制造商：
CSL Behring公司，有限责任公司
类药物：
凝血因子。
活性成分（S）：
第十三凝血因子浓缩物（人类）; 1000-1600单位，每小瓶; PWD静脉注射重组后，不含防腐剂。
指示（S）：
常规的预防性治疗先天性因子XIII（FXIII）缺乏。
药理学：
Corifact（FXIII）是一种内源性血浆糖蛋白，两个A -亚基和两个B亚基组成。 FXIII循环血液中的血小板，单核细胞和巨噬细胞存在。 FXIII出现有两种形式，一个heterotetrameric（A2B2）约320道尔顿的分子量和一个同型二聚体（A2）蜂窝形式与血浆蛋白。 FXIII是酶原被激活，钙离子的存在，成为激活FXIII（FXIIIa）的A亚基由凝血酶裂解。细胞内，只有A -亚基（A2）的同源二聚体的形式被发现。血浆中的B -亚基为A亚基的载体分子没有酶的活性和功能。他们稳定的A亚基的结构和保护他们免受水解。
FXIIIa促进在凝固过程中的纤维蛋白交联对纤维蛋白溶解血块的生理保护是必不可少的。 FXIIIa是谷氨酰胺转胺酶催化交联纤维蛋白α-和纤维蛋白稳定γ-链，并呈现更加弹性和抗纤溶纤维蛋白凝块。 FXIIIa也交叉连接α2-纤溶酶抑制剂纤维蛋白的α-链，由纤维蛋白溶酶降解纤维蛋白凝块的保护。交联纤维蛋白的凝血级联反应的最终结果，并提供抗拉强度主止血的血小板栓子。
临床试验：
12周，前瞻性，开放标签，多中心，药代动力学和安全性先天性FXIII缺乏评估40units/kg每28天为每个主题的三个剂量（年龄5-42岁）与7名女性和7名男性进行的研究。输液之前和之后的前两剂的血液采样，以确定FXIII活动，一个完整的PK分析后，第三剂量（稳态）进行。 FXIII活动水平确定Berichrom活性测定。
FXIII活动低谷水平之间保持5％和20％，预计提供临床受益。在上市后的研究将验证临床受益来衡量的，例行的预防性治疗先天性FXIII缺乏症患者的自发性出血发作的预防。剂量将个性化和调整，以保持与客观FXIII活动的5％至20％的谷底水平。
法律分类：
接收
成年人和儿童：
个性化。 ≤4mL/min的速度缓慢静脉注射给。最初40units/kg。随后的剂量：每28天;调整± 5units/kg槽FXIII活动使用Berichrom活性测定水平：<5％的低谷水平：通过5units/kg增加;槽5-20％的水平没有变化;两个> 20％的谷底水平：5units/kg;减少> 25％的低谷水平：5units/kg减少。保持槽FXIII约5-20％之间的活动水平。
警告/注意事项：
包含了人的等离子显示器可能感染的传播。长期治疗：考虑采取适当的预防接种（甲肝和乙肝病毒）。监视抑制抗体的发展。怀孕（部件C）。哺乳的母亲。
不良反应（S）：
过敏反应（过敏，皮疹，瘙痒和红斑）;停止，如果发生，畏寒/温度升高，关节痛，头痛，凝血酶 - 抗凝血酶水平升高，增加LFTs;免疫原性，血栓栓塞并发症。
如何提供：
单用小瓶- 1
最后更新：
2011年8月18日
对于先天性凝血因子ⅩⅢ缺乏症患者 首个预防出血药物corifact上市
首个用于预防先天性凝血因子ⅩⅢ缺乏症患者的药物Corifact由美国食品与药物管理局（FDA）批准上市。先天性凝血因子ⅩⅢ缺乏症是一种罕见的遗传缺陷疾病，在美国每300~500万人中有1人罹患该病。
Corifact由健康捐献者的混合血浆制备而来；最常见的副作用为过敏反应、寒战、发热、关节痛、头痛、凝血酶-抗凝血酶和肝酶水平升高。接受corifact治疗的患者可能产生凝血因子ⅩⅢ抗体，从而导致该药物失效。超剂量应用可导致凝血功能异常。Corifact在审批过程中享受了“孤儿药”和加速批准待遇，因而前期试验仅纳入了包括儿童在内的14例患者。
CORIFACT
Manufacturer:
CSL Behring, LLC
Pharmacological Class:
Clotting factor.
