The DOI matches its Crossref record; 2 of the 2 references it cites were found in a registry or an index.
Persistent succinylacetone excretion after liver transplantation in a patient with hereditary tyrosinaemia type I
Abstract
A liver transplant was performed on a 4‐year‐old female in liver failure caused by hereditary tyrosinaemia, with hepatocellular carcinoma following a negative evaluation for metastases. However, serum alpha‐fetoprotein levels never returned to normal after the surgery. Urinary succinylacetone (SA) was detected in her urine prior to transplantation despite strict adherence to a low‐tyrosine diet. Other patients with severe liver disease awaiting liver transplantation do not excrete SA in the urine. She continued to excrete SA during the postoperative period despite normal liver functions. Oral tyrosine loading resulted in significant elevation of SA excretion. Possible explanations for this observation and clinical and therapeutic relevance are discussed.
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Mice heterozygous for either mutation do not exhibit an increase in SA levels, confirming that a rise in SA accompanies the disease state.
“Mice heterozygous for either mutation do not exhibit an increase in SA levels, confirming that a rise in SA accompanies the disease state (40).”
From Point mutations in the murine fumarylacetoacetate hydrolase gene: Animal models for the human genetic disorder hereditary tyrosinemia type 1 (2001) · cites this paper as [40]
Citations
Cites
On the enzymic defects in hereditary tyrosinemia.
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Sassa, S., & Kappas, A. (1983) · Journal of Clinical Investigation · doi:10.1172/jci110809 · Cited by 145
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Research works printed but not linked: 14
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- Edwards S. W., 1955, Fumarylacetoacetate hydrolase, Meth. Enzymol., 2, 298
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Grompe, M., al-Dhalimy, M., Finegold, M., Ou, C. N., Burlingame, T., Kennaway, N. G., & Soriano, P. (1993) · Genes & Development · doi:10.1101/gad.7.12a.2298 · Cited by 355
Aponte, J. L., Sega, G. A., Hauser, L. J., Dhar, M. S., Withrow, C. M., Carpenter, D. A., Rinchik, E. M., Culiat, C. T., & Johnson, D. K. (2001) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.98.2.641 · Cited by 68
Metadata
- Authors
- Tuchman, M., Freese, D. K., Sharp, H. L., Whitley, C. B., Ramnaraine, M. L., Ulstrom, R. A., Najarian, J. S., Ascher, N., Buist, N. R. M., & Terry, A. B.
- Affiliations
- University of Minnesota (US)
- Oregon Health & Science University (US)
- ORCID
- Ascher, N.
- Venue
- Journal of Inherited Metabolic Disease
- Publisher
- Wiley
- Published
- 1984-06-21
- Volume
- 8
- Issue
- 1
- Pages
- 21-24
- ISSN
- 0141-8955 (ISSN-L)
- 1573-2665
- OpenAlex
- W2169079792
- Cited by
- 22
- References
- 2
- Citations by year
- 2021: 1 · 2013: 2
- FWCI
- 0.68
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of Inherited Metabolic Disease (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (P01AM013083)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Amino Acid Enzymes and Metabolism
- Health Sciences › Medicine › Surgery › Organ Transplantation Techniques and Outcomes
- Keywords
- succinylacetone, liver transplantation, hepatocellular carcinoma, alpha-fetoprotein, pediatric patients
- MeSH terms
- alpha-Fetoproteins, Child, Preschool, Female, Carcinoma, Hepatocellular, Heptanoates, Heptanoic Acids, Humans, Hydrolases, Liver Neoplasms, Tyrosine, Liver Transplantation
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