部分中文Aralast处方资料（仅供参考） ARALAST NP [Alpha1-Proteinase Inhibitor (Human)] Indications & Usage ARALAST NP is indicated for chronic augmentation therapy in patients having congenital deficiency of A1–PI with clinically evident emphysema. ARALAST NP is not indicated as therapy for lung disease patients in whom congenital A1–PI deficiency has not been established. Important Risk Information ARALAST NP is contraindicated in individuals with selective IgA deficiencies (IgA level less than 15 mg/dL) who have known antibody against IgA, since they may experience severe reactions, including anaphylaxis. ARALAST NP is derived from pooled human plasma. It may carry a risk of transmitting infectious agents, e.g., viruses and theoretically, the Creutzfeldt-Jakob disease (CJD) agent. The recommended rate of administration (≤ 0.08mL/kg/min) should be closely followed and vital signs monitored continuously. If anaphylactic or severe anaphylactic reactions occur, the infusion should be discontinued immediately. ARALAST NP should be administered at room temperature within three (3) hours after reconstitution and should be administered alone, without mixing with other agents or diluting solutions. The safety of ARALAST NP was evaluated with ARALAST in a crossover clinical PK comparability study. The most common adverse events deemed related to ARALAST NP included headache and musculoskeletal discomfort. No serious adverse reactions or deaths were reported in the study. In the ARALAST pivotal study, the most common adverse events were headache and somnolence ----------------------------------------------------------------- ARALAST NP ARALAST NP is indicated for chronic augmentation therapy in patients having congenital deficiency of A1-PI with clinically evident emphysema. ARALAST NP is not indicated as therapy for lung disease patients in whom congenital A1-PI deficiency has not been established. The effect of augmentation therapy with ARALAST NP on pulmonary exacerbations and on the progression of emphysema in alpha-1 antitrypsin deficiency has not been demonstrated in randomized, controlled clinical trials.1 Clinical data demonstrating the long term effects of chronic augmentation or replacement therapy of individuals with ARALAST NP or ARALAST are not available. ARALAST NP is made with a two-step viral treatment process, solvent detergent and nanofiltration, to reduce the risk of viral transmission.1 The solvent detergent method destroys lipid membranes, thereby inactivating lipid-enveloped viruses. The nanofiltration process removes pathogens by size exclusion.2 ARALAST NP is derived from pooled human plasma. It may carry a risk of transmitting infectious agents, e.g., viruses and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.1 What does ARALAST NP do? Studies show that ARALAST NP augments the concentration of A1-PI in the blood and lungs.1 The most common adverse events deemed related to ARALAST NP included headache and musculoskeletal discomfort.1 ARALAST NP [Alpha1-Proteinase Inhibitor (Human)] ARALAST NP is indicated for chronic augmentation therapy in patients having congenital deficiency of A1-PI with clinically evident emphysema. The effect of augmentation therapy with ARALAST NP on pulmonary exacerbations and on the progression of emphysema in alpha1-antitrypsin deficiency has not been demonstrated in randomized, controlled clinical trials. Clinical data demonstrating the long-term effects of chronic augmentation or replacement therapy of individuals with ARALAST NP or ARALAST are not available. ARALAST NP is not indicated as therapy for lung disease patients in whom congenital A1-PI deficiency has not been established. Detailed Important Risk Information