CLINICAL CURATIVE EFFECTS OF DIMERCAPTOSUCCINIC ACID ON HEPATOLENTICULAR DEGENERATION AND THE IMPACT OF DMSA ON BILIARY TRACE-ELEMENTS

Objective To observe the biliary copper content of nonhepatolenticular degeneration (HLD) controls and changes in the trace elements in the bile, cerebrospinal fluid, blood and urine of hepatolenticular degener ation patients before and after dimercaptosuccinic acid (DMSA) treatme nt in order to further explore the etiological mechanism of HLD and pr ove the therapeutic effect of DMSA on HLD patients. Methods A consecut ive series of 20 patients with HLD were given DMSA orally for 4 weeks. Adult dosage was 1.5 g/day and child dosage 1.0 g/day. Their bile, ce rebrospinal fluid, blood and urine samples were obtained before and af ter treatment with DMSA through duodenal drainage and lumbar puncture, Biliary samples of 22 non-HLD controls were taken by drainage tube af ter surgical operation. Hitachi-208 atom absorption spectrophotometer was used to assay the content of copper, zinc and iron of each sample. Results DR?SA could effectively improve the symptoms such as dysphasi a, salivation, dysphagia and darkening of the skin, limb trembling and myotonia fame second; but it showed no obvious effect on dysstasia, l imb contracture and deformity, and hepatosplenomegaly. It was effectiv e for the patients who were younger and had no obvious hepatic damage. No serious side effects were seen through the course of treatment. La boratory study showed that biliary copper content of HLD patients was evidently lower than that of non-HLD controls (P < 0.01), DMSA could e vidently improve biliary copper excretion besides clearly increase uri nary copper excretion (P < 0.01) and had nothing to do with biliary zi nc excretion (P > 0.05). Conclusions Biliary copper excretion disturba nce participates directly in the pathophysiology of copper retention o f HLD patients. DMSA is a favorable cupruretic drug for patients with HLD.