血友病A患者环境因素与出血严重程度相关性分析

doi:10.3969/j.issn.1006-7795.2019.01.021

首都医科大学学报 ›› 2019, Vol. 40 ›› Issue (1): 119-123.doi: 10.3969/j.issn.1006-7795.2019.01.021

血友病A患者环境因素与出血严重程度相关性分析

陈昆, 杨林花, 王刚, 刘秀娥, 秦秀玉, 张夏林

山西医科大学第二医院血液科, 太原 030001

收稿日期:2018-11-08 出版日期:2019-01-21 发布日期:2019-01-23
通讯作者: 杨林花 E-mail:Yanglh5282@163.com
基金资助:
国家自然科学基金（81700182），山西省应用基础研究项目（201601D202094）。

Correlation between environmental factors and severity of bleeding in hemophilia A

Chen Kun, Yang Linhua, Wang Gang, Liu Xiuer, Qin Xiuyu, Zhang Xialin

Department of Hematology, the Second Hospital of Shanxi Medical University, Taiyuan 030001, China

Received:2018-11-08 Online:2019-01-21 Published:2019-01-23
Supported by:
This study was supported by National Natural Science Foundation of China(81700182),Applied Basic Research Program Shanxi Province(201601D202094).

摘要/Abstract

摘要： 目的探讨内含子22（intron 22 inversion，Inv22）和内含子1（intron 1 inversion，Inv1）倒位的重型血友病A（hemophilia A，HA）患者临床表现异质性的环境因素与出血严重程度相关性。方法回顾性分析2009年至2012年在山西省血友病管理中心登记的63例Inv22和Inv1阳性重型HA患者信息，详细记录其一般情况信息、首次出血年龄、首次关节出血年龄、首次治疗时间、年出血率（annualized bleed rate，ABR）等资料，利用单因素及多因素Logistic回归分析环境因素与临床表现异质性关系。结果 63例患者平均年龄为（17.75±8.51）岁，中位年龄为15岁，平均年出血率为（77.83±56.43）次，年出血率中位数为60次，单因素分析显示，年龄、首次治疗年龄、替代治疗方式在各组间差异有统计学意义（P<0.05），有序Logistic回归结果显示，首次关节出血年龄≤ 1岁的患者更容易发生出血（OR=13.97，95%CI：2.32~84.52），首次治疗年龄≤ 1岁的患者更不容易发生出血（OR=0.03，95%CI：0.01~0.49）。结论在Inv22和Inv1的重型血友病A患者中，首次关节出血年龄≤ 1岁的患者更容易发生出血，首次治疗年龄≤ 1岁的患者更不容易发生出血。首次关节出血年龄可作为HA出血严重程度的预测指标，建议给予更早的预防治疗，以避免后期严重出血的发生。

关键词: 血友病A, 内含子倒位, 临床表现异质性, 环境因素

Abstract: Objective To observe the correlation between the environmental factors of clinical heterogeneity and the severity of bleeding in patients of severe hemophilia A with intron 22 (Inv22) and intron 1 (Inv1) inversion. Methods A retrospective analysis of 63 patients of severe hemophilia A with Inv22 and Inv1 who were enrolled in the Shanxi Hemophilia Management Center from 2009 to 2012 was conducted.The general information, first bleeding age, first joint bleeding age, first treatment age, annualized bleed rate, were recorded in detail. One-way analysis and Logistic regressive were used to analysis the correlation between environmental factors and severity of bleeding. Results Among the 63 patients, the average age was (17.75±8.51) years, the median age was 15 years, the average annual bleeding rate was (77.83±56.43) times per year, and the median annualized bleeding rate was 60 times per year. The age, the first treatment age, and treatment regimen were statistically significant(P<0.05)within univariate analysis. Ordered Logistic regression analysis results showed that patients with first joint bleeding age ≤ 1 years were more likely to suffer from bleeding (OR=13.97, 95% CI:2.32-84.52), the first treatment of patients aged ≤ 1 year was less likely to suffer from bleeding (OR=0.03, 95% CI:0.01-0.49). Conclusion Among the patients of severe hemophilia A with Inv22 and Inv1, patients with the first joint bleeding age of ≤ 1 years were more likely to suffer from bleeding, and patients with age ≤ 1 years were less likely to suffer from bleeding. The first joint bleeding age can be used as an indicator of the severity of bleeding. It is recommended to give an earlier prophylaxis to avoid the occurrence of severe bleeding later.

Key words: hemophilia A, intron inversion, clinical heterogeneity, environmental factors

中图分类号:

陈昆, 杨林花, 王刚, 刘秀娥, 秦秀玉, 张夏林. 血友病A患者环境因素与出血严重程度相关性分析[J]. 首都医科大学学报, 2019, 40(1): 119-123.

Chen Kun, Yang Linhua, Wang Gang, Liu Xiuer, Qin Xiuyu, Zhang Xialin. Correlation between environmental factors and severity of bleeding in hemophilia A[J]. Journal of Capital Medical University, 2019, 40(1): 119-123.

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血友病A患者环境因素与出血严重程度相关性分析

Correlation between environmental factors and severity of bleeding in hemophilia A

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