PubMed ID:
23703680
Public Release Type:
Journal
Publication Year: 2013
Affiliation: Johns Hopkins Medical Institutions, Baltimore, MD.
DOI:
https://doi.org/10.1002/hep.26512
Authors:
Bezerra JA,
Bove K,
Childhood Liver Disease Research and Education Network,
Haber BH,
Karpen SJ,
Kerkar N,
Mack CL,
Magee JC,
Molleston JP,
Moore J,
Murray KF,
Ng VL,
Romero R,
Rosenthal P,
Schwarz KB,
Shneider BL,
Sokol RJ,
Turmelle YP,
Wang KS,
Whitington PF
Studies:
A Prospective Database of Infants With Cholestasis
The etiology of biliary atresia (BA) is unknown. Given that patterns of anomalies might provide etiopathogenetic clues, we used data from the North American Childhood Liver Disease Research and Education Network to analyze patterns of anomalies in infants with BA. In all, 289 infants who were enrolled in the prospective database prior to surgery at any of 15 participating centers were evaluated. Group 1 was nonsyndromic, isolated BA (without major malformations) (n = 242, 84%), Group 2 was BA and at least one malformation considered major as defined by the National Birth Defects Prevention Study but without laterality defects (n = 17, 6%). Group 3 was syndromic, with laterality defects (n = 30, 10%). In the population as a whole, anomalies (either major or minor) were most prevalent in the cardiovascular (16%) and gastrointestinal (14%) systems. Group 3 patients accounted for the majority of subjects with cardiac, gastrointestinal, and splenic anomalies. Group 2 subjects also frequently displayed cardiovascular (71%) and gastrointestinal (24%) anomalies; interestingly, this group had genitourinary anomalies more frequently (47%) compared to Group 3 subjects (10%).