英文药名：Zemaira（Alpha 1-Proteinase Inhibitor[Human]）

中文药名：阿尔法1-人蛋白酶冻干粉

生产厂家：CSL Behring公司
药品介绍：
Zemaira®，阿尔法1- 蛋白酶抑制剂（人）冻干粉 供A1-PI缺​​乏和肺气肿静脉使用
美国首次批准：2003  公司：CSL Behring公司
适应症和用法
Zemaira是α1-蛋白酶抑制剂（A 1-PI）在成人以A1-PI缺​​乏和肺气肿的临床证据表明慢性增强和维持治疗。
增强治疗Zemaira或任何A 1-PI产物对肺加重和肺气肿中的A1-PI缺​​乏症的进展的影响还没有被证实在随机对照临床研究。
Zemaira不指示为治疗肺部疾病的患者中的人严重的A1-PI不足尚未建立。
用法用量
对于重组后静脉注射只使用。
Zemaira的每周推荐剂量为60毫克/公斤体重。使用剂量疗效终点尚未与Zemaira或A1-PI的产品完成范围研究。
重建后3小时内施用在室温下。
不要与其他医药产品。管理通过一个单独的专用注入管线。
管理通过合适的5微米输液过滤器（未提供）以大约0.08毫升/千克/分的速度作为由患者的响应性和舒适性来确定。
密切监测输液速度和患者的临床状态，包括生命体征，在整个输注。减缓或停止输液，如果出现不良反应。如果症状消退及时，输液可以以较低的速率是舒适的患者被恢复。
剂型和规格
Zemaira在含有约1000毫克的功能活性的A1-PI（每小瓶的测定量被印刷在小瓶标签和纸箱上）的作为用于重构冻干粉末用20ml无菌注射用水，USP单次使用的小瓶供给。
禁忌
过敏性或严重的全身反应Zemaira或A1-PI蛋白的历史。
免疫球蛋白A（IgA的）缺陷患者抗体的IgA，由于严重的超敏反应的风险。
警告和注意事项
管理Zemaira到谁经历过过敏反应或严重的全身反应到另一个A1-PI产品的个体时要小心。
患者选择性或严重IgA缺乏可以开发抗体IgA和，因此，有开发潜在的严重超敏反应和过敏反应的风险更大。如果出现过敏或严重过敏样反应，请立即停止输液。
因为Zemaira是由人血浆，它可以携带发送传染性病原体（例如，病毒和理论上，在Creutzfeldt-Jakob病[CJD]剂）的风险。
不良反应
在接收Zemaira在所有预执照受试者至少5％发生的最常见的不良反应的临床试验是头痛，鼻窦炎，上呼吸道感染，支气管炎，无力，咳嗽增加，发热，注射部位出血，鼻炎，咽痛，与血管舒张。
特殊人群中使用
怀孕：没有人或动物的数据。只有在明确需要使用。
资料修订：: 9/2015
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产地国家： 美国
原产地英文商品名:
Zemaira 1000mg/Vial(with sterile water 20ml)
原产地英文药品名:
Alpha 1-Proteinase Inhibitor (Human)
中文参考商品译名:
Zemaira冻干粉 1000毫克/瓶 (附 无菌水20毫升）
中文参考药品译名:
阿尔法1-蛋白酶抑制剂（人）
生产厂家中文参考译名:
CSL Behring, LLC
生产厂家英文名:
CSL Behring, LLC

Zemaira (alpha1-proteinase inhibitor)
Zemaira is an intravenous therapy for alpha 1 proteinase inhibitor deficiency.
It is indicated for chronic augmentation and maintenance therapy in individuals with alpha1-proteinase inhibitor (A1-PI) deficiency and clinical evidence of emphysema. Safety and effectiveness in pediatric patients have not been established.
Clinical Results
FDA approval of Zemaira is based on a several pivotal studies conducted on a total of 89 subjects with A1-PI deficiency. Subjects ranged in age from 29 to 68 years with a median age of 49 years. In one double blind, controlled clinical study, 44 subjects were randomized to receive 60 mg/kg of either Zemaira or Prolastin once weekly for 10 weeks. After 10 weeks, all subjects received Zemaira for an additional 14 weeks. All subjects were followed for a total of 24 weeks to complete the safety evaluation. The mean trough serum A1-PI levels at steady state (Weeks 7-11) in the Zemaira-treated subjects were statistically equivalent to those in the Prolastin-treated subjects. The difference between the Zemaira and the Prolastin groups was not considered clinically significant and may be related to the higher specific activity of Zemaira.
In a subgroup of subjects enrolled in the study, bronchoalveolar lavage was performed at baseline and at Week 11. Four A1-PI related analytes in ELF were measured: antigenic A1-PI, A1-PI: NE complexes, free NE, and functional A1-PI (anti-neutrophil elastase capacity, ANEC). A blinded retrospective analysis of the study, which revised the prospectively established acceptance criteria showed that within each treatment group, ELF levels of antigenic A1-PI and A1-PI:NE complexes increased from baseline to Week 11. No clinically significant differences were detected between the two treatment groups.
Side Effects
Adverse events associated with the use of Zemaira may include (but are not limited to) the following:
•Asthenia
•Injection Site Pain
•Dizziness
•Headache
•Pruritus
•Paresthesia
Mechanism of Action
Alpha1-proteinase inhibitor (A1-PI) deficiency is a chronic, hereditary disorder that can cause severe tissue damage and death. Pulmonary diseases, such as emphysema, are one of the main results of A1-PI deficiency. Emphysema is caused by the protease-antiprotease imbalance in the lungs which causes inflammation and tissue damage. A1-PI is the primary antiprotease in the lower respiratory tract, where it inhibits neutrophil elastase (NE), an enzyme that destroys pulmonary tissue. Those individuals without A1PI deficiency produce sufficient A1-PI to control the NE produced by activated neutrophils and are thus able to prevent inappropriate damage to lung tissue by NE.
Zemaira acts to increase and maintain serum levels and lung epithelial lining fluid (ELF) levels of A1-PI. Low blood levels of A1-PI are also associated with liver disease and liver cirrhosis.
http://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=0c3354b5-a1d8-4f98-ad55-2eafe4265c4e