Naderi M, Dorgalaleh A, Tabibian Sh, Alizadeh Sh, Eshghi P, Solaimani Gh
Genetic Researcher Center in Non-Communicable Disease, Zahedan University of Medical sciences.
Hematology Department, Allied Medical School, Tehran University of Medical Sciences, Tehran, Iran.
Iran J Ped Hematol Oncol. 2013;3(4):164-72. Epub 2013 Oct 22.
Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed fibrin monomers during coagulation. This stable cross linked fibrin strands are resistanttodegradationby thefibrinolyticsystem that enablesthe bodyto stoppotential bleeding episodes. In the absence or severe decrease of factor XIII, although the clot is formed, but is rapidly degraded by the fibrinolytic system, and delayed bleedingoccurs.Factor XIII deficiency is an extremely rare bleeding disorder with estimated incidence of 1/2-3000, 000 in the general population. Presumptive diagnosis of factor XIII deficiency was by clot solubility test in 5M urea or 1% monochloroacetic acid environments. In patients with abnormal screening clot solubility test, the disease can be confirmedbymore specifictestssuch as quantitative factor XIII activity assay andFXIIIAgassay.After diagnosis of disease all patients with severe factor XIII deficiency(<1 U/dl) shouldreceive prophylactic substitution therapywith fresh frozen plasma (FFP) and cryoprecipitate as traditional choices or purified concentrateof blood coagulation factor XIII (Fibrogammin P) inorder to control severe and life-threatening clinical complications of factor XIII deficiency.
因子 XIII 或“纤维蛋白稳定因子”是一种转谷氨酰胺酶，它以异源四聚体的形式在血液循环中循环，由两个催化性 A 亚基和两个载体 B 亚基组成。这种重要的凝血因子在凝血级联反应中起关键作用，并在凝血过程中在可溶性纤维蛋白单体之间形成强共价键。这种稳定的交联纤维蛋白链对纤维蛋白溶解系统的降解具有抗性，从而使身体能够阻止潜在的出血事件。在因子 XIII 缺乏或严重减少的情况下，尽管会形成凝块，但会迅速被纤维蛋白溶解系统降解，从而发生延迟性出血。因子 XIII 缺乏是一种极其罕见的出血性疾病，在普通人群中的估计发病率为 1/2000000 - 1/3000000。因子 XIII 缺乏的初步诊断是通过在 5M 尿素或 1% 一氯乙酸环境中的凝块溶解度试验。对于筛查凝块溶解度试验异常的患者，可通过更特异的试验如定量因子 XIII 活性测定和因子 XIIIA 抗原测定来确诊疾病。在确诊疾病后，所有严重因子 XIII 缺乏（<1 U/dl）的患者应接受预防性替代治疗，传统的选择是新鲜冰冻血浆（FFP）和冷沉淀，或者使用凝血因子 XIII 纯化浓缩物（纤维蛋白原复合物 P），以控制因子 XIII 缺乏导致的严重且危及生命的临床并发症。
