Auerswald G
Comprehensive Care Center for Thrombosis and Haemostasis, Klinikum Bremen-Mitte, Professor Hess Children's Hospital, Bremen, Germany.
Thromb Res. 2006;118 Suppl 1:S29-31. doi: 10.1016/j.thromres.2006.01.015. Epub 2006 Mar 30.
Factor X (FX) is a vitamin K-dependent protease that plays a central role in the blood coagulation cascade. Deficiency of FX is one of the rarest congenital coagulation disorders, resulting in a variable clinical phenotype. The molecular basis for FX deficiency is characterised by a large number of gene mutations and polymorphisms. The Greifswald Registry of FX deficiency was started in 1998 and aims to investigate the relationship between genetic mutations and the clinical severity of the disease. To date, 34 of 102 patients have been treated for severe bleeding episodes or for surgical procedures. Only seven patients, six of whom are children, receive regular prophylaxis with Factor IX (FIX) concentrate (FIX HS, ZLB Behring; also containing approximately 800IU FX). In all cases, the bleeding episodes are well controlled without any serious treatment-related complications.
凝血因子X（FX）是一种维生素K依赖的蛋白酶，在血液凝固级联反应中起核心作用。FX缺乏是最罕见的先天性凝血障碍之一，会导致多种临床表型。FX缺乏的分子基础以大量基因突变和多态性为特征。格赖夫斯瓦尔德FX缺乏症登记始于1998年，旨在研究基因突变与疾病临床严重程度之间的关系。迄今为止，102名患者中有34名因严重出血发作或外科手术接受了治疗。只有7名患者（其中6名是儿童）接受了凝血因子IX（FIX）浓缩物（FIX HS，ZLB拜耳；也含有约800IU FX）的定期预防治疗。在所有病例中，出血发作均得到良好控制，且未出现任何与治疗相关的严重并发症。
