{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T21:59:53Z","timestamp":1790200793009,"version":"4.1.0"},"reference-count":42,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2023,5,8]],"date-time":"2023-05-08T00:00:00Z","timestamp":1683504000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2023,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and Aims<\/jats:title><jats:p>In paediatrics, porto\u2010sinusoidal vascular disease (PSVD) is relatively unknown and probably underdiagnosed. We aimed to describe clinical phenotypes, histology and outcome of children diagnosed with PSVD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Retrospective multicentre study of children diagnosed with PSVD. Diagnosis of PSVD was based on histopathology reports; liver specimens were re\u2010evaluated by two expert liver pathologists.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty two children diagnosed with PSVD (M\/F\u2009=\u200936\/26, median age 6.6\u2009years, range 3.3\u201310.6), from 7 centres, were included. Thirty\u2010six presented with non\u2010cirrhotic portal hypertension, PH, (PH\u2010PSVD Group\u2009=\u200958%) while 26 had a liver biopsy because of chronic elevation of transaminases without PH (noPH\u2010PSVD Group\u2009=\u200942%). On histology review, the two groups differed for the prevalence of obliterative portal venopathy (more prevalent in PH\u2010PSVD, <jats:italic>p<\/jats:italic>\u2009=\u20090.005), and hypervascularised portal tracts (more common in noPH\u2010PSVD, <jats:italic>p<\/jats:italic>\u2009=\u20090.039), the other histological changes were equally distributed. At multivariate analysis, platelet count \u2264185\u2009000\/mm<jats:sup>3<\/jats:sup> was the only independent determinant of PH (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). After a median follow\u2010up of 7\u2009years (range 3.0\u201311.2), in PH\u2010PSVD group 3\/36 (8%) required TIPS placement, 5\/36 (14%) developed pulmonary vascular complications of PH, and 7\/36 (19%) required liver transplantation. In noPH\u2010PSVD none progressed to PH nor had complications.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Paediatric patients with PSVD present with two different clinical phenotypes, one characterised by PH and one by chronic elevation of transaminases without PH. PSVD should be included among the conditions causing isolated hypertransaminasaemia. On histology, the differences between the two groups are subtle. Medium\u2010term outcome is favourable in patients without PH; progression of the disease is observed in those with PH.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.15603","type":"journal-article","created":{"date-parts":[[2023,5,9]],"date-time":"2023-05-09T06:50:27Z","timestamp":1683615027000},"page":"1523-1536","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Paediatric porto\u2010sinusoidal vascular disease: Two different clinical phenotypes with subtle histological differences"],"prefix":"10.1111","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0363-5565","authenticated-orcid":false,"given":"Angelo","family":"Di\u00a0Giorgio","sequence":"first","affiliation":[{"name":"Paediatric Hepatology, Gastroenterology and Transplantation Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7635-3791","authenticated-orcid":false,"given":"Lorenza","family":"Matarazzo","sequence":"additional","affiliation":[{"name":"Paediatric Hepatology, Gastroenterology and Transplantation Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aurelio","family":"Sonzogni","sequence":"additional","affiliation":[{"name":"Department of Pathology ASST Bergamo Est  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuele","family":"Nicastro","sequence":"additional","affiliation":[{"name":"Paediatric Hepatology, Gastroenterology and Transplantation Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4426-0395","authenticated-orcid":false,"given":"Andrea","family":"Pietrobattista","sequence":"additional","affiliation":[{"name":"Hepatology, Gastroenterology, Digestive Endoscopy, Nutrition, and Liver Transplantation Unit IRCCS Bambino Ges\u00f9, Pediatric Hospital  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mara","family":"Cananzi","sequence":"additional","affiliation":[{"name":"Unit of Gastroenterology, Digestive Endoscopy, Hepatology and Care of the Child with Liver Transplantation University Hospital of Padova  Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Gaio","sequence":"additional","affiliation":[{"name":"Unit of Gastroenterology, Digestive Endoscopy, Hepatology and Care of the Child with Liver Transplantation University Hospital of Padova  Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Sciveres","sequence":"additional","affiliation":[{"name":"Paediatric Department and Transplantation ISMETT  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grazia","family":"Di\u00a0Leo","sequence":"additional","affiliation":[{"name":"IRCCS Burlo Garofolo  Trieste Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaele","family":"Iorio","sequence":"additional","affiliation":[{"name":"Department of Translational Medical Science, School of Medicine and Surgery University of Naples Federico II  Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Marseglia","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Casa Sollievo della Sofferenza Division of