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

被引：43

作者：

Koelink, C. J. L.

van Hasselt, P.

van der Ploeg, A.

van den Heuvel-Elbrink, M. M.

Wijburg, F. A.

Bijleveld, C. M. A.

van Spronsen, F. J.

机构：

[1] Univ Groningen, Med Ctr, Beatrix Childrens Hosp, Sect Metab Dis, NL-9700 RB Groningen, Netherlands

[2] Univ Utrecht, Med Ctr, Wilhelmina Childrens Hosp, Dept Metab Dis, Utrecht, Netherlands

[3] Sophia Childrens Univ Hosp, Erasmus Med Ctr, Dept Pediat, Div Metab Dis & Genet, Rotterdam, Netherlands

[4] Univ Amsterdam, Acad Med Ctr, Dept Pediat, NL-1105 AZ Amsterdam, Netherlands

来源：

MOLECULAR GENETICS AND METABOLISM | 2006年 / 89卷 / 04期

关键词：

tyrosinemia type I; alpha-fetoprotein; 2-(2-nitro-4-3 trifluoro-methylbenzoyl)1,3-cyclohexanedione; NTBC; liver cancer; heptocellular carcinoma;

D O I：

10.1016/j.ymgme.2006.07.009

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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. (c) 2006 Elsevier Inc. All rights reserved.

引用

页码：310 / 315

页数：6

共 19 条

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