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19 - Sclerosing Cholangitis
- from SECTION III - HEPATITIS AND IMMUNE DISORDERS
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- By Nissa I. Erickson, M.D., Assistant Professor, Department of Pediatrics, University of Wisconsin, Madison, Wisconsin; Medical Director, Pediatric Liver Program, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, University of Wisconsin Children's Hospital, Madison, Wisconsin, William F. Balistreri, M.D., Dorothy M. M. Kersten Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Director, Pediatric Liver Care Center, Department of Pediatric Gastroenterolgy, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
- Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
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- Book:
- Liver Disease in Children
- Published online:
- 18 December 2009
- Print publication:
- 07 May 2007, pp 459-477
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Summary
There is a wide spectrum of etiologically obscure inflammatory disorders of the biliary tract, including the obstructive cholangiopathies that occur in infancy (biliary atresia and related entities); primary biliary cirrhosis, which is noted in adults; and primary sclerosing cholangitis (PSC), which may affect patients of all age groups, particularly those with chronic inflammatory bowel disease (IBD). These hepatobiliary disorders differ markedly in clinical expression but display substantial overlap in morphologic features, suggesting that their pathogenesis may be shared [1]. Because the intra- and extrahepatic biliary tree may be assumed to possess a limited repertoire of reactions to injury caused by various inflammatory mechanisms, the association of PSC and IBD may provide insight into other forms of “cholangitis.” The frequency of this association also presents an opportunity to trace the evolution of PSC. In this chapter, we focus on idiopathic forms of sclerosing cholangitis (SC) in children, the PSC–IBD complex, and related disorders.
DEFINITION
Sclerosing cholangitis is a chronic hepatobiliary disorder characterized by inflammation of the intrahepatic or extrahepatic ducts (or both), leading to focal dilatation, narrowing, or obliteration accompanied by local periductular fibrosis. Progressive, obliterative fibrosis usually leads to biliary cirrhosis and end-stage liver disease. The structural abnormalities of larger bile ducts are best appreciated by cholangiography, which in most cases is essential in establishing the diagnosis. However, careful delineation of the histology of the hepatic parenchyma and smaller intrahepatic ducts may also suggest the diagnosis [2, 3].
Preface to the Third Edition
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- By Frederick J. Suchy, M.D., Professor and Chair, Department of Pediatrics, Mount Sinai School of Medicine of New York University; Pediatrician-in-Chief, Mount Sinai Hospital, New York, Ronald J. Sokol, M.D., Professor and Vice Chair, Department of Pediatrics; Chief, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, University of Colorado School of Medicine and The Childern's Hospital, Denver, Colorado, William F. Balistreri, M.D., Dorothy M. M. Kersten Professor, Department of Pediatrics, University of Cincinnati College of Medicine; Director, Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
- Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
-
- Book:
- Liver Disease in Children
- Published online:
- 18 December 2009
- Print publication:
- 07 May 2007, pp xv-xvi
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Summary
Liver Disease in Children has become the premier reference on pediatric liver disease. This third edition provides authoritative coverage of every aspect of liver disease affecting infants, children, and adolescents. This edition has been thoroughly revised and updated. In addition, it features new contributions on liver development, cholestatic and autoimmune disorders, fatty liver disease, and inborn errors of metabolism. The book offers an integrated approach to the science and clinical practice of pediatric hepatology and charts the substantial progress in understanding and treating these diseases. Chapters are written by international experts and address the unique pathophysiology, manifestations, and management of these disorders in the pediatric population.
In the six years since the publication of the second edition of Liver Disease in Children, pediatric hepatology has continued to evolve as a discipline. Our knowledge of the structural and functional development of the liver continues to grow, aided by sophisticated approaches in molecular biology. For example, the genetic basis of inherited cholestatic disorders has been further elucidated, and the clinician is now provided with useful information about the natural history, spectrum, and options for therapy. Investigators can couple this information with emerging science in their own laboratories. Several canalicular membrane transport proteins were actually discovered based upon their role in inherited disorders of cholestasis. These advances have allowed detailed studies regarding the behavior of these transporters in acquired cholestasis.
