Tyrosinemia type I treated by NTBC: how does AFP predict liver cancer?
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 alpha-fetoprotein (AFP) is an indicator of liver cancer.


OBJECTIVE
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.


CONCLUSIONS
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.
Koelink
C J L
CJ
Section of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, 9700 RB Groningen, The Netherlands.
van Hasselt
P
P
van der Ploeg
A
A
van den Heuvel-Eibrink
M M
MM
Wijburg
F A
FA
Bijleveld
C M A
CM
van Spronsen
F J
FJ
D002648:Child; D002675:Child, Preschool; D003512:Cyclohexanones / Q000627:therapeutic use*; D004791:Enzyme Inhibitors / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000209:etiology*; D008297:Male; D009579:Nitrobenzoates / Q000627:therapeutic use*; D011379:Prognosis; D020176:Tyrosinemias / Q000150:complications* / Q000188:drug therapy*; D000509:alpha-Fetoproteins / Q000032:analysis
D003512:Cyclohexanones; D004791:Enzyme Inhibitors; D009579:Nitrobenzoates; D000509:alpha-Fetoproteins; C077073:nitisinone