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血友病患者前瞻性审计:出血事件及管理

Prospective audit of patients with haemophilia: bleeding episodes and management.

作者信息

Alexander Matthew, Barnes Chris, Barnett Peter

机构信息

Department of Orthopaedics, Austin Hospital, Melbourne, Victoria, Australia.

出版信息

J Paediatr Child Health. 2012 Feb;48(2):177-9. doi: 10.1111/j.1440-1754.2011.02064.x. Epub 2011 May 12.

Abstract

BACKGROUND

Haemophilia is a congenital bleeding disorder that requires replacement of factor VIII (haemophilia A) or factor IX (haemophilia B) via intravenous infusions. Children can either be treated with prophylactic treatment to prevent bleeding or be managed with on-demand therapy and treat specific bleeding episodes.

AIMS

The aim of this paper was to prospectively follow a cohort of haemophilia patients to determine the incidence of bleeding, re-bleeding, re-treatment and adjunct management over a period of 5 months.

METHODS

Sixty-six boys with haemophilia were followed. Age range was 10 months to 19 years; 70% of patients had severe haemophilia and 38 (58%) of all patients were on prophylaxis.

RESULTS

Twenty-nine patients experienced at least one episode of bleeding during study period which included 70 home bleeding episodes and 20 emergency department (ED) presentations. Secondary treatments occurred in 38% of all bleeding episodes. The incidence of re-bleeds occurring within 3 weeks of the initial bleeding episode was 11%.

CONCLUSIONS

Further study focusing on optimising treatment regimes for patients with haemophilia presenting with bleeding episodes is necessary.

摘要

背景

血友病是一种先天性出血性疾病,需要通过静脉输注来补充凝血因子 VIII(A型血友病)或凝血因子 IX(B型血友病)。儿童可以接受预防性治疗以预防出血,也可以采用按需治疗来处理特定的出血事件。

目的

本文的目的是对一组血友病患者进行前瞻性随访,以确定在5个月的时间内出血、再次出血、再次治疗及辅助治疗的发生率。

方法

对66名患血友病的男孩进行随访。年龄范围为10个月至19岁;70%的患者患有重度血友病,所有患者中有38名(58%)接受预防性治疗。

结果

29名患者在研究期间至少经历了一次出血事件,其中包括70次在家中发生的出血事件和20次到急诊科就诊。所有出血事件中有38%进行了二次治疗。在首次出血事件后3周内再次出血的发生率为11%。

结论

有必要进一步开展研究,重点优化对出现出血事件的血友病患者的治疗方案。

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