ArticlePediatric surgery international2021
The morphological and histopathological assessment of Alagille syndrome with extrahepatic bile duct obstruction: the importance of the differential diagnosis with subgroup "o" biliary atresia.
Article in Pediatric surgery international, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed, 8 citations in OpenAlex.
- Long-Term Outcomes of Living Donor Liver Transplantation in Children With Alagille Syndrome: Results From Vanguard Multicenter Study of International Living Donor Liver Transplantation Group.Pediatric transplantation · 2026Trial
- A Rare Coexistence of Biliary Atresia and Alagille Syndrome in a Neonate: Clinical Implications of Dual Etiology in Neonatal Cholestasis.Diagnostics (Basel, Switzerland) · 2026Article
- Biliary atresia: the development, pathological features, and classification of the bile duct.Pediatric surgery international · 2024Review
- Different clinical and genetic features of Alagille patients with progressive diseaseJGH open : an open access journal of gastroenterology and hepatology · 2022Article
- Protocols of Investigation of Neonatal Cholestasis-A Critical Appraisal.Healthcare (Basel, Switzerland) · 2022Review
- Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants.Frontiers in pediatrics · 2022Article
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Authors and funding
8 authors at 1 institution in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
purposeThe differential diagnosis between Alagille syndrome (AGS) with extrahepatic bile duct obstruction (EHBDO) and biliary atresia (BA) is difficult. We report a case series of AGS with EHBDO with detailed validation of the morphological and histopathological features for the differential diagnosis of BA.
methodsSix liver transplantations (LTs) were performed for AGS with EHBDO. All patients were diagnosed with BA at the referring institution and the diagnosis of AGS was then confirmed based on a genetic analysis before LT. We verified the morphological and histopathological findings of the porta hepatis and liver at the diagnosis of BA and at LT.
resultsAll patients had acholic stool in the neonatal period and were diagnosed with BA by cholangiography. The gross liver findings included a smooth and soft surface, without any cirrhosis. The gross findings of the porta hepatis included aplasia of the proximal hepatic duct, or subgroup "o", in five patients. The histopathological examination of the EHBD also revealed obstruction/absence of the hepatic duct. There were no patients with aplasia of the common bile duct.
conclusionsAplasia of the hepatic duct and the macroscopic liver findings may help in to differentiate between AGS with EHBDO and BA.
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