Screening and outcomes in biliary atresia

Summary of a national institutes of health workshop

Ronald J. Sokol*, Ross W. Shepherd, Riccardo A Superina, Jorge A. Bezerra, Patricia Robuck, Jay H. Hoofnagle

*Corresponding author for this work

Research output: Contribution to journalArticle

139 Citations (Scopus)

Abstract

Biliary atresia is the most common cause of end-stage liver disease in the infant and is the leading pediatric indication for liver transplantation in the United States. Earlier diagnosis (<30-45 days of life) is associated with improved outcomes following the Kasai portoenterostomy and longer survival with the native liver. However, establishing this diagnosis is problematic because of its rarity, the much more common indirect hyperbilirubinemia that occurs in the newborn period, and the schedule for routine infant health care visits in the United States. The pathogenesis of biliary atresia appears to involve immune-mediated fibro-obliteration of the extrahepatic and intrahepatic biliary tree in most patients and defective morphogenesis of the biliary system in the remainder. The determinants of the outcome of portoenterostomy include the age at surgery, the center's experience, the presence of associated congenital anomalies, and the postoperative occurrence of cholangitis. A number of screening strategies in infants have been studied. The most promising are early measurements of serum conjugated bilirubin and a stool color card given to new parents that alerts them and their primary care provider to acholic stools. This report summarizes a National Institutes of Health workshop held on September 12 and 13, 2006, in Bethesda, MD, that addressed the issues of outcomes, screening, and pathogenesis of biliary atresia.

Original languageEnglish (US)
Pages (from-to)566-581
Number of pages16
JournalHepatology
Volume46
Issue number2
DOIs
StatePublished - Aug 1 2007

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Biliary Atresia
National Institutes of Health (U.S.)
Biliary Tract
Education
Infant Care
End Stage Liver Disease
Cholangitis
Hyperbilirubinemia
Morphogenesis
Bilirubin
Liver Transplantation
Early Diagnosis
Primary Health Care
Appointments and Schedules
Color
Parents
Newborn Infant
Pediatrics
Delivery of Health Care
Survival

ASJC Scopus subject areas

  • Hepatology

Cite this

Sokol, R. J., Shepherd, R. W., Superina, R. A., Bezerra, J. A., Robuck, P., & Hoofnagle, J. H. (2007). Screening and outcomes in biliary atresia: Summary of a national institutes of health workshop. Hepatology, 46(2), 566-581. https://doi.org/10.1002/hep.21790
Sokol, Ronald J. ; Shepherd, Ross W. ; Superina, Riccardo A ; Bezerra, Jorge A. ; Robuck, Patricia ; Hoofnagle, Jay H. / Screening and outcomes in biliary atresia : Summary of a national institutes of health workshop. In: Hepatology. 2007 ; Vol. 46, No. 2. pp. 566-581.
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Sokol, RJ, Shepherd, RW, Superina, RA, Bezerra, JA, Robuck, P & Hoofnagle, JH 2007, 'Screening and outcomes in biliary atresia: Summary of a national institutes of health workshop', Hepatology, vol. 46, no. 2, pp. 566-581. https://doi.org/10.1002/hep.21790

Screening and outcomes in biliary atresia : Summary of a national institutes of health workshop. / Sokol, Ronald J.; Shepherd, Ross W.; Superina, Riccardo A; Bezerra, Jorge A.; Robuck, Patricia; Hoofnagle, Jay H.

In: Hepatology, Vol. 46, No. 2, 01.08.2007, p. 566-581.

Research output: Contribution to journalArticle

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