As a result of the rarity of Wilson disease (WD) and the fact that liver biopsy is not performed routinely during follow-up, unless clinically indicated, the progression and timing of the liver pathology and its correlation with different anti-copper treatments or aminotransferase levels are poorly characterized. Previous studies have demonstrated the possibility of improvement of steatosis and inflammation grade, and of fibrosis stage during long-term follow-up. However, studies on serial liver biopsies, as well as studies on the correlation between hepatic histology and clinical parameters, are lacking.
A research article to be published on March 28 , 2010 in the World Journal of Gastroenterology addresses this question. A study conducted by the University of California Davis (USA), Texas Children's Hospital (USA), and University of Padua (Italy) under the guidance of Dr. Valentina Medici studied the evolution of liver histology in WD patients during penicillamine and zinc treatment, to define the rate of progression of liver damage and to correlate the clinical and biochemical parameters of liver injury with hepatic copper concentration.
The estimated rate of progression of hepatic fibrosis (as result of the mean difference in fibrosis scores divided by the mean interval in years between the first and second liver biopsies) in the entire group was 0 units per year between the first and second liver biopsy, and 0.25 between the second and third. However, among progressors, the rate of progression of fibrosis was estimated at 0.23 fibrosis units per year between the first and second biopsy, and 0.6 units between the second and third. Progressors showed a mean hepatic copper concentration higher than non-progressors at all time points. The histological progression did not correlate with subsequent aminotransferase levels or with type of therapy.
The observation of the inability of clinical tools to detect the progression of fibrosis despite treatment suggests that a liver biopsy with hepatic copper quantification every 3 years should be considered.
Reference: Cope-Yokoyama S, Finegold MJ, Sturniolo GC, Kim K, Mescoli C, Rugge M, Medici V. Wilson disease: Histopathological correlations with treatment on follow-up liver biopsies. World J Gastroenterol 2010; 16(12): 1487-1494 http://www.wjgnet.com/1007-9327/full/v16/i12/1487.htm
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. valentina.medici@ucdmc.ucdavis.edu Telephone: +1-916-7343751 Fax: +1-916-7347908
About World Journal of Gastroenterology
World Journal of Gastroenterology (WJG), a leading international journal in gastroenterology and hepatology, has established a reputation for publishing first class research on esophageal cancer, gastric cancer, liver cancer, viral hepatitis, colorectal cancer, and H pylori infection and provides a forum for both clinicians and scientists. WJG has been indexed and abstracted in Current Contents/Clinical Medicine, Science Citation Index Expanded (also known as SciSearch) and Journal Citation Reports/Science Edition, Index Medicus, MEDLINE and PubMed, Chemical Abstracts, EMBASE/Excerpta Medica, Abstracts Journals, Nature Clinical Practice Gastroenterology and Hepatology, CAB Abstracts and Global Health. ISI JCR 2008 IF: 2.081. WJG is a weekly journal published by WJG Press. The publication dates are the 7th, 14th, 21st, and 28th day of every month. WJG is supported by The National Natural Science Foundation of China, No. 30224801 and No. 30424812, and was founded with the name of China National Journal of New Gastroenterology on October 1, 1995, and renamed WJG on January 25, 1998.
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World Journal of Gastroenterology