{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T12:19:02Z","timestamp":1777983542627,"version":"3.51.4"},"reference-count":18,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2018,2,17]],"date-time":"2018-02-17T00:00:00Z","timestamp":1518825600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2018,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and Aim<\/jats:title><jats:p>Prognostic scoring systems (<jats:styled-content style=\"fixed-case\">PSS<\/jats:styled-content>) have not been validated in children with chronic Budd\u2010Chiari syndrome (<jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>). We aimed to analyse the long\u2010term outcome of radiological intervention (<jats:styled-content style=\"fixed-case\">RI<\/jats:styled-content>) and validate the <jats:styled-content style=\"fixed-case\">PSS<\/jats:styled-content> in children.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Chronic <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> children were analysed in four subgroups: (i) <jats:styled-content style=\"fixed-case\">SI<\/jats:styled-content>: successful intervention (primary or secondary stent patency) (ii) <jats:styled-content style=\"fixed-case\">PO<\/jats:styled-content>: poor outcome (refractory stent block or requirement of liver transplantation), (iii) <jats:styled-content style=\"fixed-case\">NU<\/jats:styled-content>: na\u00efve unintervened (awaiting <jats:styled-content style=\"fixed-case\">RI<\/jats:styled-content>) and (iv) <jats:styled-content style=\"fixed-case\">DBI<\/jats:styled-content>: died before intervention. <jats:styled-content style=\"fixed-case\">PSS<\/jats:styled-content> analysed included Paediatric end\u2010stage liver disease (<jats:styled-content style=\"fixed-case\">PELD<\/jats:styled-content>), Rotterdam, <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>\u2010Transjuglar intrahepatic Portosystemic shunt (<jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TIPS<\/jats:styled-content>) index, Zeitoun, Child\u2010Pugh and Model for end\u2010stage liver disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 113 <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> children, 48 children underwent 53 successful primary <jats:styled-content style=\"fixed-case\">RI<\/jats:styled-content>. Actuarial probability of vascular patency was 87% at 1\u00a0year and 82% at 5\u00a0years follow\u2010up. Four groups (<jats:styled-content style=\"fixed-case\">SI<\/jats:styled-content>: n\u00a0=\u00a040, <jats:styled-content style=\"fixed-case\">PO<\/jats:styled-content>: n\u00a0=\u00a07, <jats:styled-content style=\"fixed-case\">NU<\/jats:styled-content>: n\u00a0=\u00a013, <jats:styled-content style=\"fixed-case\">DBI<\/jats:styled-content>: n\u00a0=\u00a06) were analysed. Univariate analysis showed pre\u2010intervention <jats:styled-content style=\"fixed-case\">PELD<\/jats:styled-content> score [<jats:styled-content style=\"fixed-case\">PO<\/jats:styled-content>: 11 (\u22121\u201023) vs <jats:styled-content style=\"fixed-case\">SI<\/jats:styled-content>: 2 (\u22128\u201025), <jats:italic>P<\/jats:italic>\u00a0=\u00a0.009] with a cut\u2010off of 4 (<jats:styled-content style=\"fixed-case\">AUC<\/jats:styled-content>: 0.809, 86% sensitivity, 75% specificity) determined PO following intervention. In unintervened group (<jats:styled-content style=\"fixed-case\">NU<\/jats:styled-content> vs <jats:styled-content style=\"fixed-case\">DBI<\/jats:styled-content>), multivariate analysis demonstrated that Zeitoun score predicted death independently (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> 15.4, 95% CI: 1.17\u2010203.56, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.04) with a cut\u2010off of 4.3 (<jats:styled-content style=\"fixed-case\">AUC<\/jats:styled-content>: 0.923, 83% sensitivity and 77% specificity).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Children with <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> have a favourable long\u2010term outcome. Among those undergoing <jats:styled-content style=\"fixed-case\">RI<\/jats:styled-content>, pre\u2010intervention <jats:styled-content style=\"fixed-case\">PELD<\/jats:styled-content> score determines the outcome. Survival is determined by Zeitoun score in those unintervened.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.13683","type":"journal-article","created":{"date-parts":[[2018,1,3]],"date-time":"2018-01-03T11:56:04Z","timestamp":1514980564000},"page":"1308-1315","source":"Crossref","is-referenced-by-count":17,"title":["Prognostic scoring systems and outcome of endovascular radiological intervention of chronic Budd\u2010Chiari syndrome in children"],"prefix":"10.1111","volume":"38","author":[{"given":"Sumit K.","family":"Singh","sequence":"first","affiliation":[{"name":"Department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical Sciences  Lucknow India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Moinak","family":"Sen Sarma","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical Sciences  Lucknow 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