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Pediatric Liver Disease

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Liver Immunology

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Abstract

Biliary atresia, a progressive fibro-inflammatory process that damages the bile ducts, presents in infancy with cholestasis and pale stools. It is the most common indication for liver transplantation in childhood. Gene identification for several cholestatic liver diseases, including progressive familial intrahepatic cholestasis, Alagille syndrome, and neonatal sclerosing cholangitis, has reduced the number of infants who were previously placed in the category of “idiopathic giant cell hepatitis.” Alpha-1 antitrypsin deficiency is the most common genetic cause of liver disease in children, whereas nonalcoholic fatty liver disease is the most common cause of elevated serum aminotransferases in childhood.

The availability of direct-acting agents for the treatment of hepatitis C in children in recent years has changed the lives of many children and families with chronic hepatitis C.

The definition and etiologies of acute liver failure are different in children compared with adults, with almost a third of indeterminate etiology. Metabolic diseases, such as methylmalonic acidemia and ornithine transcarbamylase (OTC) deficiency, are indications of liver transplantation in children where the liver may structurally be normal but lacks a specific enzyme secondary to a genetic mutation causing progressive multisystemic damage. Autoimmune liver disease in children, including sclerosing cholangitis and de novo autoimmune hepatitis, has unique distinguishing features in childhood compared with adults.

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Authors and Affiliations

  1. Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Rochester Medical Center, Rochester, NY, USA

    Rishi Gupta & Nanda Kerkar

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  1. Rishi Gupta
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  2. Nanda Kerkar
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Correspondence to Nanda Kerkar .

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Editors and Affiliations

  1. Division of Rheumatology, Allergy and Clinical Immunology, The University of California School of Medicine, Davis, CA, USA

    M. Eric Gershwin

  2. Departments of Medicine and Surgery, Baylor College of Medicine, Houston, TX, USA

    John M. Vierling

  3. Department of Medicine, Teikyo University School of Medicine, Itabashi, Tokyo, Japan

    Atsushi Tanaka

  4. President, Hannover Medical School, Hannover, Germany

    Michael P. Manns

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Gupta, R., Kerkar, N. (2020). Pediatric Liver Disease. In: Gershwin, M.E., M. Vierling, J., Tanaka, A., P. Manns, M. (eds) Liver Immunology . Springer, Cham. https://doi.org/10.1007/978-3-030-51709-0_28

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