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肥胖对 1 型血管性血友病患者因子 VIII 和 von Willebrand 因子水平的影响:ATHNdataset 的分析。

Impact of obesity on factor VIII and von Willebrand factor levels in patients with Type 1 von Willebrand disease and low von Willebrand factor: An analysis of the ATHNdataset.

机构信息

University at Buffalo, Roswell Park Oishei Children's Cancer and Blood Disorders Program, Buffalo, NY, USA.

Western New York BloodCare, Buffalo, New York, USA.

出版信息

Haemophilia. 2022 Jan;28(1):109-116. doi: 10.1111/hae.14467. Epub 2021 Dec 7.

Abstract

INTRODUCTION

Obesity is associated with endothelial dysfunction, haemostatic and fibrinolytic disturbances, however the impact of obesity on von Willebrand factor (VWF) is unclear.

AIM

The aim of this study was to test our hypothesis that the prevalence of obesity is higher among participants with low VWF (LVWF) compared to type 1 von Willebrand disease (T1VWD).

METHODS

A retrospective review of the ATHNdataset as of March 2018 was performed. Participants were categorized as T1VWD if their VWF ristocetin cofactor activity was 30 IU/dL and LVWF if the values were 30-50 IU/dL, and by the NIH definitions for body mass index (BMI) for adult participants (≥ 18 years of age) or BMI z-score for paediatric participants (< 18 years).

RESULTS

The prevalence of obesity was not significantly different between adults with T1VWD (n = 186) and LVWF (n = 362) (32% vs 36%; p = .345). The mean factor VIII (FVIII) increased with increasing BMIs in both groups. In the paediatric cohort (T1VWD, n = 583; LVWF, n = 1702), there was no difference in the prevalence of obesity, but BMI was positively correlated with mean FVIII (p < .001). Children < 10 years were 27.6% more likely to be diagnosed with T1VWD compared to > 10 years.

CONCLUSION

Among participants in the ATHNdataset, the prevalence of obesity was similar among those with LVWF and T1VWD. However, higher BMI levels were associated with elevated FVIII. Further research is needed to evaluate the impact of obesity on bleeding phenotype and treatment practices.

摘要

简介

肥胖与血管内皮功能障碍、止血和纤维蛋白溶解紊乱有关,但肥胖对血管性血友病因子(VWF)的影响尚不清楚。

目的

本研究旨在验证我们的假设,即与 1 型血管性血友病(T1VWD)相比,低 VWF(LVWF)患者中肥胖的患病率更高。

方法

对截至 2018 年 3 月的 ATHNdataset 进行回顾性分析。如果 VWF 瑞斯托霉素辅因子活性为 30IU/dL,则将参与者归类为 T1VWD;如果值为 30-50IU/dL,则归类为 LVWF;并根据 NIH 对成年参与者(≥18 岁)的体重指数(BMI)或儿科参与者(<18 岁)的 BMI z 分数的定义进行分类。

结果

T1VWD(n=186)和 LVWF(n=362)成人患者中肥胖的患病率无显著差异(32%vs36%;p=0.345)。两组的 FVIII 因子均随 BMI 的增加而增加。在儿科队列中(T1VWD,n=583;LVWF,n=1702),肥胖的患病率无差异,但 BMI 与平均 FVIII 呈正相关(p<0.001)。<10 岁的儿童比>10 岁的儿童更有可能被诊断为 T1VWD,其可能性高 27.6%。

结论

在 ATHNdataset 参与者中,LVWF 和 T1VWD 患者的肥胖患病率相似。然而,更高的 BMI 水平与 FVIII 升高相关。需要进一步研究评估肥胖对出血表型和治疗实践的影响。

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