Brief Article
Copyright ©2010 Baishideng.
World J Gastroenterol. Mar 28, 2010; 16(12): 1487-1494
Published online Mar 28, 2010. doi: 10.3748/wjg.v16.i12.1487
Table 3 Review of the case series describing the evolution of hepatic histology in WD
Grand et al[18], 1975Treatment: PCA
Interbiopsy interval 2-7 yrHepatic copper (μg/g dry weight)AST/ALTHistopathology
Age at diagnosis (yr)BeforeAfterBeforeAfterBeforeAfter
124NA400NANAInflammation 3+; connective tissue 13%; fatty vacuolization 0.5%Inflammation 1+; connective tissue 7%; fatty vacuolization 0.5%
218NA80NANAInflammation 2+; connective tissue NA; fatty vacuolization NAInflammation 2+; connective tissue 3%; fatty vacuolization 2%
3111360757NANAInflammation 3+; connective tissue 17.6%; fatty vacuolization 5%Inflammation 0/1+; connective tissue 7.6%; fatty vacuolization 3%
413.5111290NANAInflammation 4+; connective tissue 16%; fatty vacuolization 13%Inflammation 0; connective tissue 14%; fatty vacuolization 8%
Sternlieb et al[19], 1976Treatment: PCA
Interbiopsy interval 3-5 yrHepatic copper (μg/g dry weight)AST/ALTHistopathology
Age at diagnosis (yr)BeforeAfterBeforeAfterBeforeAfter
11582110952/5939/20Mild fibrosis; steatosisMild fibrosis; marked diminution of steatosis
29100494576/13620/44SteatosisMarked diminution of steatosis
415112323922/9416/22Severe steatosisResolution of severe steatosis
51083245357/8853/48Inflammation; mild fibrosis; steatosisResolution of inflammation; mild fibrosis; diminution of steatosis
6121177105065/8545/43Inflammation; severe fibrosis and steatosisResolution of inflammation; diminution of severe steatosis and fibrosis
714NANA75/8250/35Inflammation; steatosis; cirrhosisDiminution of steatosis and inflammation; persistence of cirrhosis
Shiono et al[20], 2001Treatment: PCA
Interbiopsy interval 3-8.5 yrHepatic copper (mg/g dry weight)ALT (IU/L)Histopathology (only stage of fibrosis)
Age at diagnosis (yr)BeforeAfterBeforeAfterBeforeAfter
11699031912165Cirrhosis with chronic active hepatitisCirrhosis
2171025200241109Chronic active hepatitisChronic inactive hepatitis
Marcellini et al[12], 2005Treatment: zinc sulfate
Interbiopsy interval 10 yrHepatic copper (median of 22 pts) (mg/g dry weight)AST/ALT (mean of 22 pts)Histopathology summary of 22 pts
Mean age at diagnosis (yr)873 (670-982)690 (600-890)110/9421.7/23.7Inflammation grade 1 in 81% of pts, grade 0 in 19%; steatosis grade 1 in 50%, grade 2 in 22.7%, grade 3-4 in 27.3%; fibrosis stage 1 in 54%, stage 3 in 46%Resolution of inflammation in all pts; steatosis grade 1 in 90%, grade 2 in 10%; fibrosis stage 1 in 81%, stage 3 in 19%
6.1 ± 2.5
Askari et al[13], 2003Treatment: trientine + zinc, followed by long-term zinc
Interbiopsy interval 4.4-10 yrHepatic copperChild Pugh score (AST/ALT not available)Histopathology (only stage of fibrosis)
Mean age at diagnosis (yr)NANA9-135CirrhosisFibrosis stage 2-3
25.4 ± 3.9CirrhosisFibrosis stage 3-4
CirrhosisFibrosis stage 1
Linn et al[14], 2009Treatment: zinc
Interbiopsy intervalHepatic copper (mg/g dry weight)ALT (U/L)Histopathology
3-7 yr
Age at diagnosis (yr)BeforeAfterBeforeAfterBeforeAfter
121NANA5957Mild fibrosisNormal