Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 310-315 |
| Number of pages | 6 |
| Journal | Molecular Genetics and Metabolism |
| Volume | 89 |
| Issue number | 4 |
| DOIs | |
| Publication status | Published - Dec 2006 |
| Externally published | Yes |
Keywords
- α-fetoprotein
- 2-(2-Nitro-4-3 trifluoro-methylbenzoyl)1,3-cyclohexanedione
- Heptocellular carcinoma
- Liver cancer
- NTBC
- Tyrosinemia type I
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