Tacke Frank, Fiedler Kai, von Depka Mario, Luedde Tom, Hecker Hartmut, Manns Michael P, Ganser Arnold, Trautwein Christian
Department of Gastroenterology, Hepatology & Endocrinology, Hannover Medical School, Hannover, Germany.
Liver Int. 2006 Mar;26(2):173-81. doi: 10.1111/j.1478-3231.2005.01205.x.
BACKGROUND/AIMS: Alterations of plasma coagulation factor XIII may contribute to bleeding disorders in patients with liver cirrhosis. As standard clotting tests such as prothrombin time or activated thromboplastin time (aPTT) cannot detect factor XIII deficiency, this may often be overlooked in clinical practice. We aimed to define factor XIII's clinical and prognostic role in chronic liver disease.
Factor XIII activities were assessed among various other parameters in 111 patients with chronic liver diseases during evaluation for liver transplantation in a prospective study.
Unlike coagulation factors II, V or VII, factor XIII activity was maintained in the majority of patients with liver cirrhosis. However, although rarely, factor XIII deficiencies (<50%) occurred, especially in Child C cirrhosis. Factor XIII levels correlated with liver's biosynthetic capacity (cholinesterase activity, albumin, total protein) as well as with platelet count, global coagulation tests and other single coagulation factors. Patients reporting a current systemic bleeding tendency at study entry had significantly reduced factor XIII. In a 6-year follow-up, patients with factor XIII<50% had a significantly increased risk of severe upper gastrointestinal bleed, and reduced factor XIII (<50%, 50-75% vs. normal) was associated with increased mortality.
Factor XIII deficiency is rare in patients with liver cirrhosis, but is associated with a clinical bleeding tendency and an unfavorable prognosis for future hemorrhages and survival.
背景/目的:血浆凝血因子 XIII 的改变可能导致肝硬化患者出现出血性疾病。由于诸如凝血酶原时间或活化部分凝血活酶时间(aPTT)等标准凝血试验无法检测到因子 XIII 缺乏,因此在临床实践中这一情况常常被忽视。我们旨在明确因子 XIII 在慢性肝病中的临床及预后作用。
在一项前瞻性研究中,对 111 例慢性肝病患者在进行肝移植评估时的多种参数进行了评估,其中包括因子 XIII 活性。
与凝血因子 II、V 或 VII 不同,大多数肝硬化患者的因子 XIII 活性得以维持。然而,尽管少见,但因子 XIII 缺乏(<50%)情况仍会出现,尤其是在 Child C 级肝硬化患者中。因子 XIII 水平与肝脏的生物合成能力(胆碱酯酶活性、白蛋白、总蛋白)以及血小板计数、整体凝血试验和其他单个凝血因子相关。在研究入组时报告有当前全身出血倾向的患者,其因子 XIII 水平显著降低。在为期 6 年的随访中,因子 XIII<50%的患者发生严重上消化道出血的风险显著增加,且因子 XIII 水平降低(<50%、50 - 75% 与正常水平相比)与死亡率增加相关。
因子 XIII 缺乏在肝硬化患者中较为罕见,但与临床出血倾向以及未来出血和生存的不良预后相关。