Citation:Žigrai M, Vyskočil M, Tóthová A, Vereš P, Bluska P and Valkovič P (2020) Late-Onset Wilson's Disease.Front. Med. 7:26.

No abstract

No abstract

Summary: Vanishing bile duct syndrome is a type of drug-induced cholestatic liver injury that occurs when the body is exposed to drugs or other foreign substances. We hospitalized a 44-year-old, so far healthy, male with gradually developing painless icterus and pruritus of the whole body after severe polytrauma. The patient had previously undergone a complex treatment (antibio tics, antifungals, parenteral nutrition, LMWH). Laboratory tests revealed a several-fold elevation of total and conjugated bilirubin with a 3-fold increase in ALP levels, no signifi cant elevation of transaminases, a decrease in prothrombin time and IgG4 antibody positivity. An MRCP scan was performed revealing irregular intrahepatic bile ducts, a dilated common bile duct and characteristics of primary or secondary sclerosing cholangitis. Further examinations ruled out infectious, metabolic, toxic and oncological causes of hepatopathy. A liver bio psy ruled out both autoimmune hepatitis and IgG4 hepatopathy and displayed images of “vanishing bile duct syndrome” in drug-induced liver injury (DILI). Empirical treatment with high-dose intravenous N-acetylcysteine and other hepatoprotective agents led to a gradual decrease of bilirubin, normalization of coagulation parameters and remission of icterus and clinical complaints. This case report points to a cholestatic type of drug-induced liver injury with histologically confi rmed vanishing bile duct syndrome and the need to think of DILI as one of the causes of the otherwise unexplained hepatopathy. Key words: cholangitis – DILI – hepatopathy – IgG4 – vanishing bile duct syndrome