Pediatrics  San Giovanni Rotondo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Greta","family":"Carioli","sequence":"additional","affiliation":[{"name":"FROM Research Foundation Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Maggiore","sequence":"additional","affiliation":[{"name":"Hepatology, Gastroenterology, Digestive Endoscopy, Nutrition, and Liver Transplantation Unit IRCCS Bambino Ges\u00f9, Pediatric Hospital  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Guido","sequence":"additional","affiliation":[{"name":"Department of Medicine\u2010DIMED University of Padova  Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7150-3148","authenticated-orcid":false,"given":"Lorenzo","family":"D'Antiga","sequence":"additional","affiliation":[{"name":"Paediatric Hepatology, Gastroenterology and Transplantation Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,5,8]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2468-1253(19)30047-0"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jceh.2014.07.005"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.26904"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0028-1085095"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2007-1007285"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2014.03.025"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2022.05.033"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.24422"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.12936"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2010.07.038"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.15257"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.2147\/HMER.S282674"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2021.12.022"},{"key":"e_1_2_10_15_1","first-page":"534","article-title":"Abdominal venous thrombosis in neonates and infants: role of prothrombotic risk factors\u2014a multicenter case\u2010control study. For the childhood thrombophilia study group","volume":"111","author":"Heller C","year":"2000","journal-title":"Br J Haematol"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.14740\/gr1376"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19990101)82:1<49::AID-AJMG10>3.0.CO;2-M"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.13738"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1136\/gutjnl-2013-306658"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-018-2355-8"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-319-96400-3_19"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.3346\/jkms.2013.28.10.1507"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.transproceed.2013.01.064"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000213425.76621.f1"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840120515"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.51.2.275"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.20431"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840110512"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000003081"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.2003.036806"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2007.08.011"},{"key":"e_1_2_10_32_1","first-page":"139","article-title":"Nodular regenerative hyperplasia of the liver associated with systemic lupus erythematosus: three cases","volume":"15","author":"Leung VK","year":"2009","journal-title":"Hong Kong Med J"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1155\/2015\/349215"},{"key":"e_1_2_10_34_1","first-page":"163","article-title":"Non\u2010specific immunological abnormalities and association of autoimmune diseases in idiopathic portal hypertension. A study by questionnaire","volume":"40","author":"Saito K","year":"1993","journal-title":"Hepato\u2010Gastoenterol"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.13547"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2015.11.027"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.30724"},{"key":"e_1_2_10_38_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.31192"},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.12681"},{"key":"e_1_2_10_40_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840100533"},{"key":"e_1_2_10_41_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000003445"},{"key":"e_1_2_10_42_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1478-3231.2005.01151.x"},{"key":"e_1_2_10_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.dld.2019.02.014"}],"container-title":["Liver International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/liv.15603","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,12,8]],"date-time":"2023-12-08T13:51:17Z","timestamp":1702043477000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/liv.15603"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,8]]},"references-count":42,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2023,7]]}},"alternative-id":["10.1111\/liv.15603"],"URL":"https:\/\/doi.org\/10.1111\/liv.15603","archive":["Portico"],"relation":{},"ISSN":["1478-3223","1478-3231"],"issn-type":[{"value":"1478-3223","type":"print"},{"value":"1478-3231","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,8]]},"assertion":[{"value":"2023-03-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-04-23","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-08","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}