用NTBC治疗的I型酪氨酸血症：甲胎蛋白如何预测肝癌？
Tyrosinemia type I treated by NTBC: how does AFP predict liver cancer?
作者信息
Koelink C J L, van Hasselt P, van der Ploeg A, van den Heuvel-Eibrink M M, Wijburg F A, Bijleveld C M A, van Spronsen F J
机构信息
Section of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, 9700 RB Groningen, The Netherlands.
出版信息
Mol Genet Metab. 2006 Dec;89(4):310-5. doi: 10.1016/j.ymgme.2006.07.009. Epub 2006 Sep 27.
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.
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.
背景
I型酪氨酸血症与肝癌发生风险增加相关。2-(2-硝基-4-三氟甲基苯甲酰基)-1,3-环己二酮（NTBC）可防止致病性富马酰乙酰乙酸的形成。然而，一些接受NTBC治疗的患者仍会发生肝癌。甲胎蛋白（AFP）升高是肝癌的一个指标。
目的
研究AFP在I型酪氨酸血症患者中对区分肝癌发生高风险和低风险患者的预测价值。
方法
我们检查了11例接受NTBC治疗的荷兰I型酪氨酸血症患者的AFP值变化过程，其中4例被诊断为肝癌。
结果
4例肝癌患者的AFP变化过程在AFP下降速度、AFP恢复正常和/或AFP浓度升高方面与其他患者不同。
结论
除了AFP升高外，AFP缓慢下降以及AFP水平从未恢复正常是未来发生肝癌的重要预测指标。
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