo","family":"Di Giorgio","sequence":"additional","affiliation":[{"name":"Department of Medicine, Pediatrics Section, University of Udine, 33100 Urdine, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1097\/TP.0000000000002367","article-title":"Transplant-free Survival in Chronic Liver Disease Presenting as Acute Liver Failure in Childhood","volume":"103","author":"Nicastro","year":"2019","journal-title":"Transplantation"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1179","DOI":"10.1016\/j.dld.2019.02.014","article-title":"Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: A multicentre national study","volume":"51","author":"Cheli","year":"2019","journal-title":"Dig. Liver Dis."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1097\/MPG.0b013e3182a1cd7f","article-title":"Management of portal hypertension in children with portal vein thrombosis","volume":"57","author":"Giouleme","year":"2013","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1136\/archdischild-2015-310022","article-title":"Portal hypertension and its management in children","volume":"103","author":"Grammatikopoulos","year":"2018","journal-title":"Arch. Dis. Child."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1097\/MPG.0000000000002643","article-title":"Long-term Outcomes and Health Perceptions in Pediatric-onset Portal Hypertension Complicated by Varices","volume":"70","author":"Luoto","year":"2020","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1136\/archdischild-2020-319600","article-title":"Evolving management of paediatric portal hypertension","volume":"106","author":"Luoto","year":"2021","journal-title":"Arch. Dis. Child."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1097\/MPG.0000000000000822","article-title":"Interobserver Agreement on Endoscopic Classification of Oesophageal Varices in Children","volume":"61","author":"Betalli","year":"2015","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1368","DOI":"10.1002\/hep.28153","article-title":"Primary prophylaxis of variceal bleeding in children and the role of MesoRex Bypass: Summary of the Baveno VI Pediatric Satellite Symposium","volume":"63","author":"Shneider","year":"2016","journal-title":"Hepatology"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1016\/j.jhep.2016.09.006","article-title":"Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding","volume":"66","author":"Duche","year":"2017","journal-title":"J. Hepatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1016\/j.giec.2022.11.003","article-title":"Gastrointestinal Bleeding in Children: Current Management, Controversies, and Advances","volume":"33","author":"Novak","year":"2023","journal-title":"Gastrointest. Endosc. Clin."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"666","DOI":"10.1053\/jhep.2002.35278","article-title":"Endoscopic ligation compared with sclerotherapy for bleeding esophageal varices in children with extrahepatic portal venous obstruction","volume":"36","author":"Zargar","year":"2002","journal-title":"Hepatology"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/MPG.0000000000001882","article-title":"Non-invasive Markers of Portal Hypertension: Appraisal of Adult Experience and Potential Utilisation in Children","volume":"66","author":"Sutton","year":"2018","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_13","first-page":"341","article-title":"Clinical and Laboratory Predictors of Esophageal Varices in Children with Chronic Liver Disease","volume":"26","author":"Hasan","year":"2017","journal-title":"Mymensingh Med. J."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1610","DOI":"10.1111\/jgh.16267","article-title":"How to suspect the presence of high-risk esophageal varices and when to start endoscopic surveillance in children with biliary atresia?","volume":"38","author":"Poddar","year":"2023","journal-title":"J. Gastroenterol. Hepatol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1007\/s12098-023-04751-1","article-title":"An Approach to Investigations of Chronic Liver Disease","volume":"91","author":"Ravindranath","year":"2024","journal-title":"Indian. J. Pediatr."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"392","DOI":"10.4254\/wjh.v7.i3.392","article-title":"Cirrhosis in children and adolescents: An overview","volume":"7","author":"Pinto","year":"2015","journal-title":"World J. Hepatol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1016\/j.jhep.2015.05.022","article-title":"Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension","volume":"63","author":"Baveno","year":"2015","journal-title":"J. Hepatol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1111\/j.1399-3046.2012.01652.x","article-title":"Portal hypertension in children: Expert pediatric opinion on the report of the Baveno v Consensus Workshop on Methodology of Diagnosis and Therapy in Portal Hypertension","volume":"16","author":"Shneider","year":"2012","journal-title":"Pediatr. Transplant."