• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利：CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法，用中文像聊天一样搜索，搜遍4000万医学文献。AI智能推荐，让科研检索更轻松。

立即免费搜索

文件翻译

保留排版，准确专业，支持PDF/Word/PPT等文件格式，支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述，25分钟生成高质量综述，智能提取关键信息，辅助科研写作。

立即免费体验

3,4-二氨基吡啶治疗成人兰伯特-伊顿肌无力综合征：随机对照试验的荟萃分析

3,4-diaminopyridine treatment for Lambert-Eaton myasthenic syndrome in adults: a meta-analysis of randomized controlled trials.

作者信息

Zhang Na, Hong Daojun, Ouyang Taohui, Meng Wei, Huang Jingwei, Li Meihua, Hong Tao

机构信息

Department of Neurology, the First Affiliated Hospital of Nanchang University, Jiangxi, China.

Department of Neurosurgery, the First Affiliated Hospital of Nanchang University, No.17, Yongwai Street, Jiangxi Province, 330006, Nanchang, China.

出版信息

BMC Neurol. 2021 Sep 25;21(1):371. doi: 10.1186/s12883-021-02405-3.

DOI:10.1186/s12883-021-02405-3
PMID:34563155
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8464094/
Abstract

BACKGROUND

Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of neuromuscular transmission. The objective was to examine the efficacy and safety of 3,4-diaminopyridine (3,4-DAP) in patients with LEMS.

METHODS

We searched several databases to identify relevant studies, including PubMed, EMBASE, Web of Science, MEDLINE, Cochrane Neuromuscular Disease Group Specialized Register and the Cochrane Central Register of Controlled Trials(CENTRAL). The primary outcome, quantitative myasthenia gravis (QMG) score and the secondary outcome, compound muscle action potentials (CMAP) amplitude were pooled by meta-analysis.

RESULTS

Six randomised controlled trials (RCTs) involving 115 patients with LEMS were included. QMG score showed a significant decrease (improvement) of 2.76 points (95 % CI, -4.08 to -1.45, p < 0.001) after treatment with 3, 4-DAP. Moreover, the overall mean CMAP amplitude improved significantly in LEMS patients with 3, 4-DAP treatment, compared with placebo treatment (mean difference 1.34 mV, 95 % CI, 0.98 to 1.70, p < 0.001). The overall assessment of all included trials showed a low risk of bias and low heterogeneity.

CONCLUSIONS

The pooled results of RCTs demonsrated with moderate to high evidence that 3,4-DAP has a significant effect on LEMS treatment, with improvements in muscle strength score and CMAP amplitude.

摘要

背景

兰伯特-伊顿肌无力综合征（LEMS）是一种罕见的神经肌肉传递自身免疫性疾病。目的是研究3,4-二氨基吡啶（3,4-DAP）治疗LEMS患者的疗效和安全性。

方法

我们检索了多个数据库以识别相关研究，包括PubMed、EMBASE、科学网、MEDLINE、Cochrane神经肌肉疾病组专业注册库和Cochrane对照试验中央注册库（CENTRAL）。主要结局指标重症肌无力定量（QMG）评分和次要结局指标复合肌肉动作电位（CMAP）波幅通过荟萃分析进行汇总。

结果

纳入了6项涉及115例LEMS患者的随机对照试验（RCT）。3,4-DAP治疗后QMG评分显著降低（改善）2.76分（95%CI，-4.08至-1.45，p<0.001）。此外，与安慰剂治疗相比，3,4-DAP治疗的LEMS患者总体平均CMAP波幅显著改善（平均差值1.34mV，95%CI，0.98至1.70，p<0.001）。所有纳入试验的总体评估显示偏倚风险低且异质性低。

