Brief Article
Copyright ©2010 Baishideng. All rights reserved
World J Gastroenterol. Mar 28, 2010; 16(12): 1487-1494
Published online Mar 28, 2010. doi: 10.3748/wjg.v16.i12.1487
Wilson disease: Histopathological correlations with treatment on follow-up liver biopsies
Sandy Cope-Yokoyama, Milton J Finegold, Giacomo Carlo Sturniolo, Kyoungmi Kim, Claudia Mescoli, Massimo Rugge, Valentina Medici
Sandy Cope-Yokoyama, Department of Pathology, University of Texas Southwestern Medical Center and Children’s Medical Center, Dallas, TX 75235, United States
Milton J Finegold, Department of Pathology, Baylor College of Medicine and Texas Children’s Hospital, Houston, TX 77030, United States
Giacomo Carlo Sturniolo, Department of Surgical and Gastroenterological Sciences, University of Padua, 35128 Padua, Italy
Kyoungmi Kim, Division of Biostatistics, Department of Public Health Science, University of California Davis, One Shields Avenue Davis, CA 95616, United States
Claudia Mescoli, Massimo Rugge, Department of Diagnostic Medical Sciences and Special Therapies, Pathology Unit, University of Padua, 35100 Padua, Italy
Valentina Medici, Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of California Davis Medical Center, CA 95817, United States
Author contributions: Cope-Yokoyama S and Finegold MJ performed the histopathological analysis, made a major contribution to the data analysis and interpretation, and manuscript preparation; Sturniolo GC provided the patients and contributed to the manuscript preparation; Kim K performed the statistical analysis and contributed to the manuscript preparation; Mescoli C and Rugge M provided the slides from the patients; Medici V designed the study, analyzed and interpreted the data, and wrote the manuscript.
Correspondence to: Valentina Medici, MD, Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of California Davis Medical Center, 4150 V Street, Suite 3500, Sacramento, CA 95817, United States.
Telephone: +1-916-7343751 Fax: +1-916-7347908
Received: November 25, 2009
Revised: December 29, 2009
Accepted: January 5, 2010
Published online: March 28, 2010

AIM: To investigate the progression of hepatic histopathology in serial liver biopsies from Wilson disease (WD) patients.

METHODS: 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.

RESULTS: 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.

CONCLUSION: 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.

Keywords: Wilson disease, Copper, Liver biopsy, Histopathology