近日，美国食品药品监督管理局（FDA）批准Berinert上市。Berinert作为首个治疗遗传性血管水肿（HAE）引起的急性腹痛和面部肿胀药物，HAE是一种罕见、可致命的常染色体显性遗传性疾病，发作时以皮肤、小肠、口腔、咽喉等处水肿为特征。腹痛发作时出现腹绞痛、恶心、呕吐、痉挛和腹泻等症状。该药目前主要用于成人和青少年HAE患者HAE自行发作，或在紧张、手术、感染状态时HAE的发作。
Berinert系由人体血浆提取的蛋白制品，可调节凝血和炎性反应。凝血和炎性反应异常会导致机体局部组织肿胀。对124名成人和青少年C1酯酶抑制因子缺乏患者的临床试验研究表明，Berinert可有效治疗HAE患者的中、重度腹痛和面部水肿。
临床研究显示，Berinert严重不良反应为加剧HAE引发的疼痛；常见不良反应包括继发HAE发作、头痛、腹痛、恶心、肌痉挛、疼痛、腹泻和呕吐。
FDA提示，对C1酯酶抑制剂产生致命性过敏反应病史者，禁用Berinert。
Berinert由德国CSL Behring,Inc.公司生产。

BERINERT（人类C1酯酶抑制剂）试剂盒

[杰特贝林公司]

美国首次批准：2009

适应症及用法
Berinert是一种血浆源性C1酯酶抑制剂（人）用于治疗急性腹痛，面部，喉部发作的遗传性血管水肿（HAE）的成人和青少年患者。
Berinert预防性治疗的安全性和有效性尚未确立。

【用法用量】
只适用于静脉使用。
以避光的瓶存放在原来的纸箱。储存在2-25°C（36-77°F）。不要冻结。
管理每公斤体重20个国际单位。
重组Berinert在使用前用无菌注射用水，USP。
管理在室温下8小时内重组。
约4毫升每分钟的速率注入。
不要与其他药物的产品或解决方案混合Berinert。
适当的培训，患者可自行管理识别HAE发作后，。
 
剂型和优势
500国际单位冻干集中在用10mL无菌注射用水，USP配制的单次使用的小瓶。
 
禁忌
不要使用患者的历史危及生命的直接的过敏反应，包括过敏性休克，C1酯酶抑制剂制剂。
 
警告和注意事项
可能会出现过敏反应。的肾上腺素应该是立即可用来治疗任何急性严重的过敏反应，停止管理。
已报告的推荐剂量为C1酯酶抑制剂（人）产品，包括Berinert栓塞，治疗后的血栓性事件。血栓性事件也已在协会与Berinert使用时，标签和高于标有doses.1密切监测患者血栓性事件的已知的危险因素。
berinert由人血浆，并且可能包含传染性病原体，例如，病毒和，理论上，Creutzfeldt-Jakob病（CJD）的剂。
喉攻击：自我管理的Berinert喉攻击，建议患者立即就医。
 
不良反应
科目谁收到Berinert报告最严重的不良反应是疼痛的严重程度与栓塞中的增加。
在大于4％的受访者和大于安慰剂组之间，Berinert的安慰剂对照的临床试验的科目谁收到的最常见的不良反应是味觉障碍。

特殊人群中使用
怀孕：动物数据。有限的人力数据。使用只有在明确需要。
到成人相比，调整基线时，半衰期为Berinert短间隙（每公斤基准）儿童为快。不知道这种差异的临床意义。

更新日期：07/2012

Berinert (C1 Esterase Inhibitor (Human))
The C1 esterase inhibitor is a protein that is naturally produced by the organism. It helps the patient get over inflammatory symptoms of various diseases. A simple inflammation will go away by itself due to the protein, but depending on its severity, some actions last longer than others. However, there are individuals who have a low level of C1 esterase inhibitors in the organism. This is because they suffer from angioedema. The angiodema is similar to urticaria. It comes as a minor inflammation under the skin. It may be caused by allergies, medicines or various diseases. This is when a C1 esterase inhibitor treatment is required.

Berinert (C1 esterase inhibitor (human)) is a popular medicine based on this human protein that can only treat the hereditary angioedema. It cannot treat other causes and it requires a medical prescription. The patients have to go through a sophisticated test before getting this medicine. The causes of the angioedema are very important, as well as the frequency of angioedema attacks.

Administration
Berinert (C1 esterase inhibitor (human)) comes as a dry powder that must be administered intravenously. Therefore, it must be mixed with an injection substance before being administered. The injection should be taken by a specialist doctor. The optimal dose is 20 units per kilo, so it depends on your total weight. The injection substance is also provided in the package.

The doctor will prepare the solution by mixing the powder with the distilled water. It must be properly inspected before being taken. It has to be clear and colorless, without any visible particles. The powder composition doesn’t include any preservatives, therefore you have to make sure the injection is prepared right before the administration. It shouldn’t be stored or anything alike and used as soon as it is prepared.

The Berinert (C1 esterase inhibitor (human)) injection must be taken slowly, at about 4ml per minute. The dosage may also differ, according to the deficit of C1 esterase inhibitor in the organism.

Contraindications
An inappropriate treatment with Berinert (C1 esterase inhibitor (human)) may be fatal, so the doctor has to be very careful with the prescription and administration. If the patient is allergic or hypersensitive to such a treatment or the ingredients of this medicine, the doctor must find an alternative treatment.

It is also contraindicated in those whose medical history includes blood clots or cardiovascular diseases.

Secondary effects
The severe Berinert (C1 esterase inhibitor (human)) side effects require immediate medical attention. The patients might present:

Numbness
Temporary paralysis
Swelling
Various pains
Increased heart rate
There are also a few mild adverse reactions that are normally temporary and shouldn’t cause too much trouble, such as:

Nausea
Diarrhea
Headaches
Urticaria

Expanded Label for Berinert Approved
January 04, 2012
 
BERINERT (C1 esterase inhibitor [human]) 500units/vial injection by CSL Behring
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CSL Behring announced that the FDA has approved a label expansion for self-administration of Berinert (C1 esterase inhibitor [human]; CSL Behring), a plasma-derived concentrate of C1 esterase inhibitor, for the treatment of acute abdominal or facial attacks of hereditary angioedema (HAE). With appropriate training from a physician, patients can now self-administer Berinert by intravenous infusion. As part of the label expansion, Berinert is now also indicated to treat life-threatening laryngeal HAE attacks, as well as facial and abdominal attacks. This approval was based on data from clinical studies in which intervention with Berinert at the onset of an abdominal or facial HAE attack was shown to provide significantly faster relief (median onset of relief of 48 minutes versus more than four hours in placebo group).  In a non-placebo extension study, the use of Berinert to treat laryngeal attacks provided a median onset of relief of 15 minutes.