Active Ingredient(s):
Factor XIII concentrate (human); 1000–1600 units; per vial; pwd for IV inj after reconstitution; preservative-free.
Indication(s):
Routine prophylactic treatment of congenital Factor XIII (FXIII) deficiency.
Pharmacology:
Corifact (FXIII) is an endogenous plasma glycoprotein consisting of two A-subunits and two B-subunits. FXIII circulates in blood and is present in platelets, monocytes, and macrophages. FXIII appears in two forms, a heterotetrameric (A2B2) plasma protein with a molecular weight of about 320 kilodaltons and a homodimeric (A2) cellular form. FXIII is a proenzyme that is activated, in the presence of calcium ion, by thrombin cleavage of the A-subunit to become activated FXIII (FXIIIa). Intracellularly, the homodimeric form of only the A-subunits (A2) is found. The B-subunits in plasma have no enzymatic activity, and function as carrier molecules for the A-subunits. They stabilize the structure of the A-subunits and protect them from proteolysis.
FXIIIa promotes cross-linking of fibrin during coagulation and is essential to the physiological protection of the clot against fibrinolysis. FXIIIa is a transglutaminase enzyme that catalyzes the cross-linking of the fibrin alpha- and gamma-chains for fibrin stabilization and renders the fibrin clot more elastic and resistant to fibrinolysis. FXIIIa also cross-links alpha2-plasmin inhibitor to the alpha-chain of fibrin, resulting in protection of the fibrin clot from degradation by plasmin. Cross-linked fibrin is the end result of the coagulation cascade, and provides tensile strength to a primary hemostatic platelet plug.
Clinical Trials:
The 12 week, prospective, open-label, multicenter, pharmacokinetic and safety study conducted in 7 females and 7 males with congenital FXIII deficiency (age 5–42 years) evaluated three doses at 40units/kg every 28 days for each subject. Blood sampling before and after infusion for the first two doses was to determine FXIII activity and a complete PK analysis was conducted after the third dose (steady state). FXIII activity levels were determined by the Berichrom activity assay.
Maintaining the FXIII activity trough level between 5% and 20% is predicted to provide a clinical benefit. The clinical benefit will be verified in a post-marketing study to measure the prevention of spontaneous bleeding episodes with routine prophylactic treatment for patients with congenital FXIII deficiency. Dosing will be individualized and adjusted with the objective to maintain FXIII activity trough levels of 5% to 20%.
Legal Classification:
Rx
Adults & Children:
Individualize. Give by slow IV inj at a rate of ≤4mL/min. Initially 40units/kg. Subsequent doses: give every 28 days; adjust ±5units/kg based on trough FXIII activity levels using Berichrom Activity Assay: One trough level of <5%: increase by 5units/kg; trough level of 5–20%: no change; two trough levels of >20%: decrease by 5units/kg; one trough level of >25%: decrease by 5units/kg. Maintain trough FXIII activity level between approximately 5–20%.
Warnings/Precautions:
Contains human plasma; monitor for possible infection transmission. Long-term therapy: consider appropriate vaccination (hepatitis A and B virus). Monitor for development of inhibitory antibodies. Pregnancy (Cat. C). Nursing mothers.
Adverse Reaction(s):
Hypersensitivity reactions (allergy, rash, pruritus, and erythema); discontinue if occurs, chills/rise in temperature, arthralgia, headache, elevated thrombin-antithrombin levels, increased LFTs; immunogenicity, thromboembolic complications.
How Supplied:
Single-use vial—1