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Primary sclerosing cholangitis is a chronic disease of the intra- and extrahepatic bile ducts leading to obstructions and dilatations followed by liver cirrhosis and in some cases cholangiocarcinoma. In 50% of cases primary sclerosing cholangitis is associated with inflammatory bowel disease. The cause of the disease is unknown and many questions concerning the pathogenesis still remain open. In recent years our knowledge of the natural course, the risk of cholangiocarcinoma and the possibility of medical, endoscopic and surgical treatment has increased. Ursodeoxycholic acid, endoscopic treatment and liver transplantation have become standard therapeutic procedures.
This book is the proceedings of the Falk Workshop held on October 3, 1997, in Freiburg, Germany (Part II of the Liver Week Freiburg 1997), and covers the most important aspects of this fascinating disease.
Sommario
Preface. I: Diagnosis, Aetiology and Pathogenesis. 1. PSC: Diagnosis and Differential Diagnosis; K.M. Boberg, E. Schrumpf. 2. Histopathology of Primary Sclerosing Cholangitis; J. Ludwig. 3. Immunogenetics and Epidemiology of Primary Sclerosing Cholangitis; P.T. Donaldson. 4. Etiopathogenesis of Primary Sclerosing Cholangitis; J.M. Vierling. 5. Autoimmune Markers in Primary Sclerosing Cholangitis; U. Hopf, et al. II: Natural History and Associated Diseases. 6. The Natural History of Primary Sclerosing Cholangitis; R.W. Chapman. 7. Primary Sclerosing Cholangitis - Relationship to Inflammatory Bowel Disease; U. Broomé. 8. Risk of Cholangiocarcinoma in Primary Sclerosing Cholangitis; S.M. Riordan, R. Williams. 9. Extrahepatic Manifestations of Primary Sclerosing Cholangitis; J. Neuberger. III: Non-Surgical Treatment. 10. Treatment of Primary Sclerosing Cholangitis: On the Role of Ursodeoxycholic Acid and Endoscopy; A. Stiehl, et al. 11. Immunosuppressive Therapy in Primary Sclerosing Cholangitis; M.M. Kaplan. 12. Endoscopic Treatment of Primary Sclerosing Cholangitis Patients; S. Wagner, et al. 13. Treatment of Complications of Primary Sclerosing Cholangitis; C.I.J. Ponsioen, K. Huibregtse. Index.
Riassunto
Primary sclerosing cholangitis is a chronic disease of the intra- and extrahepatic bile ducts leading to obstructions and dilatations followed by liver cirrhosis and in some cases cholangiocarcinoma.
