Tyrosinemia type I treated by NTBC: How does AFP predict liver cancer?

C. J.L. Koelink, P. van Hasselt, A. van der Ploeg, M. M. van den Heuvel-Eibrink, F. A. Wijburg, C. M.A. Bijleveld, F. J. van Spronsen

Onderzoeksoutput: Bijdrage aan tijdschriftArtikelpeer review

54 Citaten (Scopus)


Background: Tyrosinemia type I is associated with an increased risk of liver cancer development. The formation of the pathogenic fumarylacetoacetate is prevented by 2-(2-nitro-4-3 trifluoro-methylbenzoyl)-1,3-cyclohexanedione (NTBC). Still, some patients with NTBC treatment develop liver cancer. A rise of α-fetoprotein (AFP) is an indicator of liver cancer. Aim: To study the predictive value of AFP in tyrosinemia type I patients for the discrimination between patients at high and low risk of liver cancer development. Methods: We examined the course of AFP values of 11 Dutch patients with tyrosinemia type I treated by NTBC, of whom four were diagnosed with liver cancer. Results: The four patients with liver cancer had a course of AFP different from the other patients in either velocity of the decrease of AFP, achieving normal AFP and/or having a rise of AFP concentrations. Conclusion: Apart from a rise of AFP, a slow AFP decrease, and never normalizing levels of AFP are important predictors of liver cancer development in further life.

Originele taal-2Engels
Pagina's (van-tot)310-315
Aantal pagina's6
TijdschriftMolecular Genetics and Metabolism
Nummer van het tijdschrift4
StatusGepubliceerd - dec. 2006
Extern gepubliceerdJa


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