文本框: LONG-TERM FOLLOW-UP OF THERAPY WITH LARGE-DOSE ZINC SULFATE IN CHILDREN WITH HEPATOLENTICULAR DEGENERATION LI T, LIN RJ, DU SL Department of Pediatrics, The Affiliated Hospital of Qingdao University Medical College, Qingdao, China Objective: To summarize the long-term effect of therapy with large-dose zinc sulfate in children with hepatolenticular degeneration (HLD). Methods: The patients who had symptoms were treated with large-dose zinc sulfate (100-150mg, <6yr; 150-200mg, 6-8yr; 200-300mg, 9-10yr; 300mg, >10yr; 3 times a day) in addition to low-dose penicillamine (8-10mg/kg/d) at the beginning of treatment. Zinc sulfate alone was given to the presymptomatic patients and it was used as maintenance therapy when clinical improvement was obtained. 36 children were followed up for 4-11 years. Results: In 4 presymptomatic patients, no clinical abnormalities were found. Among 32 patients with symptoms, 25 patients (78.5%) had their symptoms and signs subsided or much improved, 3 patients (12%) remained unchanged, and 4(12.5%) died. Blood concentrations of copper were persistently lower than normal. Urine copper excretion of 24 hours was significantly lower than that before the therapy in all patients, and it became normal in 8 cases (22.2%) after 6 months of treatment, and in 28 cases (77.7%) after 1-2 years of treatment. Higher blood concentrations of zinc were found in 25 cases (69.4%), and higher urine zinc excretion was noted in 30 cases (83.3%) once or more times during the therapy. Conclusion: Therapy of large-dose zinc sulfate is an effective, safe and cheap treatment for children with HLD. 0697