Wilson disease:Histopathological correlations with treatment on follow-up liver biopsies

查看全文 作　　者：Sandy Cope-[1]Yokoyama;Milton J [2]Finegold;Giacomo Carlo [3]Sturniolo;Kyoungmi [4]Kim;Claudia [5]Mescoli;Massimo [5]Rugge;Valentina [6]Medici 机构地区：[1]Department of Pathology,University of Texas Southwestern Medical Center and Children's Medical Center;[2]Department of Pathology,Baylor College of Medicine and Texas Children's Hospital;[3]Department of Surgical and Gastroenterological Sciences,University of Padua;[4]Division of Biostatistics,Department of Public Health Science,University of California Davis,One Shields Avenue Davis;[5]Department of Diagnostic Medical Sciences and Special Therapies,Pathology Unit,University of Padua;[6]Division of Gastroenterology and Hepatology,Department of Internal Medicine,University of California Davis Medical Center 出　　处：《World Journal of Gastroenterology》索引2010年第16卷第12期,共8页 摘　　要：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 me-tabolism 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. 关 键 词：Wilson disease Copper Liver biopsy HISTOPATHOLOGY