Therapeutic efficacy in 34 patients suffering from hepatolenticular degene-ration with liver disease as its initial manifestation during a 3-year follow-up
CHEN Da-wei
DONG Yi
ZHANG Hong-fei
XU Zhi-qiang
BI Jing-feng
GAN Yu
WANG Fu-chuan
WANG Li-min
ZHU Shi-shu
Abstract：Objective To assess the therapeutic efficacy in patients suffering from hepatolenticular degeneration (HLD) with liver disease as its initial manifestation during a 3-year follow-up, and to investigate its clinical significance. Methods Clinical features and laboratory findings in 34 HLD patients with liver disease as its initial manifestation before treatment and after 3-year follow-up were analyzed retrospectively. Results The total effectiveness rate was 91.2% after 3 years of anti-copper treatment. The laboratory indicators after treatment [ALT 44±33 U/L, AST 43±20 U/L, RBC (4.93±0.72)×1012/L, CHE 4951±899 U/L, serum cop-per 4.5±3.5 μmol/L and 24-hour urinary copper 198±240 μg] were significantly different from those before treatment [ALT 109±83 U/L, AST 97±55 U/L, RBC (4.28±0.56)×1012 /L, CHE 4409±1248 U/L, serum coppe 6.9±2.7 μmol/L and 24-hour urinary copper 680 ±591 μg]. Multiple regression analysis showed that the increase in CHE level in patients with liver cirrhosis was greater than that in patients without liver cirrhosis;the greater the decrease in 24-hour urinary copper, the greater the increase in CHE level after treatment. Conclusions HLD patients with liver disease as its initial manifestation can achieve better therapeutic efficacy with less adverse reactions after long-term anti-copper treatment. The early diagnosis and treatment (including anti-copper treatment) should be the key points to be followed for this kind of patients.
Keywords：hepatolenticular degenerationliver diseasesneurologic manifestationsliver cirrhosismutation
Publication Date：2014-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 223-226 )