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因子 XI 缺乏症患者的出血预测因素

Bleeding predictors in factor-XI-deficient patients.

作者信息

Brenner B, Laor A, Lupo H, Zivelin A, Lanir N, Seligsohn U

机构信息

Thrombosis & Hemostasis Unit, Rambam Medical Center, Technion, Haifa, Israel.

出版信息

Blood Coagul Fibrinolysis. 1997 Nov;8(8):511-5. doi: 10.1097/00001721-199711000-00005.

Abstract

Bleeding in factor-XI-deficient patients is mainly injury-related. Parameters influencing bleeding particularly in patients with minor factor XI deficiency have not been defined. We utilized a logistic regression model to analyze parameters influencing bleeding tendency in subjects from 45 families with factor XI deficiency. Bleeding manifestations were documented in 58% of 26 homozygous or doubly heterozygous factor-XI-deficient patients, in 20% of 46 heterozygous factor-XI-deficient patients and in 9% of 47 family members with a normal factor XI genotype. Odds ratios for bleeding in homozygotes or double heterozygotes were 13 and in heterozygotes 2.6 with 95% confidence intervals of 3.8-45 and 0.8-9.0, respectively. Bleeding correlated negatively with factor XI level (r = -0.36, P = 0.0001) with major factor XI deficiency being a strong predictor of bleeding (P = 0.011). Minor factor XI deficiency and blood group O slightly contributed to bleeding. Although factor VIII and factor XI levels were correlated (r = 0.48, P = 0.0001), levels of factor VIII and von Willebrand factor were not predictors of bleeding. Bleeding was more common following operative procedure involving mucosal membranes (P < 0.01). The designed model enabled prediction of bleeding manifestations with an overall accuracy of 78% and 82% in heterozygotes.

摘要

因子 XI 缺乏症患者的出血主要与损伤相关。影响出血的参数,尤其是轻度因子 XI 缺乏症患者的出血参数尚未明确。我们利用逻辑回归模型分析了 45 个因子 XI 缺乏症家族受试者中影响出血倾向的参数。在 26 例纯合子或双重杂合子因子 XI 缺乏症患者中,58%有出血表现;在 46 例杂合子因子 XI 缺乏症患者中,20%有出血表现;在 47 例因子 XI 基因型正常的家庭成员中,9%有出血表现。纯合子或双重杂合子出血的优势比为 13,杂合子为 2.6,95%置信区间分别为 3.8 - 45 和 0.8 - 9.0。出血与因子 XI 水平呈负相关(r = -0.36,P = 0.0001),重度因子 XI 缺乏是出血的强预测因素(P = 0.011)。轻度因子 XI 缺乏和 O 型血对出血有轻微影响。虽然因子 VIII 和因子 XI 水平相关(r = 0.48,P = 0.0001),但因子 VIII 和血管性血友病因子水平不是出血的预测因素。涉及黏膜的手术操作后出血更常见(P < 0.01)。所设计的模型能够预测出血表现,杂合子的总体准确率为 78%和 82%。

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