title: Tyrosinemia type I treated by NTBC: how does AFP predict liver cancer?
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 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.
authors.affiliation: Section of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, 9700 RB Groningen, The Netherlands.
authors.forename: C J L
authors.initials: CJ
authors.lastname: Koelink
authors.forename: P
authors.initials: P
authors.lastname: van Hasselt
authors.forename: A
authors.initials: A
authors.lastname: van der Ploeg
authors.forename: M M
authors.initials: MM
authors.lastname: van den Heuvel-Eibrink
authors.forename: F A
authors.initials: FA
authors.lastname: Wijburg
authors.forename: C M A
authors.initials: CM
authors.lastname: Bijleveld
authors.forename: F J
authors.initials: FJ
authors.lastname: van Spronsen
mesh_terms: 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
chemical_list: D003512:Cyclohexanones; D004791:Enzyme Inhibitors; D009579:Nitrobenzoates; D000509:alpha-Fetoproteins; C077073:nitisinone