本文以Tcu220c-IUD为对照,测定置MA-IUD妇女月经总量;经血纤溶系统包括组织型纤溶酶原激活剂(t-PA)活性、纤溶酶原(PLG)含量和纤溶活性(Fa);经血抗凝血物质包括抗凝血酶-Ⅲ(AT-Ⅲ)活性、蛋白 C(PC)抗原含量和 PC 活性以及经血凝血因子...本文以Tcu220c-IUD为对照,测定置MA-IUD妇女月经总量;经血纤溶系统包括组织型纤溶酶原激活剂(t-PA)活性、纤溶酶原(PLG)含量和纤溶活性(Fa);经血抗凝血物质包括抗凝血酶-Ⅲ(AT-Ⅲ)活性、蛋白 C(PC)抗原含量和 PC 活性以及经血凝血因子包括因子Ⅶ相关抗原(ⅧR:Ag)和纤维蛋白原(Fg)含量。实验表明 MA-IUD 能使妇女月经量明显减少,其与宫腔局部纤溶活性和抗凝血系统活性下降有关。宫腔局部凝血因子无明显改变,IUD 之孕激素的局部释放对肝脏无影响。展开更多
近年来,我国高度重视罕见病的防治。作为罕见病的代表疾病之一,我国的血友病防治取得了较大进步。1986年至1989年,全国血友病研究协作组按统一的方法及标准开展了我国的血友病流行病学调查,结果显示我国的血友病患病率为2.73/10万人口,...近年来,我国高度重视罕见病的防治。作为罕见病的代表疾病之一,我国的血友病防治取得了较大进步。1986年至1989年,全国血友病研究协作组按统一的方法及标准开展了我国的血友病流行病学调查,结果显示我国的血友病患病率为2.73/10万人口,国内各地区间患病率差异无统计学意义。2014年,荟萃分析显示我国的血友病患病率为2.8/10万。2018年,基于天津城市人口的数据,推算天津当地的血友病患病率为3.09/10万。随着血友病分级诊疗体系建设的全面推进,我国要求申请血友病综合管理中心和诊疗中心的医院必须能独立开展血友病诊断相关筛查试验和确诊试验。血友病诊断中,对于凝血因子活性测定,我国多数实验室通常采用基于活化部分凝血活酶时间(activated partial thromboplastin time, APTT)检测的一期法(凝固法)。但需注意,对于某些特殊类型的血友病需采用2种以上的活性检测方法。我国患者F8基因的各突变类型与国际数据报道类似;我国患者F9基因突变以单个碱基点突变为主,未发现突变热点。截至2023年6月,我国共有261家中心通过全国血友病登记系统,登记包括血友病在内的遗传性出血性疾病共计4万余例。我国的血友病治疗史经过不充分按需治疗、小剂量预防治疗探索等,目前已经往更高疗效的较高剂量预防及个体化预防治疗迈进。本报告基于登记数据及文献,全面总结我国血友病领域临床研究以及医疗保障方面的进展,分析不足之处,以期为进一步提高我国血友病诊疗水平指明方向。展开更多
Anticoagulation factor Ⅰ (ACF Ⅰ) from the venom of Agkistrodon acutus prolonged plasma prothrombin time (PPT) with dose-dependent manner and exhibited marked anticoagulant activity only at the concentration higher t...Anticoagulation factor Ⅰ (ACF Ⅰ) from the venom of Agkistrodon acutus prolonged plasma prothrombin time (PPT) with dose-dependent manner and exhibited marked anticoagulant activity only at the concentration higher than its critical concentration (12 nmol/L). It was discovered that ACF Ⅰ formed a 1:1 complex with activated coagulation factor (FXa) in the presence of Ca2+ ions by the method of polyacrylamide gel electrophoresis. Both native ACF I and decalcified ACF Ⅰ failed to form complexes with FXa in the absence of Ca2+. Sr2+ ions were able to replace Ca2+ ions in the binding of ACF I to FXa, but both Ba2+ ions and Tb3+ ions were ineffective. ACF Ⅰ was a new member of the IX/X-bp family in the C-type lectin superfamily, and had a amino acid composition similar to the other members of this family. It was composed of 251 amino acid residues with a molecular weight of 29 603.6 u on non-reducing condition, determined by MALDI-TOF-MS, and a molecular weight of 14.7 ku on reducing condition,展开更多
文摘近年来,我国高度重视罕见病的防治。作为罕见病的代表疾病之一,我国的血友病防治取得了较大进步。1986年至1989年,全国血友病研究协作组按统一的方法及标准开展了我国的血友病流行病学调查,结果显示我国的血友病患病率为2.73/10万人口,国内各地区间患病率差异无统计学意义。2014年,荟萃分析显示我国的血友病患病率为2.8/10万。2018年,基于天津城市人口的数据,推算天津当地的血友病患病率为3.09/10万。随着血友病分级诊疗体系建设的全面推进,我国要求申请血友病综合管理中心和诊疗中心的医院必须能独立开展血友病诊断相关筛查试验和确诊试验。血友病诊断中,对于凝血因子活性测定,我国多数实验室通常采用基于活化部分凝血活酶时间(activated partial thromboplastin time, APTT)检测的一期法(凝固法)。但需注意,对于某些特殊类型的血友病需采用2种以上的活性检测方法。我国患者F8基因的各突变类型与国际数据报道类似;我国患者F9基因突变以单个碱基点突变为主,未发现突变热点。截至2023年6月,我国共有261家中心通过全国血友病登记系统,登记包括血友病在内的遗传性出血性疾病共计4万余例。我国的血友病治疗史经过不充分按需治疗、小剂量预防治疗探索等,目前已经往更高疗效的较高剂量预防及个体化预防治疗迈进。本报告基于登记数据及文献,全面总结我国血友病领域临床研究以及医疗保障方面的进展,分析不足之处,以期为进一步提高我国血友病诊疗水平指明方向。
文摘Anticoagulation factor Ⅰ (ACF Ⅰ) from the venom of Agkistrodon acutus prolonged plasma prothrombin time (PPT) with dose-dependent manner and exhibited marked anticoagulant activity only at the concentration higher than its critical concentration (12 nmol/L). It was discovered that ACF Ⅰ formed a 1:1 complex with activated coagulation factor (FXa) in the presence of Ca2+ ions by the method of polyacrylamide gel electrophoresis. Both native ACF I and decalcified ACF Ⅰ failed to form complexes with FXa in the absence of Ca2+. Sr2+ ions were able to replace Ca2+ ions in the binding of ACF I to FXa, but both Ba2+ ions and Tb3+ ions were ineffective. ACF Ⅰ was a new member of the IX/X-bp family in the C-type lectin superfamily, and had a amino acid composition similar to the other members of this family. It was composed of 251 amino acid residues with a molecular weight of 29 603.6 u on non-reducing condition, determined by MALDI-TOF-MS, and a molecular weight of 14.7 ku on reducing condition,
