Original articleA multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000
Section snippets
Methods
A retrospective study was undertaken to analyze the results of a large cohort of patients with biliary atresia from a diverse group of US institutions with expertise in management of children with liver disease. As a first step, BARC embarked on a retrospective study of the clinical presentation and current management of biliary atresia and the surgical results after HPE. This analysis was particularly important because most of the literature on outcome of HPE from the United States are reports
Associated Congenital Anomalies
Among the 104 patients, 78 (75%) had no congenital anomalies identified, 13 (12.5%) had 1 anomaly, and the remaining 13 (12.5%) had more than 1 anomaly. The most common anomaly was splenic malformation (12 subjects); the second most common was interrupted inferior vena cava (11 subjects). Eleven of the 13 infants with more than 1 anomaly had BASM (1 asplenia, 10 polysplenia). Nine subjects had a cardiac malformation, and 9 had intestinal malrotation. The most common grouping included 7 subjects
Discussion
This study describes a population of 104 infants with biliary atresia diagnosed and treated between 1997 and 2000 at 9 centers participating in the BARC. Thus, the patient population and outcomes are likely to reflect the current standard practice against which future changes in management and clinical trials can be measured. At present, HPE is the only effective therapy for biliary atresia short of liver transplantation. Thus, the findings of most interest were the rate of success of HPE and
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Supported by the National Institutes of Health (grants U01DK062436, U01DK062445, U01DK062452, U01DK062453, U01DK062456, U01DK062466, U01DK062481, U01DK062497, U01DK062500, and U01DK062503).
The initial draft of the manuscript was written jointly by Benjamin Shneider, Ronald Sokol, Morton Brown, Barbara Haber, and Peter Whitington. No honorarium, grant, or other form of payment was rendered for the preparation of this manuscript.
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