Suppr超能文献

预防性输注凝血因子VIII联合个性化康复对血友病儿童关节健康和生活质量的影响。

The effect of prophylactic FVIII infusion combined with personalized rehabilitation on joint health and quality of life in children with hemophilia.

作者信息

Sun Yaojin, Zhang Qian, Gu Xiaojie, Fang Yongjun, Huang Jie, Zhang Heng

机构信息

Department of Rehabilitation Medicine, Children's Hospital of Nanjing Medical University, Nanjing, China.

Department of Ultrasound, Children's Hospital of Nanjing Medical University, Nanjing, China.

出版信息

Front Pediatr. 2025 Jul 10;13:1578617. doi: 10.3389/fped.2025.1578617. eCollection 2025.

Abstract

BACKGROUND

Hemophilia, a genetic disorder characterized by deficiencies in clotting factors VIII or IX, often results in joint and muscle bleeding, leading to hemophilic arthropathy and reduced quality of life. While multidisciplinary teams (MDTs) are widely used in hemophilia management, personalized rehabilitation strategies for pediatric patients remain underexplored. This study aimed to evaluate a 12-week individualized rehabilitation program targeting joint health in pediatric hemophilia patients.

METHODS

This prospective study enrolled 59 children with hemophilia, implementing tailored rehabilitation plans based on specific joint pathologies. Assessments before and after treatment included joint ultrasound examinations (HEAD-US-C), Hemophilia Joint Health Scores (HJHS 2.1), and the CHO-KLAT tool for quality of life. Bleeding frequency was also recorded.

RESULTS

Post-treatment, median HEAD-US-C scores decreased from 5.00 to 1.00 ( < 0.001), and HJHS 2.1 scores decreased from 9.00 to 2.00 ( < 0.001). CHO-KLAT scores increased from 71.51 to 79.15 ( < 0.001). Median bleeding episodes dropped from 3.00 to 0.00 ( < 0.001), indicating enhanced joint health and reduced bleeding frequency.

CONCLUSION

Regular prophylactic FVIII administration combined with individualized rehabilitation significantly reduces joint bleeding and enhances joint function and quality of life in children with hemophilia. The MDT approach is integral to comprehensive care, but further studies are needed to assess the long-term efficacy and safety of this therapeutic strategy.

摘要

背景

血友病是一种以凝血因子 VIII 或 IX 缺乏为特征的遗传性疾病,常导致关节和肌肉出血,进而引发血友病性关节病并降低生活质量。虽然多学科团队(MDT)广泛应用于血友病管理,但针对儿科患者的个性化康复策略仍未得到充分探索。本研究旨在评估一项针对儿科血友病患者关节健康的为期 12 周的个性化康复计划。

方法

这项前瞻性研究纳入了 59 名血友病儿童,根据特定的关节病变实施量身定制的康复计划。治疗前后的评估包括关节超声检查(HEAD-US-C)、血友病关节健康评分(HJHS 2.1)以及用于生活质量的 CHO-KLAT 工具。还记录了出血频率。

结果

治疗后,HEAD-US-C 评分中位数从 5.00 降至 1.00(<0.001),HJHS 2.1 评分从 9.00 降至 2.00(<0.001)。CHO-KLAT 评分从 71.51 升至 79.15(<0.001)。出血发作中位数从 3.00 降至 0.00(<0.001),表明关节健康得到改善且出血频率降低。

结论

定期预防性输注 FVIII 联合个性化康复可显著减少血友病儿童的关节出血,并增强关节功能和生活质量。多学科团队方法是综合护理的重要组成部分,但需要进一步研究来评估这种治疗策略的长期疗效和安全性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5d48/12287007/4383e89a8127/fped-13-1578617-g001.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验