CHINA 凝血抑制物的筛查和检测
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usually develop at an early age after a few infusions of factor VIII or IX (<10 exposure days)
通常在几次输注因子VIII 或 IX 后的早期可产生抑制物（ <10 天）
important to screen for inhibitors every 6 months to one year
绝大多数血友病患者对因子VIII输注不会产生免疫反应，例如，他们不会发 生免疫反应来抑制因子VIII的凝血活性
In a number of cases an immune response can become strong enough to render inefficient further FVIII treatment (在一些病例中，免疫反应足够强大，足以中和日 后非充足的因子VIII治疗）
Coagulation Inhibitor Screening and Inhibitor Assay 凝血抑制物的筛查和检测
Factor VIII inhibitors（ 因子VIII 抑制物）
Haemophilia A is characterized by the absence or an insufficient amount of functional Factor VIII
responders”
（那些表现为抑制物明显增加的患者被称作是“高反应者“）
patients who manifest a slight inhibitor increase are referred to as “low responders
（那些表现为抑制物轻微上升的患者被称作是“低反应者”）
FVIII inhibitors to treatment are time dependent whereas to other clotting factors they are immediate acting（因子VIII抑制物的治疗是时间依赖性的，而其 他凝血因子的治疗则是即时反应）
Other inhibitors of coagulation（凝血的 其他抑制剂）
inhibitors can develop to almost any clotting factor protein（几乎 所有的凝血因子蛋白都有相应的抑制物）
factor VIII inhibitors can also arise associated with immune disorders such as malignancy, sensitivity to drugs, post partum 因子VIII抑制物升高也可能与免疫性疾病如肿瘤、药物敏感和产后 相关）
The underlying gene mutation can prevent production of a soluble form of Factor VIII
潜在的基因突变可防止可溶性的因子VIII的产生
Replacement therapy is by administration of FVIII concentrates prepared from plasma or produced by recombinant cDNA technology
每6个月至1年筛查一次抑制物十分重要
Exact figure on the incidence of inhibitors is difficult to provide（很难提供抑制物发生率的确切数据）
8 - 20% in Haemophilia A（血友病A 8-20%）
2.5 - 16% in Haemophilia B （血友病B 2.5-16%）
血友病A的特征是有功能的因子VIII缺失或缺乏
X-linked hereditary deficiency results in uncontrolled bleeding in joints, muscle and soft tissue
X-连锁遗传性缺陷症可导致关节、肌肉和软组织不可控制的出血
Depends on methodology（取决于方法学）
the threshold level in the assay varies from one lab to another - (0.6 Bethesda units)（测试中的阈值水平每 个实验室都不相同-0.6 Bethesda单位）
treatment and taking 4 - 8 months to return to original level （每个患者体内的抗体滴度各不相同，在治疗后4-10天达到高峰，4-8个月之后回到
初始水平） patients who manifest a pronounced inhibitor increase are referred to as “high
可通过输注因子V术获得
Majority of haemophiliac patients are immunologically unresponsive to FVIII infusions ie. they do not mount an immune response that can inhibit the procoagulant activity of FVIII
inhibitors are now considered the main complication of treatment（抑制物目前被 认为是最主要的治疗并发症）
The antibodies are of IgG type（抗体主要是IgG型） The antibody titre varies from patient to patient - marked in 4 -10 days after
通常在几次输注因子VIII 或 IX 后的早期可产生抑制物（ <10 天）
important to screen for inhibitors every 6 months to one year
绝大多数血友病患者对因子VIII输注不会产生免疫反应，例如，他们不会发 生免疫反应来抑制因子VIII的凝血活性
In a number of cases an immune response can become strong enough to render inefficient further FVIII treatment (在一些病例中，免疫反应足够强大，足以中和日 后非充足的因子VIII治疗）
Coagulation Inhibitor Screening and Inhibitor Assay 凝血抑制物的筛查和检测
Factor VIII inhibitors（ 因子VIII 抑制物）
Haemophilia A is characterized by the absence or an insufficient amount of functional Factor VIII
responders”
（那些表现为抑制物明显增加的患者被称作是“高反应者“）
patients who manifest a slight inhibitor increase are referred to as “low responders
（那些表现为抑制物轻微上升的患者被称作是“低反应者”）
FVIII inhibitors to treatment are time dependent whereas to other clotting factors they are immediate acting（因子VIII抑制物的治疗是时间依赖性的，而其 他凝血因子的治疗则是即时反应）
Other inhibitors of coagulation（凝血的 其他抑制剂）
inhibitors can develop to almost any clotting factor protein（几乎 所有的凝血因子蛋白都有相应的抑制物）
factor VIII inhibitors can also arise associated with immune disorders such as malignancy, sensitivity to drugs, post partum 因子VIII抑制物升高也可能与免疫性疾病如肿瘤、药物敏感和产后 相关）
The underlying gene mutation can prevent production of a soluble form of Factor VIII
潜在的基因突变可防止可溶性的因子VIII的产生
Replacement therapy is by administration of FVIII concentrates prepared from plasma or produced by recombinant cDNA technology
每6个月至1年筛查一次抑制物十分重要
Exact figure on the incidence of inhibitors is difficult to provide（很难提供抑制物发生率的确切数据）
8 - 20% in Haemophilia A（血友病A 8-20%）
2.5 - 16% in Haemophilia B （血友病B 2.5-16%）
血友病A的特征是有功能的因子VIII缺失或缺乏
X-linked hereditary deficiency results in uncontrolled bleeding in joints, muscle and soft tissue
X-连锁遗传性缺陷症可导致关节、肌肉和软组织不可控制的出血
Depends on methodology（取决于方法学）
the threshold level in the assay varies from one lab to another - (0.6 Bethesda units)（测试中的阈值水平每 个实验室都不相同-0.6 Bethesda单位）
treatment and taking 4 - 8 months to return to original level （每个患者体内的抗体滴度各不相同，在治疗后4-10天达到高峰，4-8个月之后回到
初始水平） patients who manifest a pronounced inhibitor increase are referred to as “high
可通过输注因子V术获得
Majority of haemophiliac patients are immunologically unresponsive to FVIII infusions ie. they do not mount an immune response that can inhibit the procoagulant activity of FVIII
inhibitors are now considered the main complication of treatment（抑制物目前被 认为是最主要的治疗并发症）
The antibodies are of IgG type（抗体主要是IgG型） The antibody titre varies from patient to patient - marked in 4 -10 days after