• Pierre Russo, John C Magee, Robert A Anders, Kevin E Bove, Catherine Chung, Oscar W Cummings, Milton J Finegold, Laura S Finn, Grace E Kim, Mark A Lovell, Margret S Magid, Hector Melin-Aldana, Sarangarajan Ranganathan, Bahig M Shehata, Larry L Wang, Frances V White, Zhen Chen, Catherine Spino, and Childhood Liver Disease Research Network (ChiLDReN).
• *Department of Pathology and Laboratory Medicine, the Children's Hospital of Philadelphia, Philadelphia, PA ¶¶Department of Pathology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA Departments of †Surgery §§§Biostatistics, University of Michigan, Ann Arbor, MI ‡Department of Pathology, Johns Hopkins School of Medicine, Baltimore, MD §Division of Pediatric Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH ¶Department of Pathology, Indiana University School of Medicine, Indianapolis, IN #Department of Pathology, Texas Children's Hospital, Houston, TX **Department of Pathology, Seattle Children's Hospital, Seattle, WA ††Department of Pathology, University of California San Francisco, San Francisco, CA ***Department of Pathology, Children's Hospital Los Angeles, Los Angeles, CA ‡‡Department of Pathology, Children's Hospital Colorado, Aurora, CO §§Department of Pathology, Kravis Children's Hospital, Mount Sinai Health System, New York, NY ∥∥Department of Pathology, Ann & Robert H. Lurie Children's Hospital, Chicago, IL ##Department of Pathology, Children's Healthcare of Atlanta, Atlanta, GA †††Department of Pathology and Immunology, Washington University School of Medicine, St Louis, MO ‡‡‡Quest Diagnostics, Health Informatics, Madison, NJ ∥Division of Pathology, The Hospital of Sick Children, Toronto, ON, Canada.
• Am. J. Surg. Pathol. 2016 Dec 1; 40 (12): 1601-1615.

AbstractThe liver biopsy guides diagnostic investigation and therapy in infants with undiagnosed cholestasis. Histologic features in the liver may also have prognostic value in the patient with biliary atresia (BA). We assessed the relative value of histologic features in 227 liver needle biopsies in discriminating between BA and other cholestatic disorders in infants enrolled in a prospective Childhood Liver Disease Research Network (ChiLDReN) cohort study by correlating histology with clinical findings in infants with and without BA. In addition, we reviewed 316 liver biopsies from clinically proven BA cases and correlated histologic features with total serum bilirubin 6 months after hepatoportoenterostomy (the Kasai procedure, HPE) and transplant-free survival up to 6 years. Review pathologists were blinded to clinical information except age. Semiquantitative scoring of 26 discrete histologic features was based on consensus. Bile plugs in portal bile ducts/ductules, moderate to marked ductular reaction, and portal stromal edema had the largest odds ratio for predicting BA versus non-BA by logistic regression analysis. The diagnostic accuracy of the needle biopsy was estimated to be 90.1% (95% confidence interval [CI]: 85.2%, 94.9%), whereas sensitivity and specificity for a diagnosis of BA are 88.4% (95% CI: 81.4, 93.5) and 92.7% (95% CI: 84.8, 97.3), respectively. No histologic features were associated with an elevated serum bilirubin 6 months after HPE, although it (an elevated serum bilirubin) was associated with an older age at HPE. Higher stages of fibrosis, a ductal plate configuration, moderate to marked bile duct injury, an older age at HPE, and an elevated international normalized ratio were independently associated with a higher risk of transplantation.

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