近日，美国食品和药物管理局（FDA）已经批准Firdapse（amifampridine 中文译名：阿米法普利）10mg片剂用于治疗成人Lambert-Eaton肌无力综合征（LEMS）。
批准日期：2018年11月29日 公司：Catalyst Pharmac.icals，Inc
FIRDAPSE(阿米法普利[amifampridine])片剂，供口服使用
首次美国批准：2018
作用机理
阿米法普利定治疗LEMS的机制尚未完全阐明。氨氟普利定是一种广谱钾通道阻断剂.
适应症及用法
FIRDAPSE是一种钾通道阻滞剂，用于成人Lambert-Eaton肌无力综合征（LEMS）的治疗。
剂量与给药
推荐起始剂量是每天口服15毫克到30毫克（每日3到4次）。
对于肾功能损害、肝功能损害和已知N-乙酰转移酶2(NAT2)代谢不良的患者，起始剂量是每天15mg。
每天每3～4天可增加剂量5mg。
每日剂量不得超过80mg。
最大单次剂量为20mg。
剂型和强度
药片：10mg，功能评分。
禁忌症
FieldAPSE禁忌患者：
癫痫发作史对氨氟吡啶或另一氨基吡啶过敏。
警告和注意事项
癫痫发作：癫痫发作可引起癫痫发作。考虑在治疗期间癫痫患者停止或减少FIRDAPSE的剂量。
过敏反应：如果发生过敏反应，如过敏反应，应停止FIRDAPSE，并开始适当的治疗。
AdvE E反应
最常见的不良反应有：感觉异常、上呼吸道感染、腹痛、恶心、腹泻、头痛、肝酶升高、背痛、高血压和肌肉痉挛。
为了报告可能的ADVERSE反应，请联系CatalystPharmac.icals 1-844-347-3277(1-844-FIRDAPSE)或FDA 1-800-FDA-1088或www.fda.gov/med.。
药物相互作用
降低癫痫发作阈值的药物：同时使用FIRDAPSE和降低癫痫发作阈值的药物可能导致癫痫发作。具有胆碱能作用的药物：同时使用FIRDAPSE和具有胆碱能作用的药物（例如，直接或间接胆碱酯酶抑制剂）可增加FIRDAPSE和软管药物的胆碱能作用，并增加不良反应的风险。
特定人群使用
怀孕：基于动物数据，可能会造成胎儿伤害。
包装供应/储存和搬运
供应
FiLDAPSE 10毫克片白色至白色，圆形，并功能得分。每块药片在未打分的那一边折旧，而药片在打分时可以分成两半。
FiLDAPSE提供如下：
儿童抵抗Blister Pack
泡罩包装含10片NDC 69616-211-04
纸箱含12个泡罩包装（总共120片）NDC 69616-211-06
瓶:
60片:NDC 69616-211-08
240片:NDC 69616-211-03
储运
将FIRDAPSE片在20℃至25℃（68°F至77°F）下储存，允许偏离15°C至30°C（59°F至86°F）[见USP控制室温]。
完整资料附件：https://www.firdapse.com/pdf/firdapse-pi.pdf

U.S. Food and Drug Administration (FDA) has approved Firdapse(amifampridine)10mg tablets for the treatment of adults with Lambert-Eaton Myasthenic Syndrome (LEMS).
About Lambert-Eaton Myasthenic Syndrome (LEMS)
LEMS, is a rare autoimmune disorder, most often characterized by fatigable limb muscle weakness.  The disease is caused by autoantibodies against voltage-gated calcium channels located in the nerve-muscle junction, resulting in improper nerve-muscle communication, leading to progressive muscle weakness, when left untreated.  In approximately 50% of cases, LEMS is associated with an underlying malignancy, most commonly small cell lung cancer, and in some individuals, LEMS is the first symptom of such malignancy. 
LEMS generally affects the extremities, especially the legs.  In early course of the disease, muscles closest to the trunk are affected, which are manifested as difficulties with climbing stairs or rising from a sitting position.  Physical exercise and high temperatures tend to worsen the symptoms.  Other symptoms occasionally seen include weakness of the muscles of the mouth, throat, and eyes.  Individuals affected with LEMS also may have a disruption of the autonomic nervous system, with symptoms of dry mouth, dry skin, constipation, blurred vision, impaired sweating, and/or inappropriate blood pressure variations.
About Firdapse® (amifampridine)
Firdapse® (amifampridine) 10mg tablets is an oral, nonspecific, voltage-dependent, potassium(K+)channel blocker that causes depolarization of the presynaptic membrane and slows or inhibits repolarization. This action results in the opening of slow voltage-dependent calcium (Ca2+) channels, allowing for a subsequent influx of Ca2+. In turn, it induces the exocytosis of synaptic vesicles containing Acetylcholine(ACh)to release more ACh into the synaptic cleft, enhancing neuromuscular transmission, and providing for improved muscle function. Firdapse is approved in the U.S. and the European Union for use by patients with LEMS.