Fulminant Wilsonian hepatitis unmasked by disease progression: report of a case and review of the literature.
Among various hepatic manifestations of Wilson's disease, fulminant hepatic failure is the most uncommon entity and requires a detailed clinicopathological analysis for correct diagnosis. Left unrecognized and without proper therapy, in time the disease rapidly progresses to death. We describe a 24-year-old woman who died within five weeks of the onset of Wilson's disease, which presented with a dramatic course. Discriminating features of the disease are discussed with regard to the literature.
Ferlan-Marolt
V
V
Institute of Pathology, Medical Faculty, Ljubljana, Slovenia.
Stepec
S
S
D000328:Adult; D002570:Ceruloplasmin / Q000032:analysis; D003300:Copper / Q000097:blood; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006501:Hepatic Encephalopathy / Q000175:diagnosis* / Q000209:etiology* / Q000473:pathology; D006505:Hepatitis / Q000150:complications* / Q000473:pathology; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000473:pathology; D006801:Humans; D007687:Kidney Tubules, Proximal / Q000473:pathology
D003300:Copper; D002570:Ceruloplasmin