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血友病患者的肝移植。

Liver transplantation in the haemophilia patient.

机构信息

Department of Hematology/Oncology, Henry Ford Health System, Detroit, MI, USA.

出版信息

Haemophilia. 2011 Sep;17(5):e981-4. doi: 10.1111/j.1365-2516.2011.02533.x. Epub 2011 Apr 19.

Abstract

Hepatitis C is a chronic condition that many persons with haemophilia contracted in the 1980s due to the infusion of factor concentrates which did not have viral inactivation processes in place. Many patients with haemophilia are now living longer lives, well into 80 years of age, due to the improvement of their care. The effects of the HCV on the liver over time are now being realized as this population ages. Although the new treatments for hepatitis C have a prolonged response, as demonstrated by a persistent negative viral load, many haemophilia patients have either not responded to the therapy or had significant side-effects to the treatment, preventing continued therapy. Of these infected haemophiliacs with liver disease, many have demonstrated a slow progressive decline resulting in liver failure, cirrhosis or liver cancer. Liver transplant then becomes their only option. This article will review liver transplantation in the haemophilia patient highlighting three case studies demonstrating the effectiveness of specific short-term factor infusions and other haemostatic support to minimize bleeding during the surgical period. These cases confirm the opportunity for successful liver transplantation for haemophilia patients utilizing specific factor recommendations with minimal bleeding risk.

摘要

丙型肝炎是一种慢性疾病,许多血友病患者在 20 世纪 80 年代因输注未经过病毒灭活处理的因子浓缩物而感染。由于护理水平的提高,许多血友病患者现在的寿命更长,甚至达到 80 岁以上。随着这一人群年龄的增长,丙型肝炎对肝脏的影响现在正在显现。尽管新的丙型肝炎治疗方法具有持久的反应,如持续的病毒载量阴性所证明的那样,但许多血友病患者要么对治疗没有反应,要么治疗有明显的副作用,从而阻止了继续治疗。在这些患有肝脏疾病的感染性血友病患者中,许多人表现出缓慢的进行性下降,导致肝功能衰竭、肝硬化或肝癌。肝移植成为他们唯一的选择。本文将回顾血友病患者的肝移植,强调三个病例研究,展示特定短期因子输注和其他止血支持的有效性,以最大限度地减少手术期间的出血。这些病例证实了利用特定因子建议和最小出血风险成功进行肝移植的机会。

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