结论

RCTs的汇总结果以中等到高度的证据表明，3,4-DAP对LEMS治疗有显著效果，可改善肌肉力量评分和CMAP波幅。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/dfd7181eb044/12883_2021_2405_Fig6_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/537c42044914/12883_2021_2405_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/5cf9159e2b23/12883_2021_2405_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/619d6e65bbd0/12883_2021_2405_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/8d90ede796cf/12883_2021_2405_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/488ad00ab6bd/12883_2021_2405_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/dfd7181eb044/12883_2021_2405_Fig6_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/537c42044914/12883_2021_2405_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/5cf9159e2b23/12883_2021_2405_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/619d6e65bbd0/12883_2021_2405_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/8d90ede796cf/12883_2021_2405_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/488ad00ab6bd/12883_2021_2405_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac5d/8464094/dfd7181eb044/12883_2021_2405_Fig6_HTML.jpg

相似文献

1
3,4-diaminopyridine treatment for Lambert-Eaton myasthenic syndrome in adults: a meta-analysis of randomized controlled trials.3,4-二氨基吡啶治疗成人兰伯特-伊顿肌无力综合征：随机对照试验的荟萃分析
BMC Neurol. 2021 Sep 25;21(1):371. doi: 10.1186/s12883-021-02405-3.
2
Treatment for Lambert-Eaton myasthenic syndrome.兰伯特-伊顿肌无力综合征的治疗。
Cochrane Database Syst Rev. 2011 Feb 16;2011(2):CD003279. doi: 10.1002/14651858.CD003279.pub3.
3
Treatment for Lambert-Eaton myasthenic syndrome.兰伯特-伊顿肌无力综合征的治疗。
Cochrane Database Syst Rev. 2003(2):CD003279. doi: 10.1002/14651858.CD003279.
4
Treatment for Lambert-Eaton myasthenic syndrome.兰伯特-伊顿肌无力综合征的治疗。
Cochrane Database Syst Rev. 2005 Apr 18(2):CD003279. doi: 10.1002/14651858.CD003279.pub2.
5
A randomized trial of 3,4-diaminopyridine in Lambert-Eaton myasthenic syndrome.一项关于3,4-二氨基吡啶治疗兰伯特-伊顿肌无力综合征的随机试验。
Neurology. 2000 Feb 8;54(3):603-7. doi: 10.1212/wnl.54.3.603.
6
Amifampridine for the Management of Lambert-Eaton Myasthenic Syndrome: A New Take on an Old Drug.氨吡啶用于 Lambert-Eaton 肌无力综合征的治疗：老药新用。
Ann Pharmacother. 2020 Jan;54(1):56-63. doi: 10.1177/1060028019864574. Epub 2019 Jul 18.
7
3,4-Diaminopyridine (DAP) in the treatment of Lambert-Eaton myasthenic syndrome (LEMS).3,4-二氨基吡啶（DAP）治疗兰伯特-伊顿肌无力综合征（LEMS）。
Ann N Y Acad Sci. 1998 May 13;841:811-6. doi: 10.1111/j.1749-6632.1998.tb11022.x.
8
Current therapy for Lambert-Eaton myasthenic syndrome: development of 3,4-diaminopyridine phosphate salt as first-line symptomatic treatment.当前 Lambert-Eaton 肌无力综合征的治疗：3,4-二氨基吡啶磷酸盐盐作为一线对症治疗药物的发展。
Curr Med Res Opin. 2010 Jun;26(6):1363-75. doi: 10.1185/03007991003745209.
9
3,4-Diaminopyridine is more effective than placebo in a randomized, double-blind, cross-over drug study in LEMS.在一项针对兰伯特-伊顿肌无力综合征（LEMS）的随机、双盲、交叉药物研究中，3,4-二氨基吡啶比安慰剂更有效。
Muscle Nerve. 2009 Nov;40(5):795-800. doi: 10.1002/mus.21422.
10
Treatment in Lambert-Eaton myasthenic syndrome. Lambert-Eaton 肌无力综合征的治疗。
Ann N Y Acad Sci. 2012 Dec;1275:78-84. doi: 10.1111/j.1749-6632.2012.06769.x.

