Department of Pathology, University of Texas Southwestern Medical Center and Children's Medical Center, Dallas, TX 75235, USA.
World J Gastroenterol. 2010 Mar 28;16(12):1487-94. doi: 10.3748/wjg.v16.i12.1487.
To investigate the progression of hepatic histopathology in serial liver biopsies from Wilson disease (WD) patients.
We report a group of 12 WD patients treated with zinc and/or penicillamine who underwent multiple follow-up liver biopsies. Demographic, clinical and laboratory data were gathered and all patients underwent an initial biopsy and at least one repeat biopsy.
Time to repeat biopsy ranged from 2 to 12 years. Six patients (non-progressors) showed stable hepatic histology or improvement. In one case, we observed improvement of fibrosis from stage 2 to 0. Six patients (progressors) had worsening of fibrosis. There was no significant correlation between the histological findings and serum aminotransferases or copper metabolism parameters. The hepatic copper concentration reached normal levels in only two patients: one from the non-progressors and one from the progressors group. The estimated rate of progression of hepatic fibrosis in the entire group was 0 units per year in the time frame between the first and the second liver biopsy (4 years), and 0.25 between the second and the third (3 years). In the progressors group, the rate of progression of liver fibrosis was estimated at 0.11 fibrosis units per year between the first and second biopsy and, 0.6 fibrosis units between the second and third biopsy.
The inability of clinical tools to detect fibrosis progression in WD suggests that a liver biopsy with hepatic copper quantification every 3 years should be considered.
研究肝豆状核变性（WD）患者连续肝活检的肝组织病理学进展。
我们报告了一组 12 例接受锌和/或青霉胺治疗的 WD 患者，他们接受了多次随访肝活检。收集了人口统计学、临床和实验室数据，所有患者均进行了初次活检和至少一次重复活检。
重复活检时间从 2 年到 12 年不等。6 例（非进展者）显示肝组织学稳定或改善。在 1 例中，我们观察到纤维化从 2 期改善到 0 期。6 例（进展者）纤维化加重。组织学发现与血清转氨酶或铜代谢参数之间无显著相关性。只有 2 例患者的肝铜浓度恢复正常：1 例来自非进展者，1 例来自进展者。在整个研究组中，从第一次和第二次肝活检之间（4 年），肝纤维化进展的估计速度为每年 0 个单位，从第二次和第三次肝活检之间（3 年）为 0.25 个单位。在进展者组中，第一次和第二次活检之间肝纤维化的进展速度估计为每年 0.11 个纤维化单位，第二次和第三次活检之间为 0.6 个纤维化单位。
临床工具无法检测 WD 患者的纤维化进展，提示应考虑每 3 年进行一次肝活检和肝铜定量。
