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目的 :探讨锌盐对肝豆状核变性的远期疗效、用法及长期大剂量锌制剂的不良反应。方法 :96例患儿接受大剂量硫酸锌 (10 0 30 0mg ,一日 3次 ) ,病情严重的 5 2例治疗开始时联合使用低剂量青霉胺 (6 10mg/kg .d) ,症状改善后停作 (平均用药 18个月 ) ,仅用锌维持治疗。随访 5 18年 (平均 9年 10个月 ) ,观察锌的疗效 ,并对锌的不良反应进行监测。结果 :88例(91.7% )有效 ,其中 6 9例治愈或基本治愈 ;6例死亡 (其中 2例自行中断治疗 ,1例死于严重感染 ) ;2例病情无变化。 12例(12 5 % )有消化道反应 ;8例锌维持治疗期间血清铁减低 ,调整饮食结构后自行恢复 ;49例免疫球蛋白和T细胞亚群检测 ,35例甲状腺功能检测均在正常范围。 1/9生长激素激发试验峰值 <10 μg/L ,2 0例姐妹染色单体交换频率 (SCE)与对照组无差异。尿锌排泄量均有增加 ,35例有一次或多次血清锌增高。结论 :锌盐治疗肝豆状核变性有良好效果 ,可作为治疗本病的一线药物 ;锌的用量应较大 ;除少数病例有消化道反应外 ,未发现长期大剂量锌有其他严重不良反应。
Objective: To investigate the long-term curative effect and usage of zinc salt on hepatolenticular degeneration and the adverse reactions of long-term high-dose zinc preparations. Methods: Ninety-six children underwent high-dose zinc sulfate (10 030 mg, 3 times a day) and 52 cases of serious disease were treated with low-dose penicillamine (6 10 mg / kg · d) After stopping (average medication 18 months), only zinc maintenance treatment. The follow-up was performed for 18 to 18 years (average 9 years and 10 months). The curative effect of zinc was observed. Adverse reactions of zinc were monitored. Results: 88 cases (91.7%) were effective, among them, 69 cases were cured or basically cured; 6 cases died (2 cases were interrupted by themselves and 1 case died of serious infection); 2 cases had no change. 12 patients (12 5%) had gastrointestinal reactions; serum iron was decreased in 8 patients during zinc maintenance therapy and self-recovery after adjusting dietary structure; 49 cases of immunoglobulin and T cell subsets, 35 cases of thyroid function tests were in the normal range . 1/9 peak growth hormone test <10 μg / L, 20 cases of sister chromatid exchange frequency (SCE) and the control group no difference. Urinary zinc excretion have increased, 35 cases of one or more serum zinc increased. CONCLUSION: Zinc salt has a good effect on the treatment of Wilson’s disease. It can be taken as the first-line drug for the treatment of this disease. The dosage of zinc should be large. Except for a few cases of gastrointestinal reactions, no serious serious adverse reactions have been found in long-term large doses of zinc .