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轻度血友病患者关节病的比较负担:瑞典一项基于登记处的研究

Comparative burden of arthropathy in mild haemophilia: a register-based study in Sweden.

作者信息

Osooli M, Lövdahl S, Steen Carlsson K, Knobe K, Baghaei F, Holmström M, Astermark J, Berntorp E

机构信息

Centre for Thrombosis and Haemostasis, Skane University Hospital, Malmo, Sweden.

Department of Translational Sciences, Faculty of Medicine, Lund University, Lund, Sweden.

出版信息

Haemophilia. 2017 Mar;23(2):e79-e86. doi: 10.1111/hae.13166. Epub 2017 Feb 2.

Abstract

INTRODUCTION

Mild haemophilia is a congenital bleeding disorder affecting males. The burden of arthropathy in mild haemophilia has not been comprehensively described.

AIM

The aim of this study was to compare the incidence, age at diagnosis and surgery for arthropathy and related hospitalizations between people with mild haemophilia and the general population in Sweden.

METHODS

This was a register-based cohort study. Eligible participants were those with mild haemophilia born between 1941 and 2008 and a randomly selected, birthdate and sex-matched comparison group from the general population. Follow-up was from birth (or earliest 1984) until death, emigration or end of the study in 2008. Data on arthropathy were obtained from a national patient register. Negative binomial and competing risk regression and Kaplan-Meier estimate curves were used in the analysis.

RESULTS

Overall, 315 people with haemophilia and 1529 people in the comparison group were included. Participants with haemophilia born between 1984 and 2008 had a ninefold (95% CI: 3.3-27.2) and 16-fold (95% CI: 6.7-36.5) increased incidence of arthropathy-related hospital admission and arthropathy diagnosis respectively. None in this cohort underwent surgery. Among participants with haemophilia born prior to 1984, the rates of arthropathy diagnosis and surgery of the index joints (knee, elbow, ankle) were increased twofold (95% CI: 1.0-3.2) and fivefold (95% CI: 1.7-17.8) respectively.

CONCLUSION

Our data suggested a higher burden of arthropathy among individuals with mild haemophilia compared to the general population. Further research should investigate the need for targeted joint screening programmes among individuals with mild haemophilia.

摘要

引言

轻度血友病是一种影响男性的先天性出血性疾病。轻度血友病关节病的负担尚未得到全面描述。

目的

本研究的目的是比较瑞典轻度血友病患者与普通人群之间关节病的发病率、诊断年龄、手术情况以及相关住院情况。

方法

这是一项基于登记的队列研究。符合条件的参与者为1941年至2008年出生的轻度血友病患者,以及从普通人群中随机选取的、出生日期和性别匹配的对照组。随访从出生(或最早1984年)开始,直至死亡、移民或2008年研究结束。关节病数据来自国家患者登记处。分析中使用了负二项式和竞争风险回归以及Kaplan-Meier估计曲线。

结果

总体而言,纳入了315名血友病患者和1529名对照组人员。1984年至2008年出生的血友病患者关节病相关住院和关节病诊断的发病率分别增加了9倍(95%置信区间:3.3 - 27.2)和16倍(95%置信区间:6.7 - 36.5)。该队列中无人接受手术。在1984年之前出生的血友病患者中,主要关节(膝、肘、踝)的关节病诊断率和手术率分别增加了2倍(95%置信区间:1.0 - 3.2)和5倍(95%置信区间:1.7 - 17.8)。

结论

我们的数据表明,与普通人群相比,轻度血友病患者的关节病负担更高。进一步的研究应调查轻度血友病患者中针对性关节筛查项目的必要性。

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