Liver cell dysplasia and early liver transplantation in hereditary tyrosinemia.

Z. Manowski*, M. M. Silver, E. A. Roberts, R. A. Superina, M. J. Phillips

*Corresponding author for this work

Research output: Contribution to journalArticle

25 Scopus citations

Abstract

Two cases of hereditary tyrosinemia presented with ascites and coagulopathy in infancy. Both patients underwent liver transplantation at the age of 25 and 36 mo, respectively. Both cases had normal liver function 37 and 24 mo later. The native liver in each case showed mixed micro- and macronodular cirrhosis with hepatocellular dysplasia, including both the large and small cell varieties. One of the subjects had also shown dysplasia in a prior liver biopsy. We compared the hepatic morphology with that from two other cases from our autopsy files. One of these (a female, 9 mo old) showed dysplasia, and the other (her male sibling, 4 yr old) had a liver cell carcinoma with lung metastases. These observations confirm prior reports that neoplastic transformation occurs early in the natural history of hereditary tyrosinemia despite meticulous dietary management and other supportive treatment. With the detection of liver cell dysplasia, efforts should be intensified to find an appropriate donor. Liver transplantation cures the hepatic disease and should be performed before malignancy develops.

Original languageEnglish (US)
Pages (from-to)694-701
Number of pages8
JournalModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
Volume3
Issue number6
StatePublished - Nov 1990

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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