A cross-sectional study of bleeding phenotype in haemophilia A carriers.
A cross-sectional study of bleeding phenotype in haemophilia A carriers.
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DOI：
10.1111/bjh.13423
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发表时间：
2015-07
影响因子：
6.5
通讯作者：
Sidonio RF Jr
中科院分区：
文献类型：
--
作者：
Paroskie A;Gailani D;DeBaun MR;Sidonio RF Jr
A型血友病携带者历来被认为具有正常的止血功能。然而，最近的数据表明，尽管因子VIII正常，血友病A携带者表现出出血倾向增加。我们检验了专性血友病携带者出血症状增加的假设。横断面研究进行比较血友病A携带者和正常妇女。问卷评估包括一般出血问卷、浓缩MCMDM-1VWD出血评估工具和图像出血评估图（PBAC）。实验室评估包括全血细胞计数、凝血酶原时间、活化的部分凝血活素时间、纤维蛋白原活性、FVIII活性（FVIII:C）、血管性血友病因子抗原水平、瑞斯托司汀辅助因子、血小板功能分析仪-100™和ABO血型。A型血友病携带者44例，对照组43例。人口统计学特征相似。实验室结果显示，只有FVIII:C有统计学差异（82.5%比134%，p值< 0.001）。携带者报告了更多的出血事件，并且浓缩MCMDM-1 VWD出血评分（5比1，p值< 0.001）和PBAC评分（423比182.5，p值= 0.018）在携带者中都显着更高。与正常女性相比，A型血友病携带者出血症状增加。需要进一步的研究来充分了解该人群的出血表型并优化临床管理。
Haemophilia A carriers have historically been thought to demonstrate normal haemostasis. However, recent data demonstrates that despite normal factor VIII, haemophilia A carriers demonstrate an increased bleeding tendency. We tested the hypothesis that obligate haemophilia carriers demonstrate an increase in bleeding symptoms. A cross sectional study was performed comparing haemophilia A carriers to normal women. Questionnaire assessment included a general bleeding questionnaire, condensed MCMDM-1VWD bleeding assessment tool and Pictorial Bleeding Assessment Chart (PBAC). Laboratory assessment included complete blood count, prothrombin time, activated partial thromboplastin time, fibrinogen activity, FVIII activity (FVIII:C), von Willebrand factor antigen level, ristocetin cofactor, platelet function analyser-100™ and ABO blood type. 44 haemophilia A carriers and 43 controls were included. Demographic features were similar. Laboratory results demonstrated a statistically significant difference only in FVIII:C (82.5 versus 134%, p value < 0.001). Carriers reported a higher number of bleeding events, and both condensed MCMDM-1 VWD bleeding scores (5 versus 1, p value < 0.001) and PBAC scores (423 versus 182.5, p value = 0.018) were significantly higher in carriers. Haemophilia A carriers exhibit increased bleeding symptoms when compared to normal women. Further studies are necessary to fully understand the bleeding phenotype in this population and optimize clinical management.
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影响因子：
20.3
作者：
Plug, Iris;Mauser-Bunschoten, Eveline P.;Rosendaal, Frits R.
通讯作者：
Rosendaal, Frits R.
影响因子：
1.2
作者：
Paroskie, Allison;Oso, Olatunde;Sidonio, Robert F., Jr.
通讯作者：
Sidonio, Robert F., Jr.
影响因子：
3.9
作者：
Miesbach, W.;Alesci, S.;Oldenburg, J.
通讯作者：
Oldenburg, J.
DOI：
10.1111/j.1471-0528.1990.tb16249.x
发表时间：
1990-08-01
期刊：
BRITISH JOURNAL OF OBSTETRICS AND GYNAECOLOGY
影响因子：
--
作者：
HIGHAM, JM;OBRIEN, PMS;SHAW, RW
通讯作者：
SHAW, RW
影响因子：
1.1
作者：
Olsson, Anna;Hellgren, Margareta;Baghaei, Fariba
通讯作者：
Baghaei, Fariba