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Autoimmune Diseases in Pediatric Gastroenterology / Edition 1

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Overview

This book is the proceedings of the Falk Symposium No. 127 on 'Autoimmune Diseases in Paediatric Gastroenterology' (IV International Falk Symposium on Paediatric Gastroenterology), held in Basel, Switzerland, on November 8-9, 2001. The symposium focused on the role of the immune system, both the acquired and the innate systems, in inflammatory bowel disease (IBD) in children and adolescents. The innate system has an important fundamental role in host defence by initiating immune responses against potentially deleterious matter. However, a mutation within the innate system may elicit an immune response against the host: hence, an autoimmune response.

Chronic autoimmune hepatitis occurs predominantly in young people, and especially in women. Immunological changes are conspicuous. Tissue antibodies are found in a large number of patients. This is a disease of disordered immunoregulation marked by a deficit in suppressor T cells causing the production of autoantibodies against specific hepayte surface antigen. Liver membrane protein is found in the sera of patients with autoimmune chronic acute hepatitis and with primary biliary cirrhosis (PBC). The latter condition of progressive granulomatous destruction of intrahepatic bile ducts is, in many respects, analogous to the graft-versus-host syndrome where the immune system has become sensitized to foreign HLA-molecules.

Primary sclerosing cholangitis (PSC) is another condition of unknown origin. All parts of the biliary tree can be involved in a chronic, fibrosing, inflammatory process that results in obliteration of the biliary tree and ultimately in biliary cirrhosis. About half of the patients also suffer from ulcerative colitis and rarely from Crohn's disease. Circulating antibodies to some antigens are found in obstructed portal tracts, as well as increased concentrations of biliary immune complexes in patients with PBC.
In all three previous Falk symposia on paediatric gastroenterology, attention was focused on the role of the innate immune system in the aetiology of IBD. It has become increasingly clear in recent years that the innate system has a much more important and fundamental role in host defence. The decision to initiate an immune response is one of the major roles of the innate system. Mutations within this system could transform it into becoming constitutively active, resulting in an inflammatory reaction and thus eliciting an autoimmune response.

Following an introduction to the basic phenomena of autoimmunity, the proceedings discuss clinical aspects of autoimmune diseases. In particular, current knowledge and the state of the art about the diagnosis and treatment of the autoimmune diseases of the gastrointestinal tract are described by world-renowned experts. The book also contains the short presentations on selected topics, as well as abstracts of the mini-posters read by title, which were included in the symposium.

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Editorial Reviews

Doody's Review Service
Reviewer: Mark Rhoads, MD (Ochsner Clinic Foundation)
Description: This book details the proceedings of a symposium held in November 2001 focusing on autoimmune GI/Liver diseases. Some reviews are comprehensive while other presentations are brief and poorly illustrated. Particularly instructive and enjoyable are the chapters on molecular mimicry by Vergani et al., on autoimmune enteropathy by Seidman et al., and on the role of enterocytes in intestinal and systemic autoimmunity by Eisenstein and Lebenthal. The chapter on pathology of ulcerative colitis and Crohn's disease is excellent and is one of the few that are well illustrated.
Purpose: The purpose is to address chronic autoimmune diseases in children, including autoimmune hepatitis, autoimmune enteropathy, primary sclerosing cholangitis and inflammatory bowel disease. Both clinical and basic science research are presented, although the majority of presentations are clinical in nature. The book's important objectives are successfully met.
Audience: The book is aimed at clinicians, particularly pediatric gastroenterologists, but would also be useful for investigators who study autoimmune diseases. Many of the authors are internationally recognized experts. Clinicians who treat IBD would especially profit from this book.
Features: The book has several sections which contain excellent 3-10 page reviews on liver and bile duct autoimmunity, inflammatory bowel disease and chronic autoimmune enteropathy. There are subsections that deal with the basic mechanisms of epithelial antigen presentation, T-cell responses, and perpetuation of autoimmunity. Interspersed are short vignettes on these topics. At the end are a number of poster presentations that are of variable quality. The basic science mechanisms of autoimmunity are not clearly presented or diagrammed to benefit novices in the field. As a whole, there are too few diagrams and illustrations.
Assessment: Overall, the book is interesting and easy to read. The longer reviews are excellent. A couple of the smaller vignettes are fascinating, including a description of a number of children who developed autoimmune liver disease after liver transplantation. I also enjoyed the reviews of medical therapy for inflammatory bowel disease, including short articles on budesonide and antitumor necrosis factor therapy. My only criticisms are that there should be more illustrations and that some of the reviews express opinions that are somewhat controversial. (These include the statement that "azathioprine is often hepatotoxic," and the view that alternate day therapy with steroids for inflammatory bowel disease is unsubstantiated.) To my knowledge, there are no other books in the field that are relevant to children with autoimmune liver disease or enteropathy. The book should prove to be useful.

