[一项关于甲型血友病患儿凝血因子 VIII 抑制剂的横断面调查]

[A cross-sectional survey of coagulation factor VIII inhibitor in children with hemophilia A].

作者信息

Wei Qiqi, Li Gang, Tang Ling, Chen Zhenping, Zhen Yingzi, Wu Xinyi, Zhang Ningning, Zhang Jishui, Yu Guoxia, Wu Runhui

机构信息

Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.

Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China? Email:

出版信息

Zhonghua Er Ke Za Zhi. 2014 Feb;52(2):99-102.

PMID:24739719

Abstract

OBJECTIVE

To study the current situation of coagulation factor VIII (FVIII) inhibitor development in children with hemophilia A (HA) through a cross-sectional survey, and to explore the risk factors of inhibitor development in order to provide evidence for further prevention and management strategies.

METHOD

The clinical data of outpatients with hemophilia A in Beijing Children's Hospital seen from November 2012 to May 2013 were collected, FVIII inhibitor was screened and analyzed its risk factors.

RESULT

A total of 102 HA children were enrolled, 5 were mild cases, 32 were moderate, and 65 were severe cases; the median age on enrollment was 55.5 (3.0-200.0) months:19(18.6%) of patients had inhibitors and 9 (8.8%) had low-titer inhibitors, 10 (9.8%) had high-titer inhibitors. Receiving FVIII treatment for life-threatening bleeding (P = 0.03) ,OR 4.10 (95%CI:1.17-14.32) was a risk factor for inhibitor generation and patients within 20 exposure days have more chances of inhibitor development (P = 0.04) ,OR 3.32 (95%CI:1.02-10.86) . High and intense FVIII exposure within short term was the risk factor for high titer inhibitor development (P = 0.01) ,OR 5.25 (95%CI:1.45-21.92) .

CONCLUSION

Intense FVIII exposure for severe hemorrhage was the risk factor of inhibitors development especially of high titer inhibitors.

摘要

目的

通过横断面调查研究甲型血友病（HA）患儿凝血因子 VIII（FVIII）抑制物产生的现状，并探讨抑制物产生的危险因素，为进一步的预防和管理策略提供依据。

方法

收集 2012 年 11 月至 2013 年 5 月在北京儿童医院就诊的甲型血友病门诊患者的临床资料，筛查 FVIII 抑制物并分析其危险因素。

结果

共纳入 102 例 HA 患儿，轻度 5 例，中度 32 例，重度 65 例；入组时中位年龄为 55.5（3.0 - 200.0）个月；19 例（18.6%）患者产生抑制物，其中 9 例（8.8%）为低滴度抑制物，10 例（9.8%）为高滴度抑制物。因危及生命的出血接受 FVIII 治疗（P = 0.03），比值比（OR）为 4.10（95%可信区间：1.17 - 14.32）是抑制物产生的危险因素，且在 20 次暴露日内的患者产生抑制物的机会更多（P = 0.04），OR 为 3.32（95%可信区间：1.02 - 10.86）。短期内高剂量和高强度的 FVIII 暴露是高滴度抑制物产生的危险因素（P = 0.01），OR 为 5.25（95%可信区间：1.45 - 21.92）。