Extrahepatic Anomalies in Infants With Biliary Atresia: Results of a Large Prospective North American Multicenter Study

• DOI: 10.1002/hep.26512
• Publication Date: 2013-05-23T21:32:47Z
• Authors: Kathleen B. Schwarz; Barbara H. Haber; Philip Rosenthal; Cara L. Mack; Jeffrey Moore; Kevin Bove; Jorge A. Bezerra; Saul J. Karpen; Nanda Kerkar; Benjamin L. Shneider; Yumirle P. Turmelle; Peter F. Whitington; Jean P. Molleston; Karen F. Murray; Vicky L. Ng; René Romero; Kasper S. Wang; Ronald J. Sokol; John C. Magee
• Keywords: Etiology

The etiology of biliary atresia (BA) is unknown. Given that patterns anomalies might provide etiopathogenetic clues, we used data from the North American Childhood Liver Disease Research and Education Network to analyze in infants with BA. In all, 289 who were enrolled prospective database prior surgery at any 15 participating centers evaluated. Group 1 was nonsyndromic, isolated BA (without major malformations) (n = 242, 84%), 2 least one malformation considered as defined by National Birth Defects Prevention Study but without laterality defects 17, 6%). 3 syndromic, 30, 10%). population a whole, (either or minor) most prevalent cardiovascular (16%) gastrointestinal (14%) systems. patients accounted for majority subjects cardiac, gastrointestinal, splenic anomalies. also frequently displayed (71%) (24%) anomalies; interestingly, this group had genitourinary more (47%) compared (10%).