for ARALAST NP ARALAST NP is contraindicated in IgA deficient patients with antibodies against IgA, due to the risk of severe hypersensitivity. ARALAST NP is derived from pooled human plasma. It may carry a risk of transmitting infectious agents, e.g., viruses and theoretically, the Creutzfeldt-Jakob disease (CJD) agent. The recommended rate of administration (≤0.08 mL/kg/min) should be closely followed and vital signs monitored continuously. If anaphylactic or severe anaphylactoid reactions occur, the infusion should be discontinued immediately. Safety and effectiveness in patients over age 65 years of age have not been established. ARALAST NP should be administered at room temperature within three (3) hours after reconstitution and should be administered alone, without mixing with other agents or diluting solutions. The safety of ARALAST NP was evaluated with ARALAST in a crossover clinical PK comparability study. The most common adverse events deemed related to ARALAST NP included headache and musculoskeletal discomfort. No serious adverse reactions or deaths were reported in the study. In the ARALAST pivotal study, the most common adverse events were headache and somnolence. 美国FDA已批准Alpha Therapeutic公司和Baxter公司的人源α1蛋白酶抑制剂Aralast作为治疗患有先天性缺乏α1-抗胰蛋白酶(也称作α1-蛋白酶抑制剂)和临床证明肺气肿病人的增强治疗剂。 商品名 Aralast 开发商与上市厂商 本品由美国Alpha Therapeutic Corp公司研制开发，2003年5月在美国首次上市。 适应证 本品可用于先天性α1-蛋白酶抑制因子（PI）缺乏且具有临床肺气肿症状患者的治疗。 药理作用 本品可抑制肺部丝氨酸蛋白酶如中性粒细胞弹性蛋白酶(NE)。NE能降解肺泡壁的蛋白组分，并长期存在于肺部。通常认为肺部正常者，下呼吸道90%以上抗NE保护作用由 α1-PI提供。 α1-PI缺乏症是一种常染色体显性遗传性疾病，表现为血清和肺部α1- PI水平较低。较为严重的α1-PI缺乏症常与30～40岁之间发生的、缓慢渐进的中到重度全腺泡性肺气肿有关，可显著缩短患者的预期寿命。 NE由下呼吸道的中性粒细胞低水平缓慢释放，可导致肺部蛋白酶和蛋白酶抑制因子比例失衡。与α1-PI缺乏有关的肺气肿在下肺区尤其严重。由于在下呼吸道内没有足够的α1-PI 来对抗NE，病情会进一步发展。蛋白酶和蛋白酶抑制因子比例失衡，使得肺薄壁组织的结缔组织框架很容易遭到毁坏。 临床评价 α1-PI缺乏症有很多显型差异。 PiZZ显型患者的平均血清α1-PI低于正常水平的35%，Pi显型患者的血清(α1-PI低于检测下限。血清α1-PI水平较低即低于11μmol/L(80mg/dL) 者，有发生肺气肿的危险。两项研究显示，此类患者肺气肿发生率为 54.2%和57.0％。α1-PI严重缺乏且吸烟者，发生肺气肿的危险性高于曾经吸烟或不吸烟者。α1-PI缺乏症是最常见的可能致死的遗传性疾病之一。 一项临床研究对本品和已上市的 Prolastin(由拜耳公司生产的α1-PI产品）进行了比较。所有受试者都为确诊的患有先天性α1-PI缺乏症的肺气肿患者，并且之前6个月内未接受α1- PI补充治疗。28例受试者随机接受本品或对照药物，每周静注60mg/ kg，连续，0周。2例受试者过早从研究中退出：1例接受本品的受试者在6次输液后退出；1例接受 Prolastin的受试者在1次输液后，由于未预先安排支气管镜检查去除异物导致肺炎而退出。治疗前每例患者均检测血清α1-PI最低水平（抗原测定）和抗NE能力（功能测定）。在第一个为期10周的输液之后，接受对照药物的受试者转而使用本品，先前接受本品的则继续原先的治疗。在第7 周治疗前，对受试者进行支气管肺泡灌洗（BALs）。在12～24周治疗之前，对平均血清α1-PI最低水平的维持情况进行评估。 每周进行补充治疗后，可见血清α1-PI的最高和最低水平均稳步上升并维持数周。本品的代谢半衰期（t1/2为5.9天。所有受试者的血清抗 NE能力的最低水平在第2周均明显上升，到第3周，大多数受试者的血清抗NE能力的最低水平超过11μmol/ L。除少数患者以外，研究第3到24 周期间，受试者的血清α1-PI水平均保持在推荐的临界水平以上。 综上所述，每周1次静脉给予 60mg/kg补充治疗，本品和对照药物在保持目标血清α1-PI的最低水平和升高粘膜上皮液中α1-PI的抗原水平方面效果相似。 不良反应 临床最常见的不良反应为咽炎（1.6%）、头痛（0.7%）和咳嗽增加 (0.6%）。此外，支气管炎、鼻窦炎、皮疹、背痛、病毒性感染、周围性水肿、腹胀、眩晕、嗜睡、哮喘和鼻炎也较常见。所有症状均为轻到中度。 注意事项 本品应在配制完毕并达到室温后 3个小时之内使用。超过3小时后未使用的药品只能丢弃，不可留用。该溶液中不含防腐剂。 本品应单独使用，不可与其他制剂或稀释溶液混合使用。 本品对患者生育能力的影响以及对孕妇和哺乳期妇女的安全性尚未确定，临床使用需谨慎。 本品对儿童的安全性和有效性也尚未明确。 用法与用量 本品仅可静脉使用。推荐剂量为 60mg/kg，每周静注1次。推荐在配制完毕并达到室温后3个小时之内使用以避免在配制过程中可能造成的微生物污染。未用完的药品应丢弃。 本品的滴注速度不应超过每分钟 0.08mL/kg。如出现不良反应，应减慢滴速或停止滴注直至症状消失，然后以受试者可以耐受的速度重新继续滴注。 制剂 本品为无菌、无热原的冻干粉剂，每瓶为单次剂量，还包括注射所需的符合美国药典规定的适量无菌注射用水（每瓶25mL/0.5g；每瓶 50mL/1.0g)。本品单位以对猪胰弹性蛋白酶的抑制能力计。制剂包装还包括一只无菌双头转移针头和一个无菌的20μm过滤器。 --------------------------------------------------------------- 注：以下产品不同规格和不同价格，购买时请以电话咨询为准！ -------------------------------------------------------------- 原产地英文商品名: ARALAST NP 1G/50ML/VIAL 原产地英文药品名: ALPHA-1-PROTEINASE INHIBITOR 中文参考商品译名: ARALAST NP 1克/50毫升/瓶 中文参考药品译名: α1-蛋白酶抑制剂 生产厂家中文参考译名: 美国百特 生产厂家英文名: Baxter Healthcare Corporation --------------------------------------------------------------- 原产地英文商品名: ARALAST NP 0.5G/25ML/VIAL 原产地英文药品名: ALPHA-1-PROTEINASE INHIBITOR 中文参考商品译名: ARALAST NP 0.5克/25毫升/瓶 中文参考药品译名: α1-蛋白酶抑制剂 生产厂家中文参考译名: 美国百特 生产厂家英文名: Baxter Healthcare Corporation