Autoimmune Liver Diseases (AILD)

  • Autoimmune Hepatitis (AIH)

    Autoimmune Hepatitis (AIH) is a chronic liver disease characterised by increased serum transaminases and immunoglobulin G, circulating autoantibodies and infiltration of immune cells on liver histology. Its pathogenesis is largely unknown, but an autoimmune reaction directed against hepatocytes is assumed.…

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  • Primary Biliary Cholangitis (PBC, formerly Primary Biliary Cirrhosis)

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  • Sclerosing cholangitis

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    • IgG4-related sclerosing cholangitis

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    • Primary sclerosing cholangitis (PSC)

      PSC is a chronic cholestatic hepatobiliary disease that has an incidence of approximately 1 case per 100,000 persons/year. Two-third of patients are male, and of middle-age. The pathogenesis is not well understood, although immunological mechanisms and the gut-liver axis seems…

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Metabolic, Biliary Atresia & Related Diseases

  • Alpha-1 Antitrypsin Liver Disease (AATD)

    AATD is a common inherited genetic condition that increases the risk of lung and liver disease. The prevalence of the severe forms is approximately 1 in 2500 individuals. The disease results from a mutation leading to the production of a…

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  • Biliary Atresia

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  • Genetic Cholestatic Disease

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  • Wilson Disease

    Wilson disease (WD) is a rare autosomal recessive genetic disorder of hepatic copper metabolism. In WD the biliary copper excretion is impaired leading to is accumulation, primary in the liver, and then, when the liver’s capacity for copper storage is…

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Structural Liver Disease

  • Biliary Malformations

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  • Polycystic Liver Disease (PLD)/ Congenital Fibrosis

    Polycystic liver disease (PLD) is characterized by the formation of fluid-filled cysts throughout the liver. It can occur as an isolated form in autosomal dominant polycystic liver disease (ADPLD), which was formerly abbreviated as polycystic liver disease (PCLD). It can…

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  • Rare liver tumors

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    • Fibrolamellar hepatocarcinoma

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    • Hepatic epitheloid hemangioendothelioma & Angiosarcoma

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    • Hepatobiliary tumors with stem cell features & mixed types (CCA/HCC)

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    • Hepatoblastoma

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    • Intrahepatic Cholangiocarcinoma

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    • Primary hepatic neuroendocrine tumors (NET)

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  • Vascular Liver Disease

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    • Budd-Chiari-Syndrome

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    • Early-onset familial non-cirrhotic portal hypertension

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    • Nodular regenerative hyperplasia of the liver

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Liver disease in pregnancy

  • General information

    This theme will focus on data acquisition and development of guidelines for management of rare gestational liver diseases (acute fatty liver of pregnancy; HELLP syndrome; severe, early-onset intrahepatic cholestasis of pregnancy) and pregnancy in women with rare liver diseases (cirrhosis…

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Rare causes of acute liver failure

Clinical practice guidelines: Links

Below you will find specific information on the diseases covered by our three pillars. In addition, we provide links to the clinical practice guideline pages of the professional societies:

European Association of the Liver (EASL)

https://easl.eu/publications/clinical-practice-guidelines/

European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)

https://espghan.info/