引用本文的文献

1
Clinical presentations, electrophysiologic features, and long-term follow-up in Lambert-Eaton myasthenic syndrome: a series of six patients.兰伯特-伊顿肌无力综合征的临床表现、电生理特征及长期随访：6例患者系列报道
Front Neurol. 2024 Dec 13;15:1525155. doi: 10.3389/fneur.2024.1525155. eCollection 2024.
2
Foodborne botulism outbreak with potential new management options.食源性肉毒杆菌中毒爆发，具有潜在的新管理选择。
Saudi Med J. 2024 Jun;45(6):626-632. doi: 10.15537/smj.2024.45.6.20240419.
3
The European Lambert-Eaton Myasthenic Syndrome Registry: Long-Term Outcomes Following Symptomatic Treatment.

本文引用的文献

1
Amifampridine Phosphate (Firdapse) Is Effective in a Confirmatory Phase 3 Clinical Trial in LEMS.磷酸阿米芬啶（Firdapse）在一项关于兰伯特-伊顿肌无力综合征的3期确证性临床试验中有效。
J Clin Neuromuscul Dis. 2019 Mar;20(3):111-119. doi: 10.1097/CND.0000000000000239.
2
Lambert-Eaton Myasthenic Syndrome.兰伯特-伊顿肌无力综合征
Neurol Clin. 2018 May;36(2):379-394. doi: 10.1016/j.ncl.2018.01.008.
3
Amifampridine phosphate (Firdapse(®)) is effective and safe in a phase 3 clinical trial in LEMS.磷酸阿米芬啶（Firdapse(®)）在一项针对兰伯特-伊顿肌无力综合征的3期临床试验中有效且安全。
欧洲兰伯特-伊顿肌无力综合征注册研究：对症治疗后的长期结局
Neurol Ther. 2022 Sep;11(3):1071-1083. doi: 10.1007/s40120-022-00354-8. Epub 2022 May 5.
Muscle Nerve. 2016 May;53(5):717-25. doi: 10.1002/mus.25070. Epub 2016 Mar 3.
4
The European LEMS Registry: Baseline Demographics and Treatment Approaches.欧洲 LEMS 登记处：基线人口统计学和治疗方法。
Neurol Ther. 2015 Dec;4(2):105-24. doi: 10.1007/s40120-015-0034-0. Epub 2015 Nov 2.
5
Voltage gated calcium channel antibody-related neurological diseases.电压门控钙通道抗体相关神经系统疾病
World J Clin Cases. 2015 Mar 16;3(3):293-300. doi: 10.12998/wjcc.v3.i3.293.
6
Long-term follow-up in infantile-onset lambert-eaton myasthenic syndrome.婴儿期起病的兰伯特-伊顿肌无力综合征的长期随访
J Child Neurol. 2014 Sep;29(9):NP58-61. doi: 10.1177/0883073813499970. Epub 2013 Oct 10.
7
Treatment in Lambert-Eaton myasthenic syndrome. Lambert-Eaton 肌无力综合征的治疗。
Ann N Y Acad Sci. 2012 Dec;1275:78-84. doi: 10.1111/j.1749-6632.2012.06769.x.
8
Long-term disease history, clinical symptoms, health status, and healthcare utilization in patients suffering from Lambert Eaton myasthenic syndrome: Results of a patient interview survey in Germany.患有 Lambert-Eaton 肌无力综合征患者的长期疾病史、临床症状、健康状况和医疗保健利用情况：德国患者访谈调查结果。
J Med Econ. 2012;15(3):521-30. doi: 10.3111/13696998.2012.660897. Epub 2012 Feb 21.
9
Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies. Lambert-Eaton 肌无力综合征：从临床特征到治疗策略。
Lancet Neurol. 2011 Dec;10(12):1098-107. doi: 10.1016/S1474-4422(11)70245-9.
10
Treatment for Lambert-Eaton myasthenic syndrome.兰伯特-伊顿肌无力综合征的治疗。
Cochrane Database Syst Rev. 2011 Feb 16;2011(2):CD003279. doi: 10.1002/14651858.CD003279.pub3.