Biliary Atresia Remnants Revisited: Myogenesis, Hepatic Duct-Like Structures, and Fate of Peribiliary Glands

Author:

Bove Kevin E.1,Finegold Milton J.2,Harpavat Sanjiv3

Affiliation:

1. Department of Pathology, University of Cincinnati College of Medicine, Cincinnati, OH, USA

2. Department of Pathology-Immunology, Baylor College of Medicine, Houston, TX, USA

3. Department of Pediatrics-Gastroenterology, Baylor College of Medicine, Houston, TX, USA

Abstract

Purpose and Context: Proximal levels of excised remnants from youngest infants may reveal early features of biliary atresia (BA). Method: A targeted IHC survey was applied to 34 most proximal 2 levels in 17 BA remnants excised at age 10–74 days including 7 = <30 days old and 6 control hepatic ducts (HD). Key Results: Severity of inflammation and extent of active fibroplasia do not distinguish proximal remnants in younger (n = 7) and older (n = 10) infants. In 27/34 levels of 14/17 remnants, reactive stroma is focally SM-MHC-2 (+), marking smooth muscle myosin, termed reactive myogenesis (RM), that is absent in controls. RM facilitates identification of 3 novel hepatic duct remnants (HDR): an HD-like collagen collar lined by degenerating cholangiocytes (n = 5); erosion defects in loose reactive stroma (n = 14); solitary foci of hyperplastic squamoid epithelium (n = 4). Peribiliary glands are either hyperplastic or atretic and typically lack RM. Conclusion: Minimally inflammed end-stage lesions in BA remnants occur at youngest ages favoring prenatal onset. Three novel HDR are defined. RM, a useful surrogate for HDR, is a prevalent inappropriate stromal reaction in proximal remnants of uncertain biological significance. RM is the source of mature smooth muscle in BA remnants.

Publisher

SAGE Publications

Subject

General Medicine,Pathology and Forensic Medicine,Pediatrics, Perinatology and Child Health

Cited by 1 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Biliary stem cells in health and cholangiopathies and cholangiocarcinoma;Current Opinion in Gastroenterology;2024-02-08

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