Autoimmune Liver Diseases (AILD)

  • Autoimmune Hepatitis (AIH)

    Autoimmune hepatitis (AIH) is a rare autoimmune liver disease. The immune system of patients with AIH mistakenly targets the body’s own liver cells causing damage and inflammation. AIH can be diagnosed at any age and affects more females than males.…
  • IgG4-related sclerosing cholangitis

  • Isolated neonatal sclerosing cholangitis

  • Primary Biliary Cholangitis (PBC)

    Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a chronic inflammatory autoimmune liver disease, affecting the small intra-hepatic bile ducts. Over time, PBC can lead to fibrosis and eventually to cirrhosis. The term fibrosis refers to the presence…
  • Primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome

  • Primary sclerosing cholangitis (PSC)

    PSC is a rare, immune-mediated liver disease that leads to scarring (sclerosing) following inflammation in the bile ducts (cholangitis). This means that bile, which normally flows through the bile ducts into the intestines, can’t drain properly and it builds up…
  • Secondary sclerosing cholangitis

Metabolic, Biliary Atresia & Related Diseases

  • Alpha-1 Antitrypsin Deficiency (AATD)

    Alpha1-antitrypsin deficiency is an inborn genetic disorder that affects 1 in 2500 individuals. It increases susceptibility to lung disease in adults (emphysema), and liver disease in both children and adults. It is one of the main causes of neonatal cholestasis,…
  • Biliary Atresia

    Biliary atresia is a rare and life-threatening biliary tract disease that occurs exclusively in newborns. It is characterised by an obstruction of the bile ducts. This leads to a back-up of toxic bile (“cholestasis”) that damages the liver and, if…
  • Genetic/ Familial Cholestatic Disease

    Watch here a short educational video on Neonatal Cholestasis: video://neonatal_cholestasis-easl_school.jpg. Short educational video on MDR3 deficiency and cholestatic liver diseases: video://mdr3_deficiency-cholestatic_ld-easl_school.jpg…
    • Alagille Syndrome

    • Congenital bile acid synthesis defect

    • Crigler-Najjar syndrome

    • Disorder of porphyrin and heme metabolism, Bilirubin metabolism disorder

    • Dubin-Johnson syndrome

    • Progressive familial intrahepatic cholestasis (PFIC)

      Bile is essential in order to break down fats and is produced in the liver. In PFIC patients, the transport of bile from the liver to the intestine is disrupted. Therefore, bile acids and in some cases, other metabolites such…
    • Rotor syndrome

  • Low phospholipid associated cholelithiasis (LPAC) syndrome

    © association albi www.albi-france.org Understanding LPAC Syndrome LPAC syndrome is a genetic predisposition to the formation of gallstones in and outside the liver. It manifests itself by intense intermittent abdominal pain in the hepatic region, linked to the presence and/or…
  • Wilson's Disease

    Wilson disease is a rare inherited disorder that causes copper to accumulate in your liver, brain and other vital organs. Most people with Wilson’s disease are diagnosed between the ages of 5 and 35, but it can affect younger and…

Structural Liver Disease

  • Biliary Malformations/ Choledochal malformations (CM)

    Choledochal malformations (CM, previously also called ‘choledochal cysts’ or ‘bile duct cysts’), are congenital malformations of the biliary tract. Bile is secreted by the liver into small ducts that join to form the common bile duct. Between meals, secreted bile…
    • Caroli Disease

  • Polycystic Liver Disease (PLD)/ Congenital Fibrosis

    Polycystic Liver Disease (PLD) is a genetic disease, which means it is caused by mutations in your DNA. Patients with PLD have numerous cysts in their liver; a cyst is a closed sac filled with fluid. The buildup of multiple…
  • Rare liver tumors

    • Fibrolamellar hepatocarcinoma

    • Hepatic epitheloid hemangioendothelioma & Angiosarcoma

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

    • Hepatoblastoma

      Hepatoblastoma is considered to be the most common type of malignant paediatric liver tumour. For instance in a small country as the Netherlands with 17 million inhabitants, this accounts for up to 6 newly diagnosed patients per year. Furthermore, hepatoblastoma…
    • Intrahepatic Cholangiocarcinoma

    • Primary hepatic neuroendocrine tumors (NET)

  • Vascular Liver Disease

    Vascular liver diseases are disorders of the hepatic vasculature that lead to obstruction of blood vessels in and near the liver. Vascular liver diseases (VLD) are rare diseases characterized by a primary involvement of the hepatic veins (Budd-Chiari syndrome: BCS,…
    • Budd-Chiari-Syndrome

      Budd-Chiari syndrome is a condition in which the hepatic veins (veins that drain the liver) are blocked or narrowed by a clot (mass of blood cells). Representation of a Clot in a vessel Clot in hepatic veins or inferior vena…
    • Nodular regenerative hyperplasia of the liver

    • Portal Hypertension

      Early-onset familial non-cirrhotic portal hypertension…
    • Porto-sinusoidal vascular disease (PSVD)

      video://psvd-easl_school.jpg…
    • Sinusoidal obstruction syndrome (SOS) / Hepatic veno-occlusive disease

      Sinusoidal obstruction syndrome, also previously known as hepatic veno-occlusive disease, is a rare condition in which the endothelial cells of the liver sinusoids prolapse into the sinusoid leading to its obstruction. As a consequence the vessel is narrowed, creating an…

Pregnancy in liver disease

  • Contraception and Pregnancy for people with Autoimmune Hepatitis (AIH)

    Autoimmune hepatitis (AIH) belongs to the group of autoimmune liver diseases and usually requires long-term treatment with immunosuppression. AIH is a rare disease and can lead to scar tissue in the liver (called fibrosis and, when more advanced, cirrhosis) and…
  • Contraception and Pregnancy in people with primary biliary cholangitis (PBC)

    Primary biliary cholangitis (PBC) belongs to the group of autoimmune liver diseases. In PBC the immune system causes “irritation” of the bile ducts in the liver which can lead to scar tissue in the liver and rarely poorer liver function.…
  • Pregnancy and breastfeeding among women with Wilson's Disease

    It is well known that patients with Wilson’s disease often experience irregular menstrual cycles, such as missed periods, infrequent periods or irregular cycles. This can make conception difficult. Additionally, in non-treated Wilson’s disease patients miscarriages have been observed. However, this…

Liver disease in pregnancy

  • Acute fatty liver of pregnancy

    Pregnancy related liver disease represents a challenging problem. Often, there is overlap between many of the individual conditions. In general, if any of the following conditions have occurred in one pregnancy, there is a high likelihood of occurrence in a…
  • HELLP syndrome

    Pregnancy related liver disease represents a challenging problem. Often, there is overlap between many of the individual conditions. In general, if any of the following conditions have occurred in one pregnancy, there is a high likelihood of occurrence in a…
  • Intrahepatic cholestasis of pregnancy (ICP)

Rare causes of acute liver failure

  • Acute Liver failure

    Acute liver failure can happen to anyone! Acute Liver failure occurs suddenly. If liver damage develops over a long period of time we speak about chronic liver failure. Here, we speak about Acute Liver Failure. Acute liver failure is relatively…

Transplantation

  • Liver transplantation

    A liver transplant is a major life change. But in the case of various liver diseases, it can become the only chance to live a long time with a good quality of life. Good information then helps to cope with…