Prospective Reevaluation of the Association Between Thrombotic Diathesis and Legg-Perthes Disease

M. Hresko, P. McDougall, J. Gorlin, E. Vamvakas, J. Kasser, E. Neufeld
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引用次数: 59

Abstract

Background: Legg-Perthes disease is associated with ischemia of the capital femoral epiphysis in children. Thrombophilia has been implicated as a potential cause of the condition, and screening of patients with Legg-Perthes disease for thrombophilia has been recommended. We analyzed the value of screening for inherited thrombophilia in patients with Legg-Perthes disease by examining the association between Legg-Perthes disease and abnormalities in the thrombotic pathway.Methods: A random series of consecutive patients with Legg-Perthes disease were prospectively enrolled in this study. Assays for the detection of factor-V Leiden mutation and the plasma concentrations of protein C, protein S, antithrombin III, and lipoprotein (a) were performed on plasma samples from children with Legg-Perthes disease, and the results were compared with those for pooled plasma from normal controls. Plasma concentrations below the 95% midrange of the control values were classified as protein deficiencies. The estimated population frequency of each coagulation abnormality was compared with the proportion of the study group with the corresponding abnormality.Results: The proportion of abnormalities observed in the study group did not differ from the estimated population frequency for protein C, protein S, antithrombin III, or factor-V Leiden mutation. A lipoprotein (a) level of >30 mg/dL (>1.07 &mgr;mol/L) was found in 16% of the study group.Conclusions: Our data do not suggest that thrombotic diatheses due to deficiency of protein C, protein S, or antithrombin III or due to factor-V Leiden mutation are major causes of Legg-Perthes disease. The elevated levels of lipoprotein (a) in children with Legg-Perthes disease suggest that they may be at risk for atherosclerosis as adults.
血栓形成素质与Legg-Perthes病相关性的前瞻性再评价
背景:Legg-Perthes病与儿童股骨骨骺缺血有关。血栓形成被认为是该病的潜在病因,建议对Legg-Perthes病患者进行血栓形成筛查。我们通过研究Legg-Perthes病与血栓形成途径异常之间的关系,分析了筛查遗传性血栓性疾病在Legg-Perthes病患者中的价值。方法:前瞻性随机纳入一系列连续的Legg-Perthes病患者。对Legg-Perthes病患儿血浆样本进行了因子- v Leiden突变检测以及血浆中蛋白C、蛋白S、抗凝血酶III和脂蛋白(a)的浓度检测,并将结果与正常对照的合并血浆进行了比较。血浆浓度低于控制值的中间95%被归类为蛋白质缺乏。将每种凝血异常的估计人群频率与研究组出现相应异常的比例进行比较。结果:研究组中观察到的异常比例与估计的蛋白C、蛋白S、抗凝血酶III或因子v Leiden突变的人群频率没有差异。16%的研究组发现脂蛋白(A)水平>30 mg/dL (>1.07 mol/L)。结论:我们的数据并不表明由于蛋白C、蛋白S或抗凝血酶III缺乏或因子v Leiden突变引起的血栓性糖尿病是Legg-Perthes病的主要原因。患有Legg-Perthes病的儿童脂蛋白(a)水平升高表明他们成年后可能有动脉粥样硬化的风险。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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