Cholangiocyte Injury and Ductopenic Syndromes

Xuefeng Xia; Sharon DeMorrow; Heather Francis; Shannon Glaser; Gianfranco Alpini; Marco Marzioni; Giammarco Fava; Gene LeSage

doi:10.1055/s-2007-991516

Seminars in Liver Disease, Table of Contents

Semin Liver Dis 2007; 27(4): 401-412
DOI: 10.1055/s-2007-991516

Cholangiocyte Injury and Ductopenic Syndromes

Xuefeng Xia¹ , Sharon DeMorrow² , Heather Francis² , Shannon Glaser² ^, ⁴ , Gianfranco Alpini³ ^, ⁴ , Marco Marzioni⁵ , Giammarco Fava⁵ , Gene LeSage¹

¹The University of Texas Houston Medical School, Houston, Texas
²Division R&E, Scott & White Hospital, College of Medicine, Temple, Texas
³Division Research, Central Texas Veterans Health Care System, Department of Medicine, Scott & White and The Texas A&M University System Health Science Center, College of Medicine, Temple, Texas
⁴Systems Biology and Translational Medicine, The Texas A&M University System Health Science Center, College of Medicine, Temple, Texas
⁵Department of Gastroenterology, Polytechnic University of Marche, Ancona, Italy

Abstract

ABSTRACT

Cholangiopathies are characterized by a predominately bile duct-directed inflammatory response that leads to bile duct injury and, if the injury is persistent, bile duct loss and the chance of developing bile duct cancer (cholangiocarcinoma). Although the cholangiopathies have broad range of etiologies and pathogenesis (e.g., inherited disorders, autoimmune disorders, infections, drug-induced, ischemia, and unknown etiology), all share the common pathogenetic target-the biliary epithelial cell (cholangiocyte). For the most part, the pathogenesis of these diseases is poorly understood, which correspondingly has restricted clinicians to nonspecific and usually ineffective therapies for these disorders. Nevertheless, significant advances toward the understanding of the mechanisms involved in cholangiocyte-directed inflammation, biliary fibrosis, cholangiocyte death, and cholangiocarcinoma have unfolded over the past 15 years that may provide us new hopes and schemes for treatment of these disorders.

KEYWORDS

Bile duct epithelium - ductopenia - primary biliary cirrhosis - primary sclerosing cholangitis - cholangiopathy

Full Text

References

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Gene LeSageM.D.

Professor of Medicine, The University of Texas Houston Medical School

6431 Fannin Street, MSB 4.234, Houston, TX 77030

Email: gene.lesage@uth.tmc.edu