17 - Acute and Chronic Viral Hepatitis
- from SECTION III - HEPATITIS AND IMMUNE DISORDERS
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- By Jay A. Hochman, M.D., Clinical Associate Professor, Division of Pediatric Gastroenterology, Emory University School of Medicine, Atlanta, Georgia; Attending Physician, Children's Center for Digestive Health Care, LLC, and Children's Healthcare of Atlanta, Atlanta, Georgia, William F. Balistreri, M.D., Dorothy M. M. Kersten Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Director, Pediatric Liver Care Center, Department of Pediatric Gastroenterolgy, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
- Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
-
- Book:
- Liver Disease in Children
- Published online:
- 18 December 2009
- Print publication:
- 07 May 2007, pp 369-446
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Summary
Optimal care of children with viral hepatitis necessitates incorporation of recent advances in diagnosis, prevention, and treatment into clinical practice. Though primary viral infection of the liver has been recognized since the time of Hippocrates (460–375 b.c.), only in the past two decades have significant scientific advancements allowed clinicians to alter the outcomes of these infections [1]. Specifically, viral hepatitis can be prevented with vaccines and passive immunization and can be treated with antiviral medications. The availability to detect these infections rapidly and accurately has led to changes in the epidemiology of viral hepatitis.
This chapter details the history, epidemiology, and clinical features as well as the diagnostic, preventative, and therapeutic strategies for the most important group of hepatotropic viruses: hepatitis A (HAV), hepatitis B (HBV), hepatitis C (HCV), hepatitis D (HDV), and hepatitis E (HEV). In 2002, the estimated numbers of new infections in the United States were as follows: 73,000 caused by HAV, 79,000 caused by HBV, and 29,000 caused by HCV/non-A, non-B (NANB) hepatitis [2]. The absolute number of cases of acute hepatitis had been reduced by more than 50% during the preceding 10-year interval (1992–2002). The availability and expansion of vaccination efforts have helped decrease the rates of hepatitis A and hepatitis B. Improvements in the blood supply have dramatically reduced transmission of HCV; the primary means of infection in children is now maternal–infant transmission.
12 - Biliary Atresia and Other Disorders of the Extrahepatic Bile Ducts
- from SECTION II - CHOLESTATIC LIVER DISEASES
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- By William F. Balistreri, M.D., Dorothy M. M. Kersten Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Director, Pediatric Liver Care Center, Department of Pediatric Gastroenterolgy, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Jorge A. Bezerra, M.D., Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Pediatric Gastroenterology, Hepatology, and Nutrition and the Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Ryckman C. Frederick, M.D., Professor, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio; Surgical Director, Liver Transplant Program, Department of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
- Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
-
- Book:
- Liver Disease in Children
- Published online:
- 18 December 2009
- Print publication:
- 07 May 2007, pp 247-269
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Summary
Biliary atresia and related disorders of the biliary tract, such as choledochal cysts, must be considered in the differential diagnosis of prolonged conjugated hyperbilirubinemia in the newborn (neonatal cholestasis). In this chapter, we review the current status of diagnosis and management of these disorders, as well as advances in the intriguing quest for an understanding of their pathogenesis.
OVERVIEW
Neonatal hepatobiliary diseases, including biliary atresia, choledochal cysts, and “idiopathic” neonatal hepatitis, have historically been viewed as a continuum – a gradation of manifestations of a basic underlying disease process in which giant cell transformation of hepatocytes is strongly associated with inflammation at any level of the hepatobiliary tract. These disease entities may be polar end points of a common initial insult, as originally stated in the unifying hypothesis of Landing [1]. The end result represents the sequela of the inflammatory process at the primary site of injury. Landing suggested that this inflammatory process may injure bile duct epithelial cells, leading to either duct obliteration (biliary atresia) or weakening of the bile duct wall with subsequent dilatation (choledochal cyst). The lesions may be dependent on the stage of fetal development when the injury occurs and the site within the developing hepatobiliary tree at which the injury occurs [1, 2]. A relationship of the pathogenesis of these obstructive cholangiopathies of infancy to the process of development is suggested by the association with disorders of situs determination such as the polysplenia syndrome and the observation of the so-called ductal plate malformation within the liver of a few patients with biliary atresia.