Introduction to Mini-Symposium on Cholangiocyte Pathophysiology—Part I

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It is my great pleasure to present in this issue of Digestive and Liver Disease, Part I of the 2010 Mini-Symposium on Cholangiocyte Pathophysiology, for which I have gladly accepted the role of Guest Editor. For this collection of articles, the Editorial Board has invited an impressive line-up of renowned international experts to write comprehensive and up-to-date reviews on a wide range of topics centered on cholangiocyte proliferation, differentiation, and mechanisms of disease. To all the dedicated Authors whose efforts made this series possible I would like to extend the gratitude of the Editorial Board, and congratulate them for the outstanding quality of their manuscripts. I am confident that these articles will benefit both researchers and clinicians alike, and I look forward to enriching the series in upcoming issues of the Journal throughout the year.

In Part I you will find the following manuscripts exploring both the biological basis as well as the clinical manifestations of cholangiocyte pathology.

The first review article entitled “Clinical implications of novel aspects of biliary pathophysiology” by Marzioni et al. is an overview of the most recent findings on cholangiocyte pathophysiology, and their clinical interpretations. The review highlights how the heterogeneity of cholangiocytes, their ability to synthesize and release peptides in response to injury, their phenotypical changes, and their response to infectious and inflammatory soluble factors are all relevant components for a better understanding the mechanisms underlying the development of chronic cholestatic diseases and of biliary cancers.

The second review article entitled “Recent Advances in the Regulation of Cholangiocyte Proliferation and Function During Extrahepatic Cholestasis” by Glaser et al. summarizes recent advances in the mechanisms of cholangiocyte hyperplasia in the cholestatic bile duct ligated (BDL) rat model. Cholangiocyte proliferation and death is present in virtually all human cholangiopathies, and several recent studies have provided insights into the key mechanisms that regulate the proliferation and function of cholangiocytes during the pathogenesis of cholestatic liver diseases. The proliferation of cholangiocytes is regulated by a number of neuroendocrine factors that cholangiocytes secrete and respond to in order to regulate their own proliferation. Understanding the factors that regulate cholangiocyte proliferation during cholestasis is critical for the development of treatments for cholestatic liver diseases.

The next review article entitled “Cholangiocarcinoma: update and future perspectives”, by Gatto et al. explores the management of cholangiocarcinoma (CCA), a rare but extremely challenging cancer with a very poor prognosis. Several recent epidemiological studies have shown that incidence and mortality rates for CCA are increasing worldwide, in particular for intrahepatic CCA. The review also explores the potential for screening biomarkers, as well as diagnostic approaches and current treatment options.

Finally, in the review article entitled “Polycystic liver diseases”, Onori et al. tackle the complex field of polycystic kidney and liver disease. Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary disease characterized by the formation of innumerable cysts in the kidney accompanied, in over 90% of cases, by bile duct-derived liver cysts. The paper illustrates the known mechanisms promoting the growth and development of liver cysts, which could guide the development of pharmacological therapies to block liver cyst growth, thus reducing the clinical consequences of ADPKD cystic liver disease.

I am confident that our readers will benefit from these comprehensive manuscripts, and hope that they will enjoy reading them as much as I have.

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Conflict of interest statement 

None declared.