关键词: annual bleeding rate arthropathy haemophilia pharmacokinetics rare disease sport

Mesh : Humans Hemophilia A / prevention & control Factor VIII / pharmacokinetics Prospective Studies Hemorrhage / prevention & control Exercise

来  源:   DOI:10.1111/hae.14965

Abstract:
BACKGROUND: Regular physical activity (PA) is recommended for patients with haemophilia (PwH). For PwH it is crucial to ensure a sufficient factor level to prevent PA-induced bleedings. However, there is a gap in the literature dealing with specific factor levels, which are needed when performing specific types of PA.
OBJECTIVE: To provide data on factor VIII (FVIII) levels at the start of PA performed by PwH.
METHODS: In this prospective 12-month real-world observational study, 23 PwH recorded every PA they performed and the FVIII levels at the start of the PA using a pharmacokinetic application. PA types were clustered according to the collision and injury risk into three categories (Cat I = low, Cat II = medium, Cat III = high risk). Haemophilia Joint Health Scores (HJHS) were performed at baseline, after 6 and 12 months.
RESULTS: 795 PA sessions of Cat I, 193 of Cat II, and 23 of Cat III were documented. FVIII levels at the start of PA were different between categories (Cat I: 29.8 ± 32.1%, Cat II: 38.3 ± 33.4%, Cat III: 86.6 ± 29.2%). Out of all PA sessions, 145 (14%) were performed at a factor level of ≤3%. Three PA-induced bleeding occurred. Baseline HJHS was 14.5 ± 13.6 points and did not change throughout the study.
CONCLUSIONS: This study provides real-life data on FVIII levels at the start of 1011 PA sessions. PwH are mainly active in low-risk sports with higher FVIII levels observed in Cat II and III, respectively. Only three PA-induced bleeding occurred, even though several PA were started with low FVIII levels.
摘要:
背景:血友病(PwH)患者建议定期进行体育锻炼(PA)。对于PwH,至关重要的是确保足够的因子水平以防止PA诱导的出血。然而,在处理特定因素水平的文献中存在差距,执行特定类型的PA时需要。
目的:提供PwH在PA开始时因子VIII(FVIII)水平的数据。
方法:在这项为期12个月的前瞻性真实世界观察研究中,23PwH使用药代动力学应用记录了他们进行的每个PA以及PA开始时的FVIII水平。PA类型根据碰撞和伤害风险分为三类(类别I=低,第II类=中等,第III类=高风险)。血友病联合健康评分(HJHS)在基线进行,6个月和12个月后。
结果:第I类的795次PA会议,193猫II和23的猫III被记录。PA开始时的FVIII水平在类别之间是不同的(类别I:29.8±32.1%,第II类:38.3±33.4%,第III类:86.6±29.2%)。在所有PA会议中,145(14%)在≤3%的因子水平下进行。3例发生PA诱导的出血。基线HJHS为14.5±13.6分,在整个研究过程中没有变化。
结论:本研究提供了1011次PA会议开始时FVIII水平的真实数据。PwH主要在低风险运动中活跃,在CatII和III中观察到较高的FVIII水平,分别。仅发生3次PA诱导的出血,即使一些PA开始与低FVIII水平。
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