Fulminant Hepatitis as the First Presentation of Wilson’s Disease

• Published in: Brazilian Journal of Transplantation Vol. 26; no. 1
• Main Authors: Frainer, Djulia Adriani, Welter, Carolina da Silveira, Theis, Claudia, Garcia, Raquel Francine Liermann, Lemos, Ricardo, Haritsch, Franco, Lima, Andre Carminati, Garcia, Christian Evangelista
• Format: Journal Article
• Language: English
• Published: 19-06-2023
• ISSN: 2764-1589; 2764-1589
• DOI: 10.53855/bjt.v26i1.497_ENG

Wilson’s disease is rare, but it affects 6-12% of patients with an indication for urgent liver transplantation. The main manifestations, in addition to the liver, are neurological and psychiatric, with evolution with fulminant hepatitis without neuropsychiatric symptoms being rarer. Despite the urgency, the prognosis for post-transplant patients averages 85% 5-year survival. In this report, we present the case of a female patient, 18 years old, with the onset of abdominal pain, jaundice and choluria that progressed to fulminant hepatitis and the need for urgent liver transplantation. The patient evolved postoperatively with septic shock due to herpetic encephalitis, duodenal ulcer with active bleeding and hepatic artery pseudoaneurysm. Despite measures for stabilization and the request for a transplant again, the patient died.