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1016\/j.jhep.2021.12.022","article-title":"Baveno VII\u2014Renewing consensus in portal hypertension","volume":"76","author":"Bosch","year":"2022","journal-title":"J. Hepatol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1200","DOI":"10.4103\/1735-1995.172990","article-title":"Gastric varices: Classification, endoscopic and ultrasonographic management","volume":"20","author":"Wani","year":"2015","journal-title":"J. Res. Med. Sci."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"2009","DOI":"10.1053\/j.gastro.2011.08.049","article-title":"A clinical prediction rule and platelet count predict esophageal varices in children","volume":"141","author":"Gana","year":"2011","journal-title":"Gastroenterology"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1016\/j.dld.2022.10.009","article-title":"Non-invasive tools for compensated advanced chronic liver disease and portal hypertension after Baveno VII\u2014An update","volume":"55","author":"Segna","year":"2023","journal-title":"Dig. Liver Dis."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/j.transproceed.2018.04.074","article-title":"Primary Prophylaxis for Gastrointestinal Bleeding in Children With Biliary Atresia and Portal Hypertension Candidates for Liver Transplantation: A Single-Center Experience","volume":"51","author":"Angelico","year":"2019","journal-title":"Transplant. Proc."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1097\/MPG.0b013e318205993a","article-title":"Primary prophylaxis of variceal hemorrhage in children with portal hypertension: A framework for future research","volume":"52","author":"Ling","year":"2011","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1097\/MPG.0000000000001453","article-title":"Survey on Clinical Practice of Primary Prophylaxis in Portal Hypertension in Children","volume":"64","author":"Duche","year":"2017","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1097\/MPG.0b013e318213be81","article-title":"Variation in care for children with esophageal varices: A study of physicians\u2019, patients\u2019, and families\u2019 approaches and attitudes","volume":"52","author":"Gana","year":"2011","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1007\/s11894-015-0450-4","article-title":"Screening and Prophylaxis for Varices in Children with Liver Disease","volume":"17","author":"Bozic","year":"2015","journal-title":"Curr. Gastroenterol. Rep."},{"key":"ref_28","first-page":"CD011561","article-title":"Band ligation versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis","volume":"1","author":"Cifuentes","year":"2021","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_29","first-page":"299","article-title":"Effectiveness of beta blockers in primary prophylaxis of variceal bleeding in children with portal hypertension","volume":"32","author":"Samanta","year":"2011","journal-title":"Trop. Gastroenterol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"e010902","DOI":"10.1136\/bmjopen-2015-010902","article-title":"Carvedilol for portal hypertension in cirrhosis: Systematic review with meta-analysis","volume":"6","author":"Li","year":"2016","journal-title":"BMJ Open"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"510","DOI":"10.1007\/s12072-019-09948-1","article-title":"Biliary features in liver histology of children with autoimmune liver disease","volume":"13","author":"Marseglia","year":"2019","journal-title":"Hepatol. Int."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1203","DOI":"10.1007\/s00270-023-03520-z","article-title":"Outcome of Children with Transjugular Intrahepatic Portosystemic Shunt: A Meta-Analysis of Individual Patient Data","volume":"46","author":"Deniz","year":"2023","journal-title":"Cardiovasc. Intervent Radiol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"18","DOI":"10.25259\/JCIS_36_2023","article-title":"Transjugular intrahepatic portosystemic shunt for pediatric portal hypertension: A meta-analysis","volume":"13","author":"Raissi","year":"2023","journal-title":"J. Clin. Imaging Sci."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1097\/MPG.0000000000002597","article-title":"Long-term Outcome of Transjugular Intrahepatic Portosystemic Shunt in Children With Portal Hypertension","volume":"70","author":"Nicastro","year":"2020","journal-title":"J. Pediatr. Gastroenterol. 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