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 Citations (Scopus)

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 - Jan 1 1990

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Tyrosinemias
Liver Transplantation
Liver
Ascites
Siblings
Hepatocellular Carcinoma
Autopsy
Fibrosis
Tissue Donors
Neoplasm Metastasis
Biopsy
Lung

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

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title = "Liver cell dysplasia and early liver transplantation in hereditary tyrosinemia.",
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.",
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Liver cell dysplasia and early liver transplantation in hereditary tyrosinemia. / Manowski, Z.; Silver, M. M.; Roberts, E. A.; Superina, R. A.; Phillips, M. J.

In: Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, Vol. 3, No. 6, 01.01.1990, p. 694-701.

Research output: Contribution to journalArticle

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