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近十年来大量研究证明,口服硫酸锌能抑制小肠对铜的吸收,它可替代D-青霉胺作为成人和小儿肝豆状核变性(Wilson 病)的长期维持疗法。目前,大多开始时给病人用D-青霉胺排出多余的铜,然后主要用口服锌;仅少数报道从开始就用口服锌治疗。我们在1例Wilson 病儿,从病程早期首选口服硫酸锌治疗,并作为唯一的治疗方法。病例8岁、男孩,无神经系统症状,外表看来很健康,其姐死于不明原因的急性肝功衰竭。患儿SGOT、SGPT 和SGGT 都升高,肝明显增大,脾中等大小,血浆铜和血清铜蓝蛋白基本正常,无K-F
A large number of studies over the past decade have demonstrated that oral administration of zinc sulfate inhibits copper absorption in the small intestine and is an alternative to D-penicillamine as a long-term maintenance therapy for Wilson’s disease in adults and children. Most of the time, most of the time, D-penicillamine is used to excrete excess copper and then mainly to oral zinc; only a few are reported to be treated with oral zinc from the outset. We are in a Wilson disease in children, preferred early oral zinc sulfate treatment, and as the only treatment. Case 8 years old, boy, no neurological symptoms, looks very healthy, his sister died of unexplained acute liver failure. Children SGOT, SGPT and SGGT were elevated, liver increased significantly, spleen size, plasma copper and serum ceruloplasmin were normal, no K-F