摘要
目的 了解肝豆状核变性(HLD)治疗过程中核磁共振成像(MRI)的动态变化及相关因素。方法对63例HLD患儿行脑及肝MRI常规序列扫描。42/63例显示异常MRI,其中22/42例MRI异常者复查1次/1.0-1.5年,共2-4次结果 10/22例惠儿MRI显示脑和肝异常信号,9/22例脑受累,3/22例肝受累。随访显示,青霉胺剂量不足且初治年龄>15岁者MRI病灶改善缓慢或无变化;停药2个月以上MRI病灶即可重现。恢复治疗后临床和MRI改善缓慢。治疗合理组临床痊愈率和MRI病灶消失率及好转率均明显高于治疗不合理组。结论HLD经合理治疗,肝及脑部MRI异常信号可随着临床改善而渐减甚至消失。治疗不合理者MRI病灶改善缓慢、无变化或重现。MRI有助于HLD疗效及预后的判断。
Objectives To observe the changes of magnetic resonance imaging(MRI) during the course of treatment in hepatolen-ticular degeneration (HLD) and to evaluate its related factors.Methods Sixty - three patients with HLD received routine SE sequence MRI of brain and liver.Twenty - two patients with abnormal MRI in 42 patients and each of them had 2-4 times of serial MRI ex-aminations at interval of 1.0- 1.5 year.Results Ten patients had lesion in both brain and liver in 22 patients. Nine patients only in brain, 3 patients in liver.The major lesions disappear or decrease after rational treatments. There were only slight or no changes of MRI abnormalities in patients receiving treatments or age of patients was over 15 year old. Abnormal MRI findings shown again after stopped treatments over 2 months. In such cases, the clinical and MRI improvements were slow.The rate of clinical and MRI improvement in patients with adequate treatment were markedly higher than patients with inadequate treatment.Conclusions Liver and brain lesion will show in MRI disappear after adequate treatment;There are only slight or no change or showing again in MRI abnormalities in pa-tients with inadequate treatments compared with poor clinical improvement. MRI is beneficial to understanding therapeutic effect and prognosis of HLD.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2004年第12期1070-1072,共3页
Journal of Applied Clinical Pediatrics
关键词
肝豆状核变性
儿童
磁共振成像
动态变化
hepatolentieular degeneretion
children
magnetic resonance imaging
changes
