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《HEAD-US 评分系统对血友病患者预防保护效果观察的影响:一项前瞻性、多中心、观察性研究》。

Impact of the HEAD-US Scoring System for Observing the Protective Effect of Prophylaxis in Hemophilia Patients: A Prospective, Multicenter, Observational Study.

机构信息

Ege University Children’s Hospital, Clinic of Children’s Health and Diseases, Division of Pediatric Hematology, İzmir, Turkey

Ege University Medical Faculty Hospital, Clinic of Radiology, Division of Hematology, İzmir, Turkey

出版信息

Turk J Haematol. 2021 Jun 1;38(2):101-110. doi: 10.4274/tjh.galenos.2021.2020.0717. Epub 2021 Jan 29.

Abstract

OBJECTIVE

This study aimed to observe the preventive effect of prophylactic treatment on joint health in people with hemophilia (PwH) and to investigate the importance of integration of ultrasonographic examination into clinical and radiological evaluation of the joints.

MATERIALS AND METHODS

This national, multicenter, prospective, observational study included male patients aged ≥6 years with the diagnosis of moderate or severe hemophilia A or B from 8 centers across Turkey between January 2017 and March 2019. Patients were followed for 1 year with 5 visits (baseline and 3, 6, 9, and 12 month visits). The Hemophilia Joint Health Score (HJHS) was used for physical examination of joints, the Pettersson scoring system was used for radiological assessment, point-of-care (POC) ultrasonography was used for bilateral examinations of joints, and the Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score was used for evaluation of ultrasonography results.

RESULTS

Seventy-three PwH, of whom 62 had hemophilia A and 11 had hemophilia B, were included and 24.7% had target joints at baseline. The HJHS and HEAD-US scores were significantly increased at the 12 month in all patients. These scores were also higher in the hemophilia A subgroup than the hemophilia B subgroup. However, in the childhood group, the increment of scores was not significant. The HEAD-US total score was significantly correlated with both the HJHS total score and Pettersson total score at baseline and at the 12 month.

CONCLUSION

The HEAD-US and HJHS scoring systems are valuable tools during follow-up examinations of PwH and they complement each other. We suggest that POC ultrasonographic evaluation and the HEAD-US scoring system may be integrated into differential diagnosis of bleeding and long-term monitoring for joint health as a routine procedure.

摘要

目的

本研究旨在观察预防性治疗对血友病患者关节健康的预防作用,并探讨将超声检查纳入关节临床和影像学评估的重要性。

材料和方法

这是一项全国性、多中心、前瞻性、观察性研究,纳入了 2017 年 1 月至 2019 年 3 月期间土耳其 8 个中心的年龄≥6 岁、诊断为中重度血友病 A 或 B 的男性患者。患者接受为期 1 年的随访,共进行 5 次就诊(基线和 3、6、9 和 12 个月就诊)。采用血友病关节健康评分(HJHS)进行关节体格检查,采用 Pettersson 评分系统进行影像学评估,采用即时护理(POC)超声进行双侧关节检查,采用血友病早期关节病超声检测(HEAD-US)评分进行超声检查结果评估。

结果

共纳入 73 例血友病患者,其中 62 例为血友病 A,11 例为血友病 B,24.7%的患者在基线时有靶关节。所有患者在 12 个月时 HJHS 和 HEAD-US 评分均显著升高。血友病 A 亚组的这些评分高于血友病 B 亚组。然而,在儿童组中,评分的增加并不显著。HEAD-US 总分与基线和 12 个月时的 HJHS 总分和 Pettersson 总分均显著相关。

结论

HEAD-US 和 HJHS 评分系统是血友病患者随访检查中有用的工具,它们相互补充。我们建议将 POC 超声评估和 HEAD-US 评分系统纳入出血的鉴别诊断和关节健康的长期监测,作为常规程序。

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