Literature DB >> 28433112

Wilson disease - liver pathology.

Maciej Pronicki1.   

Abstract

The liver in Wilson disease may demonstrate a wide range of damage patterns. Some patients may present almost no detectable microscopic pathology, while others display lesions consistent with fulminant hepatitis or acute liver failure. Most liver biopsy specimens show moderate to severe steatosis, variable degree of portal and/or lobular inflammation, and fibrosis eventually progressing to cirrhosis. Additional findings include liver cell degeneration and ballooning, Mallory hyaline bodies, liver cell necrosis, and glycogenation of periportal hepatocytic nuclei. None of the above lesions are specific for Wilson disease and should be interpreted in a wider medical context and particular clinical setting. The main message concerning liver pathology is that Wilson disease may be microscopically misinterpreted as many other liver diseases, including viral or autoimmune hepatitis, alcoholic/nonalcoholic steatohepatitis, toxic liver injury, cryptogenic cirrhosis, metabolic liver disease, and many others. The possibility of Wilson disease should be considered in all patients, especially young ones presenting unexplained liver diseases with many variable patterns of microscopic liver involvement.
© 2017 Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Wilson disease; copper deposition; fatty liver disease; hepatitis; hepatolenticular degeneration; liver cirrhosis; liver steatosis; metabolic liver disease

Mesh:

Year:  2017        PMID: 28433112     DOI: 10.1016/B978-0-444-63625-6.00007-0

Source DB:  PubMed          Journal:  Handb Clin Neurol        ISSN: 0072-9752


  3 in total

Review 1.  [Lysosomal acid lipase deficiency (LAL-D) : Diagnostic and therapeutic options in an underdiagnosed disease].

Authors:  S Synoracki; S Kathemann; K W Schmid; H Jastrow; H A Baba
Journal:  Pathologe       Date:  2018-05       Impact factor: 1.011

Review 2.  Wilson disease and the differential diagnosis of its hepatic manifestations: a narrative review of clinical, laboratory, and liver histological features.

Authors:  Shannon M Schroeder; Karen E Matsukuma; Valentina Medici
Journal:  Ann Transl Med       Date:  2021-09

3.  Hepatic copper and other trace mineral concentrations in dogs with hepatocellular carcinoma.

Authors:  Cailin C Harro; Rebecca C Smedley; John P Buchweitz; Daniel K Langlois
Journal:  J Vet Intern Med       Date:  2019-09-07       Impact factor: 3.333

  3 in total

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