Familial elevated factor VIII in children with symptomatic venous thrombosis and post-thrombotic syndrome:: Results of a multicenter study
Familial elevated factor VIII in children with symptomatic venous thrombosis and post-thrombotic syndrome:: Results of a multicenter study
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DOI：
10.1161/01.atv.0000227510.36653.ed
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发表时间：
2006-08-01
影响因子：
8.7
通讯作者：
Nowak-Goettl, Ulrike
中科院分区：
文献类型：
--
作者：
Kreuz, Wolfhart;Stoll, Monika;Nowak-Goettl, Ulrike
评价因子（F）VIII在儿童非癌相关静脉血栓形成（DVT）、血栓后综合征（PTS）或复发性DVT中的作用。方法和结果测定白色患者和年龄和性别匹配的健康对照者的FVIII水平。采用SOLAR中的方差分量法估计了99个家系的因子VIII遗传度。103例患者组的FVIII中位值高于206例对照组[FVIII：Ag，115 vs 96 IU/dL，P < 0.0001; FVIII：C，119 vs 106 IU/dL，P=0.0009]，并且纤维蛋白原校正的FVIII水平升高的比值比（OR）显著增加[FVIII >第0百分位数vs低于临界值的数值：FVIII：Ag，OR 4.3，95%置信区间（CI）1.5 - 12.1; FVIII：C，OR 5.5，CI 2.03 - 15.06]。59名儿童中有19名发生PTS，5名持续存在。8例患者出现DVT复发。计算FVIII：Ac水平（h2，0.48 +/- 0.15，P=0.0008; c2，0.21）和FVIII：C水平（h2，0.61 +/- 0.15，P <0.0001; c2，0.41）的遗传（h2）/家庭（c2）成分。当将h2和c2的估计，FVIII：Ag水平的表型方差主要是由h2解释，而c2保持显着的模型中的FVIII：C（P=0.00002）。
Objective-To evaluate the role of factor (F) VIII in children with non-cancer related venous thrombosis (DVT), post-thrombotic syndrome (PTS) or recurrent DVT.Methods and Results-FVIII levels were measured in White patients and age- and gender-matched healthy controls. Heritability of factor VIII was estimated in 99 pedigrees by the variance component method implemented in SOLAR. The group of 103 patients showed higher median values of FVIII than 206 controls [FVIII:Ag, 115 versus 96 IU/dL, P < 0.0001: FVIII:C, 119 versus 106 IU/dL, P=0.0009], and had a significantly increased odds ratio (OR) for fibrinogen-adjusted elevated FVIII levels [FVIII > 0th percentile versus values below the cut-off: FVIII:Ag, OR 4.3, 95% confidence interval (CI) 1.5 to 12.1; FVIII:C, OR 5.5, CI 2.03 to 15.06]. PTS occurred in 19 of 59 children and persisted in 5 individuals. Recurrent DVT was seen in 8 patients. The heritable(h2)/household(c2) components were calculated for FVIII:Ac, levels (h2, 0.48 +/- 0.15, P=0.0008; c2, 0.21), and FVIII:C (h2, 0.61 +/- 0.15, P < 0.0001; c2, 0.41). When incorporating h2 and c2 in the estimate, the phenotypic variance for FVIII:Ag levels is predominantly explained by h2, whereas c2 stayed significant in the model for FVIII:C (P=0.00002).Conclusions-Elevated FVIII levels increase the DVT-risk in children.