英文名称：IDELVION I.V. Injection（Albutrepenonacog Alfa[Genetical Recombination]）

中文名称：长效重组白蛋白融合蛋白注射剂

生产厂家：CSL Behring

イデルビオン静注用250／イデルビオン静注用500／イデルビオン静注用1000／イデルビオン静注用2000

药物分类名称
转基因凝血因子IX清蛋白融合蛋白制剂
批准日期：2016年11月
商標名
IDELVION I.V. Injection
一般名
アルブトレペノナコグ　アルファ（遺伝子組換え）
Albutrepenonacog Alfa（Genetical Recombination）
本質
Albrepsinnonagoguα是重组融合糖蛋白，1至415和434至1,018的氨基酸序列对应于人凝血因子IX和人白蛋白。 Albrepsinnonagoguα是一种糖蛋白（分子量：约125,000），由中国仓鼠卵巢细胞产生的1,018个氨基酸残基组成。
处理注意事项
由于该药物不是特定生物体的产品，而是血液制品替代药物，当给予或处方该药物时，药物名称（销售名称）及其序列号（批号），给药当天或处方 记录接受管理或处方的患者的姓名，地址等，并保存自使用之日起至少20年。
批准条件
制定药品风险管理计划并适当实施。
药用药理学
1.作用机制
该药物的结构和作用类似于内源性凝血因子IX，并暂时校正血浆凝血因子IX水平并改善血液凝固障碍。 重组白蛋白和重组凝血因子IX的融合被认为可延长血液半衰期。
2.主要的非临床研究
在血友病B犬和血友病B小鼠（FIX敲除小鼠）中，该药物的止血作用得到认可。 此外，在血友病B犬中，已经认识到血浆凝固因子IX活性的延长与该药物的血浆药代动力学相关。
适应症
抑制血液凝固因子IX缺乏患者的出血倾向
用法与用量
将该产物溶解在附着的裂解溶液的总量中，并静脉内缓慢注射。
通常，每公斤体重给予50个国际单位，但根据患者的情况适当增加或减少。
经常给药时，通常每7天给予每公斤体重35至50国际单位一次。根据患者的情况，可以改为每14天给药一次，每公斤体重75国际单位。剂量在任何给药间隔适当调整，但一次不应超过每公斤体重75国际单位。
包装
250个国际单位×1个小瓶
[裂解液（注射用水注射液）2.5mL×1瓶]
500国际单位×1小瓶
[裂解液（注射用水注射液）2.5mL×1瓶]
1000国际单位×1小瓶
[裂解液（注射用水注射液）2.5mL×1瓶]
2000国际单位×1小瓶
[裂解液（注水注射用水）5mLx1小瓶]
制造和销售（进口）
CSL Behring

注：以上中文处方资料不够完整，使用者以原处方资料为准。
完整说明书附件：http://www.info.pmda.go.jp/go/pack/6343448D1020_1_02/
Albumin fusion technology realizes a high hemostatic effect at the maximum administration interval of 14 days Half-life extended hemophilia B therapeutic preparation
• Fusion of genetically engineered albumin and recombinant blood coagulation factor IX prolongs the half-life in blood
• Average trough value of blood clotting activity (lowest value), 12% (once age 12 years) with administration once every 2 weeks
• Median bleeding (AsBR), median number of annual bleeds is 0 (12 years or older)
• Prevention and improvement of joint disorders are expected due to high bleeding preventive effect
Today, CSL Behring Co., Ltd. (Headquarters: Koto Ward, Tokyo, President and Representative Director: Jean Marc Moranju) announced today that on November 29, Genetic Modified Blood Coagulation Factor IX Albumin Fusion Protein Formulation, Hemophilia B Treatment Preparation We are pleased to inform you that "Idealbion® for intravenous injection 250, 500, 1000, 2000" (hereinafter, Ideerbion®) has been released.
IDERBION ® is a half-life extended recombinant blood coagulation Factor IX albumin fusion protein preparation adapted for "suppression of bleeding tendency in patients with blood coagulation factor IX deficiency." The world's first proprietary technology to combine albumin known for its long biological half-life with blood coagulation Factor IX at the gene level has realized sustained high blood coagulation Factor IX activity over a long period of time.
Haemophilia is a disease that is difficult to stop blood when bleeding, which causes various restrictions and difficulties in daily life due to internal bleeding such as joints and muscles, intracranial bleeding, and the like. Also, as symptoms progress, there are also onset of joint disorder. Therefore, depending on the patient's symptoms and lifestyle, there are cases such as refraining from active activity due to anxiety about bleeding.
Treatment of haemophilia is mainly supplementary therapy that supplements missing blood coagulation factors with blood products, regularly maintaining the activity of blood coagulation factors by regularly administering the formulation to prevent bleeding beforehand Replacement therapy is commonly practiced. For conventional formulations, injections of 2 to 4 times per week, dosing intervals of about once every 10 days are required, which was the burden of patients and their families.
Ideerbion ® maintains a high average trough value of 12% at dosing intervals of 14 days, confirming the effect not found in the conventional formulation that the median value of natural bleeding (AsBR) and joint hemorrhage is 0 . Due to this high bleeding preventive effect, it is anticipated that burden by frequent injections will be reduced and joint disorder prevention / improvement will be anticipated, so the release in Japan as a new treatment option was awaited.
Jean Marc Moranju, President and Representative Director of CSL Bearing Co., Ltd. "Idealbion ® is one of the innovative products produced by years of R & D efforts to treat hemorrhagic diseases in the 100-year history of CSL . This product, as a preparation that can support the more active life of patients, reduce burden from frequent injections, and prevent arthropathy, patients, their families, health care workers I am convinced that it will be an important treatment option for patients. "
Product overview of IDERBION ®
Sales name
Ideerbion ® for intravenous injection 250/500/1000/2000
common name
Albre trepe nonacoag alpha (genetical recombination)
Application category New medicine containing active ingredients
Restriction classification
Biological products
Indication or effect
Suppression of bleeding tendency in blood coagulation factor IX deficient patients
Manufacturing sales
CSL Bearing Co., Ltd.
Manufacturing marketing approval acquisition date: September 28, 2016
Drug price
Iderbion ® for intravenous injection 250
Ideerbion ® for intravenous injection 500
Iderbion ® for intravenous injection 1000
Iderbion ® for intravenous injection 2000
Drug price criteria
November 18, 2016
Approval abroad Approved in USA, European Union, Switzerland, Canada and Australia
About hemophilia B
Haemophilia B (blood coagulation factor IX deficiency) is a hereditary hemorrhagic disease, it takes time to stop the hemostasis due to deficiency or loss of function of blood clotting factor IX, and it is difficult to treat it in the joint, muscle, or brain Symptoms such as spontaneous bleeding are seen. Hemophilia B is mostly a disease found in males, domestic statistics are affected by 1 to 2 people per 100,000 men and the number of patients is about 1000 people.
About CSL Bearing Co., Ltd.
Global company of biological products, Japan corporation of CSL Bering (head office: USA). We are focusing on the hemophilia area, critical care and hemostasis area, immune and rare disease area, we will contribute to medical treatment in Japan by stably supplying highly innovative formulation with world standard safety. In August 2016, we applied for domestic approval of prothrombin complex formulation (BE1116) which corrects the anticoagulant state of patients receiving treatment of vitamin K antagonist (warfarin etc) by treatment / prevention of thromboembolism.