...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Analysis and applications >Successful liver transplant from a hemophilia A donor with no development of hemophilia A in recipient
【24h】

Successful liver transplant from a hemophilia A donor with no development of hemophilia A in recipient

机译：成功的肝脏从血友病中移植富含血友病A中的血友病

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Background Hemophilia A is an X-linked inherited bleeding disorder caused by deficiency of coagulation factor VIII. Factor VIII is activated as part of the intrinsic coagulation cascade. It plays a crucial role as the cofactor in the intrinsic "tenase" complex activating factor X to assist in clot formation. Absence or mutation of this coagulation factor leads to excessive bleeding. Clinical manifestations of hemophilia relate to bleeding from impaired hemostasis, sequelae from bleeding, or complications of coagulation factor infusion. Diagnostic criteria for Hemophilia A include factor VIII activity level below 40% of normal, presence of a mutated F8 gene, and the absence of von Willebrand disease (F8 gene - Genetics Home Reference - NIH. ). Patients who have this intrinsic defect in the coagulation cascade have a characteristically prolonged PTT. It is theorized that the majority of factor VIII is synthesized mainly in the liver, by way of liver sinusoidal endothelial cells (Arruda VR. Haematologica. 2015;100(7):849-850). Extrahepatic production also occurs in the endothelial cells, kidneys, and lymphatic tissue. In 1969, Marchioro et al showed that transplantation of normal liver to hemophilia dog could normalize plasma factor VIII levels (Marchioro T L, Science. 1969;163(3863):188-190). These results were subsequently proven in humans. Liver transplantation from hemophilia A donors without factor VIII inhibitor is not commonly performed due to the perceived risk of developing hemophilia A in the recipient. There is currently limited literature aimed at elucidating this risk. We present a case of liver transplantation in a hemophilia A donor to a recipient with no history of hemophilia A with literature reviewis a case report, objective and method do not apply.
机译：背景技术血友病A是由凝血因子VIII缺乏引起的X链接遗传失调障碍。因子VIII被激活为内在凝固级联的一部分。它在内在的“诱导”复杂的激活因子X中起着至关重要的作用，以协助凝块形成。这种凝血因子的缺失或突变导致过度出血。血友病的临床表现涉及从止血受损，出血后遗症的出血，或凝血因子输注的并发症。血友病A的诊断标准包括低于正常的40％的因子VIII活性水平，突变的F8基因的存在，以及von Willebrand疾病的缺失（F8基因 - 遗传学家庭参考 - NIH。）。在凝固级联中具有这种固有缺陷的患者具有特征性地延长的PTT。理论上，大多数因子VIII通过肝窦内皮细胞主要在肝脏中合成（Aruda VR。2015年; 100（7）：849-850）。脱胸部产生也发生在内皮细胞，肾脏和淋巴组织中。 1969年，Marchioro等人表明，正常肝脏移植到血友病犬可以正常化血浆因子VIII水平（Marchioro T L，Science。1969; 163（3863）：188-190）。随后这些结果被证明在人体中。由于在接受者中发育血友病A的风险，血友病血友病患者的血友移植不常见于没有因子VIII抑制剂的供体。目前有有限的文学旨在阐明这种风险。我们在血友病中呈现肝移植的情况，给血友病患者没有血友病历史，案例报告，目标和方法不适用。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号