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A 型和 B 型血友病-通过磁共振成像评估瑞典预防方案。

Haemophilia A and B - evaluation of the Swedish prophylactic regimen by magnetic resonance imaging.

机构信息

Department of Clinical Sciences Lund (IKVL) - Radiology, Lund University, Lund, Sweden and Dept. of Medical Imaging and Physiology, Skåne University Hospital, Lund/Malmö, Sweden.

Coagulation Centre, Department of Medicine/Section of Hematology and Coagulation, Sahlgrenska University Hospital, Gothenburg, Sweden.

出版信息

Haemophilia. 2023 Jan;29(1):193-198. doi: 10.1111/hae.14693. Epub 2022 Dec 5.

Abstract

INTRODUCTION

Sweden has been a pioneer in the prophylactic treatment of haemophilia. Magnetic resonance imaging (MRI) can detect small changes in joints and can therefore give an indication of a risk of developing arthropathy.

AIM

To use MRI to evaluate the outcome of the Swedish 'high-dose regimen' and correlate the findings to age, bleeds, joint score and physical activity.

METHODS

The study group comprised 48 Swedish male patients, mean age 25 years (range 12-33 years), with severe or moderate haemophilia A or B. Data on the Haemophilia Joint Health Score (HJHS) were available and physical activity was evaluated by a self-reported questionnaire.

RESULTS

MRI score was recorded in 188 joints. Twenty out of 48 patients had a score of ≥1 (range 1-13) in 31 joints of which 3/31 scores were in the knees and 28/31 in the ankles. No correlation was found between the number of recorded bleeds and the MRI score or between HJHS and MRI score. There was no correlation between the physical activity and the number of joint bleeds per se, but a trend (OR 3.0) that those most physically active (19/48; 39.6%), more frequently had an MRI score of ≥1 with an overweight for the right ankle.

CONCLUSION

The Swedish prophylactic model offers protection against haemophilia joint arthropathy but will still not prevent osteochondral changes in some patients at young age. MRI of the ankles can signal risk of future arthropathy and indicate need to modify the prophylactic regimen.

摘要

简介

瑞典一直是预防性治疗血友病的先驱。磁共振成像(MRI)可以检测到关节的微小变化,因此可以提示发生关节病的风险。

目的

使用 MRI 评估瑞典“高剂量方案”的结果,并将结果与年龄、出血、关节评分和身体活动相关联。

方法

研究组包括 48 名瑞典男性患者,平均年龄 25 岁(范围 12-33 岁),患有严重或中度血友病 A 或 B。可获得血友病关节健康评分(HJHS)的数据,身体活动通过自我报告问卷进行评估。

结果

在 188 个关节中记录了 MRI 评分。48 名患者中有 20 名在 31 个关节中的得分≥1(范围 1-13),其中 3/31 得分在膝关节,28/31 在踝关节。记录的出血次数与 MRI 评分之间或 HJHS 与 MRI 评分之间均无相关性。身体活动与关节出血次数本身之间没有相关性,但存在趋势(OR 3.0),即活动量最大的患者(48 例中的 19 例;39.6%),超重的右踝关节发生 MRI 评分≥1 的情况更频繁。

结论

瑞典的预防性治疗模式可预防血友病性关节病,但仍无法预防某些年轻患者的软骨骨病变。踝关节 MRI 可提示未来发生关节病的风险,并表明需要修改预防性治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be5c/10107095/7a7cc28d5165/HAE-29-193-g001.jpg

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