硫酸锌与青霉胺联合治疗儿童肝豆状核变性  

COMBINED THERAPY OF ZINC SULFATE AND PENICILAMINE WITH HEPATOLENTICULAR DEGENERATION IN CHIL DREN

作　　者：彭春雷[1] 李堂[2] 

机构地区：[1]菏泽医学专科学校药理学教研室,山东省菏泽市274030 [2]青岛医学院附属医院儿内科

出　　处：《齐鲁医学杂志》1998年第3期181-182,共2页Medical Journal of Qilu

摘　　要：1目的　探讨大剂量硫酸锌与小剂量青霉胺联合治疗儿童肝豆状核变性 (HL D)的远期效果。 2方法有症状病儿初期用小剂量青霉胺并大剂量硫酸锌联合治疗 ;病情好转后和症状前期病儿单用硫酸锌维持治疗。对31例病儿进行 4～ 11年随访。 3结果　 3例症状前期病儿未出现异常 ;2 8例有症状病儿 2 3例临床症状基本消失或明显好转 ,2例无改善 ,3例死亡。全部病儿血铜持续低于正常 ;2 4h尿铜于服药后 180 d均较治疗前降低 ,降至正常者 5例 ;服药后 1～ 2年降至正常者 2 6例。疗程中 2 0例有 1次或多次血锌增高 ,2 5例 2 4h尿锌增高。4结论　大剂量硫酸锌与小剂量青霉胺联合应用治疗儿童 HL D效果可靠 。Objective To summarize the long-term effect of combined treatm ent with large-dose zinc sulfate and low- dose penicillamine in children with hepatolenticular degeneration(HL D) . Methods Children with clinical symptoms were treated with large-dose of zinc sulfate and low dose of penicillamine at the beginning of treatment.Only zinc sulfate were used as mainte- nance therapy when clinical improvement was obtained or in the presymptomatic patients from the beginning.A follow-up study was made in 31children for 4— 11years. Results No clinical abnormalities had been found in 3 cases of presymptomatic pa- tients.Among2 8patients with symptoms,clinical symptoms and signs disappeared or much improved in 2 3cases(82 % ) ,un- changed in 2 cases (7% ) ,3 cases died(11% ) .Blood concentrations of copper were lower persistently than norm al.U rine copper excretion of2 4hours was decreased significantly180 days after treatm entin all patients,and became normal in5 cases(16 % ) after 6 months of therapy;normal in 2 6 cases(84% ) after 1— 2 years of treatment.Up shoots of blood zinc concentrations were found in 2 0 cases(6 5 % ) ,and higher urine zine excretion in2 5 cases(81% ) during the therapy. Conclusion Com bined therapy of large- dose zinc sulfate and low-dose penicillamine is an effictive and safe treatment for children with HLD.

关 键 词：肝豆状核变性 硫酸锌 青霉胺 儿童 

分 类 号：R725.8[医药卫生—儿科]