{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T17:46:45Z","timestamp":1791481605931,"version":"4.3.4"},"reference-count":41,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2011,8,8]],"date-time":"2011-08-08T00:00:00Z","timestamp":1312761600000},"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":[[2011,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n            <jats:bold>Background: <\/jats:bold> Biliary atresia (BA) is associated with extrahepatic congenital malformations in a minority of affected infants. The term commonly applied to this subgroup is \u2018BASM\u2019 for <jats:italic>biliary atresia splenic malformation<\/jats:italic> syndrome, as spleen abnormalities are prominent.<\/jats:p><jats:p>\n            <jats:bold>Aims and methods: <\/jats:bold> To examine clinical outcome in Canadian BA patients with extrahepatic congenital malformations in the Canada\u2010wide BA database of patients born between 1985 and 2002, and additionally, to recharacterized the syndrome. Patients had \u22651 of the following: a\/polysplenia, abnormal abdominal situs, intestinal malrotation, abdominal vascular anomaly or congenital heart disease.<\/jats:p><jats:p>\n            <jats:bold>Results: <\/jats:bold> Among 328 BA patients, 44 (13%) had associated congenital abnormalities. Intra\u2010abdominal anomalies included polysplenia (<jats:italic>n<\/jats:italic>=25), abnormal abdominal situs (<jats:italic>n<\/jats:italic>=9), intestinal malrotation (<jats:italic>n<\/jats:italic>=19), portal vein anomaly (<jats:italic>n<\/jats:italic>=12), hepatic artery anomaly (<jats:italic>n<\/jats:italic>=3) and inferior vena cava interruption (<jats:italic>n<\/jats:italic>=20). Twenty\u2010six patients had cardiac malformations including pulmonary stenosis (<jats:italic>n<\/jats:italic>=11), ventricular septal defect (<jats:italic>n<\/jats:italic>=10), atrial septal defect (<jats:italic>n<\/jats:italic>=7), total anomalous pulmonary venous return (<jats:italic>n<\/jats:italic>=3), double outlet right ventricle (<jats:italic>n<\/jats:italic>=3), tetralogy of Fallot (<jats:italic>n<\/jats:italic>=2), atrioventricular canal (<jats:italic>n<\/jats:italic>=2), dextrocardia (<jats:italic>n<\/jats:italic>=2), bicuspid aortic valve (<jats:italic>n<\/jats:italic>=2), hypoplastic left heart (<jats:italic>n<\/jats:italic>=1) and partial anomalous pulmonary venous return (<jats:italic>n<\/jats:italic>=1). Age at Kasai operation, performance of liver transplant, overall survival, post\u2010Kasai native liver survival and transplant survival were comparable to isolated BA. Presence of polysplenia or complex cardiac disease did not reduce post\u2010Kasai native liver survival. Three patients had \u22652 typical abnormalities without polysplenia: thus, splenic malformations are not essential to this BA subgroup. Hierarchical cluster analysis demonstrated characteristic abnormalities grouped in a multiplicity of combinations, consistent with a spectrum of defective lateralization.<\/jats:p><jats:p>\n            <jats:bold>Conclusion: <\/jats:bold> We suggest that the acronym \u2018BASM\u2019 be redefined as \u2018biliary atresia <jats:italic>structural<\/jats:italic> malformation\u2019.<\/jats:p>","DOI":"10.1111\/j.1478-3231.2011.02578.x","type":"journal-article","created":{"date-parts":[[2011,8,8]],"date-time":"2011-08-08T04:29:15Z","timestamp":1312777755000},"page":"1485-1493","source":"Crossref","is-referenced-by-count":51,"title":["Biliary atresia with associated structural malformations in Canadian infants"],"prefix":"10.1111","volume":"31","author":[{"given":"Orlee R.","family":"Guttman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eve 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