3 Stars from Doody
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Product Details

  • ISBN-13: 9780792387787
  • Publisher: Springer Netherlands
  • Publication date: 9/28/2002
  • Series: Falk Symposium Series , #127
  • Edition description: 2002
  • Edition number: 1
  • Pages: 280
  • Product dimensions: 7.00 (w) x 8.90 (h) x 0.88 (d)

Table of Contents

Preface. List of Principal Authors. Section I: Autoimmunity of the Gastrointestinal Tract. 1. Dynamics of T lymphozyte responses: Intermediates, effectors and memory cells; A. Lanzavecchia. 2. Gut-oriented immune response and oral tolerance to dietary antigens; O. Alpan. 3. Role of molecular mimicry in viral and autoimmune liver disease; D. Vergani. 4. Autoantibodies in autoimmune hepatitis; W.B. Storch. Section II: Pathology and Clinical Manifestations. 5. Pathology of autoimmune hepatitis; D. Huff. 6. Autoimmune hepatitis and sclerosing cholangitis; G. Mieli-Vergani. 7. Primary biliary cirrhosis; U. Leuschner. Section III: Treatment. 8. Paediatric liver transplantation. Geneva experience from 1989 to 2001; C. Le Coultre. 9. Patient management after paediatric liver transplantation; M. Burdelski, et al. 10. De novo autoimmune hepatitis after liver transplantation; N. Hadzic, et al. 11. Ursodeoxycholic acid: its cytoprotective action in liver disease; E.L. Renner. 12. Alopecia associated with anti-LKM antibody-positive AIH; D. Belli. Section IV: Pathology and Aetiology of gut autoimmunity. 13. Acute portal vein thrombosis: treatment by the transjugular approach - A case report; M. Rössle. 14. Pathology of ulcerative colitis and Crohn's disease; J.-O. Gebbers. Section V: Clinical manifestations and treatment of inflammatory bowel disease. 15. Novel perspective on the possible role of enterocytes in intestinal and systemic autoimmunity; E.M. Eisenstein, E. Lebenthal. 16. Long-term outcome of autoimmune enteropathy (AE); P.J. Milla. 17. Aetiology of inflammatory bowel disease; A.J. Wakefield. 18. Medical therapies from inflammatory bowel disease: an overview; G.R. Lichtenstein. 19. Azathioprine in the treatment of paediatric ulcerative colitis; H.K. Harms, et al. 20. Molecular mechanisms of steroid resistance in chronic inflammatory bowel disease; H. Bantel, K. Schulze-Osthoff. 21. Treatment with budesonide in inflammatory bowel disease; R. Behrens. 22. Clinical outcome of IBD during long-term 5-ASA treatment, and gender-related differences in children; F. Hadziselimovic, et al. 23. Anti-TNF strategies in paediatric Crohn's disease; C.P. Braegger. 24. Side-effects associated with use of oral budesonide in children and adolescents; A. Levine. Section VI: Short presentations of selected topics. Section VII: Mini-posters read by title; Case Reports: I: Liver. II: Intestine – Crohn's disease. III: Intestine: – Crohn's disease. Index.

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