Early liver transplantation is indicated for tyrosinemia type I

Deborah K. Freese, Mendel Tuchman, Sarah J Schwarzenberg, Harvey L. Sharp, Jeffrey M. Rank, Joseph R. Bloomer, Nancy L. Ascher, William D Payne

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36 Citations (Scopus)

Abstract

Liver transplantation is now accepted as the treatment of choice for tyrosinemia type I (hereditary tyrosinemia). In an effort to determine whether any factors in these patients would aid in predicting optimal timing of the transplant procedure, we evaluated several clinical, biochemical, and radiographic parameters in five successive patients undergoing liver transplant for tyrosinemia type I at the University of Minnesota. All five patients evidenced prolonged periods of clinical and metabolic stability with dietary therapy and four of five remained stable at the time of evaluation for transplantation. Nevertheless, all five suffered significant and unexpected complications of tyrosinemia prior to the time of liver transplant. Four developed renal stones, two were in liver failure, and one developed a neurologic crisis that left him completely paralyzed. Hepatocellular carcinoma was found in one of the five at transplant. We could identify no clinical, biochemical, or radiographic study that was predictive of the likelihood of significant complications of the disorder. Survival from the transplant procedure itself was 100%. The inability to predict or prevent significant complications of tyrosinemia and the favorable outcome from transplantation lead us to recommend liver transplant for all patients with tyrosinemia type I by 12 months of age.

Original languageEnglish (US)
Pages (from-to)10-15
Number of pages6
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume13
Issue number1
DOIs
StatePublished - Jan 1 1991

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Tyrosinemias
Liver Transplantation
Transplants
Liver
Transplantation
Liver Failure
Nervous System
Hepatocellular Carcinoma
Kidney
Survival
Therapeutics

Keywords

  • Hepatocellular carcinoma
  • Liver transplantation
  • Metabolic liver disease
  • Tyrosinemia type I

Cite this

Early liver transplantation is indicated for tyrosinemia type I. / Freese, Deborah K.; Tuchman, Mendel; Schwarzenberg, Sarah J; Sharp, Harvey L.; Rank, Jeffrey M.; Bloomer, Joseph R.; Ascher, Nancy L.; Payne, William D.

In: Journal of Pediatric Gastroenterology and Nutrition, Vol. 13, No. 1, 01.01.1991, p. 10-15.

Research output: Contribution to journalArticle

Freese, Deborah K. ; Tuchman, Mendel ; Schwarzenberg, Sarah J ; Sharp, Harvey L. ; Rank, Jeffrey M. ; Bloomer, Joseph R. ; Ascher, Nancy L. ; Payne, William D. / Early liver transplantation is indicated for tyrosinemia type I. In: Journal of Pediatric Gastroenterology and Nutrition. 1991 ; Vol. 13, No. 1. pp. 10-15.
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