摘要
目的总结肝豆状核变性并肾损害患者临床表现、诊断及治疗。方法对36例儿童肝豆状核变性并肾损害的临床资料进行分析。结果36例患儿肾损害均明显改善,以“急性肾炎”收住院的患儿6例双下肢浮肿减退,36例患儿中28例蛋白尿均消失。结论儿童肝豆状核变性并肾损害起病隐袭易被临床忽视,治疗上以减少铜摄入和吸收,增加铜排泄、维持铜代谢的负平衡及对症治疗为主,预后较好。
Objective To summarize clinical manifestation, diagnosis and treatment of wilson disease complicated with renal impairment. Methods Clinical data of 36 children with wilson disease complicated with renal impairment were analyzed retrospectively. Results Renal impairment in 36 cases was improved obviously. Edema of both lower limbs decreased in six cases misdiagnosed as acute nephritis. Proteinuria disappeared in 28 cases out of 36 cases. Conclusion Wilson disease complicated with renal impairment in children was easy to be neglected due to the insidious onset. The therapy included of reducing copper intake and absorption, increasing copper excretion and maintaining negative balance of copper metabolism for treatment,and the prognosis is relatively better.
出处
《新乡医学院学报》
CAS
2006年第3期285-286,共2页
Journal of Xinxiang Medical University
关键词
肝豆状核变性
肾损害
儿童
hepatolenticular degeneration
renal impairment
children
