This volume contains the contributions to the International Falk Workshop `Vanishing Bile Duct Syndrome' held in Spoleto, Italy, May 30 - June 1, 1996. Vanishing bile duct syndrome comprises a number of chronic cholestatic diseases caused by primary immunological and functional damage to the biliary epithelium. Important advances have recently been made in the biology, physiology and treatment of vanishing bile duct syndrome. In particular, recent progress in the biology and physiology of the intrahepatic biliary epithelium has added considerably to the current understanding of the pathophysiology of this syndrome. The guiding principle behind the Workshop and these proceedings was to pursue an interdisciplinary approach to research aspects, clinical management and therapeutic strategy of vanishing bile duct syndrome. This book summarizes the experience of investigators and clinicians actively working on the various aspects of this syndrome, from basic science to clinical practice.
Preface. Rome: Foundation and Mysteries; L. Capocaccia.Section I:Basic Sciences.1. Opening Lecture: Vanishing Bile Duct Syndrome Pathophysiology and Treatment; J.L. Boyer.2. Anatomy, Development and Pathology of the Biliary Tree; V.J. Desmet.3. Subcellular Features of the Biliary Epithelium in Health and Disease; A.M. Jezequel, et al.4. Experimental Models to Study the Physiology of Biliary Epithelium; S.A. Levine, N.F. LaRusso.5. Morphological and Functional Heterogeneity of the Rat Intrahepatic Biliary Tree; G. Alpini, et al.6. Hormonal Regulation of Electrolyte Transport in Biliary Epithelial Cells; D. Alvaro, et al.7. Evidence for Paracrine Regulation of Biliary Cl-Secretion by Purinergic Signalling; J.G. Fitz.8. Transport of Non-Electrolyte Biliary Components in Cholangiocytes; A. Benedetti.9. Regulation of Biliary Bicarbonate Secretion by Bile Salts; C.Clerici, et al.10. The Cholehepatic Circulation of Organic Anions: A Decade of Progress; A.F. Hofmann, et al.Section II: Pathophysiology.11. Molecular Biology of Cholestasis; P.J. Meier.12. Pathophysiology of the Biliary Epithelium; M. Strazzabosco, et al.13. The Role of mdr2 P-Glycoprotein in Lipid Secretion and Ductular Damage; C. vanNieuwkerk, et al.14. Pathogenesis of Non-Suppurative Destructive Cholangitis; R. Joplin, J. Neuberger.15. Immunological Mechanisms of Cholangiocyte Injury; J.M. Vierling.16. Immunopathology of Vanishing Bile Duct Syndrome; C. Galperin, et al.17. Immunomodulatory Properties of Bile Acids; Y. Calmus, et al.Section III: ClinicalAspects and Therapy.18. Acute and Chronic Liver Allograft Rejection; A.J. Demetris, et al.19. Transplantation for Vanishing Bile Duct Syndromes; J. Neuberger.20. Medical Treatment of Vanishing Bile Duct Syndrome; U. Leuschner.21. Treatment of Vanishing Bile Duct Syndrome with Ursodeoxycholic Acid; M. Podda, et al.22. Mechanism of Action of UDCA in Cholestatic Syndromes; U. Beuers, et al.23. Vanishing Bile Duct Syndrome: From Bench to Bedside; J.L. Boyer. Index.