Understand your Diagnosis

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 …
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IgG4-related sclerosing cholangitis
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Isolated neonatal sclerosing cholangitis
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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 …
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Primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome
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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 …
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Secondary sclerosing cholangitis
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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. …
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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 …
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Genetic/ Familial Cholestatic Disease
Watch here a short educational video on Neonatal Cholestasis: Short educational video on MDR3 deficiency and cholestatic liver diseases: …
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Alagille Syndrome
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Congenital bile acid synthesis defect
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Crigler-Najjar syndrome
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Disorder of porphyrin and heme metabolism, Bilirubin metabolism disorder
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Dubin-Johnson syndrome
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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. …
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Rotor syndrome
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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 …
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Wilson 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 disease are diagnosed between the ages …
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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 …
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Caroli Disease
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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 …
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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
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 …
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Intrahepatic Cholangiocarcinoma
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Primary hepatic neuroendocrine tumors (NET)
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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 …
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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 …
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Nodular regenerative hyperplasia of the liver
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Portal Hypertension
Early-onset familial non-cirrhotic portal hypertension …
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Porto-sinusoidal vascular disease (PSVD)
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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 …
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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 …
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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 …
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Intrahepatic cholestasis of pregnancy (ICP)
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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 …
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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 …
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Pregnancy and breastfeeding among women with Wilson 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 …
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Metabolic, Biliary Atresia & Related Diseases
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 …
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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 …
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Tips
Tips how to take your medication regularly
TIPS TO INTEGRATE THE MEDICATION IN DAILY LIFERoutines• Try to take your pills at the same times every day.• Try to associate taking your medication with an activity of your …
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