Teich Martin, Longin Elke, Dempfle Carl-Erik, König Stephan
Department of Pediatrics, Mannheim University Hospital, University of Heidelberg, Heidelberg, Germany.
Epilepsia. 2004 Feb;45(2):187-9. doi: 10.1111/j.0013-9580.2004.28302.x.
We present two children who developed a deficiency of factor XIII with valproate (VPA) treatment. This coagulation disorder has not been described in association with VPA treatment in children, and only very recently in one adult patient.
Both patients showed recurrent epistaxis as major clinical sign of a combination of decreased coagulation parameters (factor XIII deficiency with thrombocytopenia and decreased von Willebrand factor, respectively). A few days after reduction or withdrawal of VPA treatment, clinical symptoms disappeared, and laboratory findings were within normal range.
VPA is known to influence the synthetic function of the liver and the number and function of megakaryocytes. Therefore an alteration of the factor XIII level by VPA is conceivable. Our case reports suggest that bleeding symptoms during VPA treatment may be caused or aggravated by a decreased factor XIII activity. A determination of factor XIII activity should be considered before surgical procedures during VPA treatment to minimize the risk of (severe) postsurgical bleeding complications.
我们报告了两名儿童在接受丙戊酸盐（VPA）治疗后出现因子 XIII 缺乏症。这种凝血障碍在儿童 VPA 治疗中尚未见报道，仅在最近有一名成年患者出现过相关报道。
两名患者均表现为反复鼻出血，这是凝血参数降低（分别为因子 XIII 缺乏伴血小板减少和血管性血友病因子降低）的主要临床症状。在减少或停用 VPA 治疗几天后，临床症状消失，实验室检查结果恢复正常。
已知 VPA 会影响肝脏的合成功能以及巨核细胞的数量和功能。因此，VPA 导致因子 XIII 水平改变是可以想象的。我们的病例报告表明，VPA 治疗期间的出血症状可能由因子 XIII 活性降低引起或加重。在 VPA 治疗期间进行手术前，应考虑测定因子 XIII 活性，以尽量降低（严重）术后出血并发症的风险。
