[Values of serum copper and serum free copper in the diagnosis and monitoring of Wilson's disease and its carriers].
OBJECTIVE
To explore the values of serum copper and serum free copper in the diagnosis of Wilson's disease (WD), its carrier and viral hepatitis and explore the guiding significance of monitoring serum copper in the treatment of WD.


METHODS
A total of 80 WD patients (hepatic type, n = 60; encephalic type, n = 20), 30 carriers, 20 patients with viral hepatitis were enrolled and their levels of serum copper were determined. The neural symptoms were scored by modified Young grade. Hemogram, hepatic functions, blood clotting functions, serum copper and urinary copper were tested throughout all 8 courses of treatment with sodium dimercaptopropane sulfonate (DMPS). The patients were treated with zinc after discharging. All data were analyzed.


RESULTS
The free serum copper increased in the patients with WD (0.17 mg/L ± 0.04 mg/L), carriers (0.13 mg/L ± 0.03 mg/L) and severe viral hepatitis (0.12 mg/L). A slight increase was also observed in the WD carriers. The level of serum copper was correlated with hepatic functions but not with the severity of neural symptoms. The serum copper increased in the patients with no improvement of neural symptoms. However, the serum copper decreased in the WD patients with the improvement of neural symptoms. The serum copper was stabilized at approximately 0.2 mg/L during the long-term treatment period.


CONCLUSIONS
There is auxiliary diagnosis significance of serum copper in the determination of WD. Hepatic functions in hepatic type WD affect the level of serum copper. The serum copper of encephalic type WD can not indicate the severity of neural symptoms. The elevated level of serum copper indicates a poor prognosis. The serum copper is an effective marker in monitoring the development and therapeutic efficacy of the disease.
Department of Neurology, Sun Yat-Sen University, Guangzhou, China.
Xiang-xue
XX
Zhou
Xun-hua
XH
Li
Hai-wei
HW
Huang
Bing
B
Liu
Rong-lan
RL
Zhu
Ying-yin
YY
Liang
Jian-zhong
JZ
Zhu
Xiu-ling
XL
Liang
D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D003300:Copper / Q000097:blood*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000097:blood* / Q000175:diagnosis / Q000188:drug therapy; D006579:Heterozygote; D006801:Humans; D008297:Male; D055815:Young Adult
D003300:Copper