凝血因子V莱顿纯合子、呼吸困难与肺功能减退

Factor V leiden homozygosity, dyspnea, and reduced pulmonary function.

作者信息

Juul Klaus, Tybjaerg-Hansen Anne, Mortensen Jann, Lange Peter, Vestbo Jørgen, Nordestgaard Børge G

机构信息

Department of Clinical Biochemistry, Herlev University Hospital, Herlev, Denmark.

出版信息

Arch Intern Med. 2005 Sep 26;165(17):2032-6. doi: 10.1001/archinte.165.17.2032.

DOI:10.1001/archinte.165.17.2032

PMID:16186475

Abstract

BACKGROUND

Factor V Leiden homozygosity predisposes patients to deep venous thrombosis and major pulmonary thromboembolism. Consequently, factor V Leiden homozygosity could, via unrecognized repeated minor pulmonary thromboemboli, cause chronic pulmonary disease. We tested the hypothesis that factor V Leiden homozygosity is associated with pulmonary symptoms and signs.

METHODS

We studied a general population sample of 9253 individuals from the Copenhagen City Heart Study who were examined in 1991-1994. Of these, 6475 participants were also examined in 1976-1978 and/or 1981-1983. End points were dyspnea and lung function.

RESULTS

Among 20 factor V Leiden homozygotes, a mean +/- SD of 32% +/- 11% had severe dyspnea compared with 6% +/- 0.3% of 8534 noncarriers (chi(2) test; P<.001). The corresponding adjusted odds ratio for severe dyspnea was 5.4 (95% confidence interval, 1.9-15.7). During follow-up, forced expiratory volume in 1 second and forced vital capacity were 5% to 10% lower in homozygotes vs noncarriers (analysis of variance; P = .003 and P = .03). The annual mean +/- SD loss of forced expiratory volume in 1 second and forced vital capacity was 39 +/- 8 mL/y and 35 +/- 8 mL/y in homozygotes vs 21 +/- 10 mL/y and 15 +/- 10 mL/y in noncarriers (t test; P = .03 and P = .04), respectively. Factor V Leiden heterozygosity (n = 699) did not influence pulmonary symptoms and signs.

CONCLUSION

We demonstrate a previously unrecognized clinical presentation of factor V Leiden homozygosity with severe dyspnea and decreased pulmonary function.

摘要

背景

凝血因子V莱顿纯合子使患者易患深静脉血栓形成和严重肺血栓栓塞。因此，凝血因子V莱顿纯合子可能通过未被识别的反复轻微肺血栓栓塞导致慢性肺部疾病。我们检验了凝血因子V莱顿纯合子与肺部症状和体征相关的假说。

方法

我们研究了哥本哈根市心脏研究中9253名个体的一般人群样本，这些个体在1991年至1994年接受了检查。其中，6475名参与者在1976年至1978年和/或1981年至1983年也接受了检查。终点指标为呼吸困难和肺功能。

结果

在20名凝血因子V莱顿纯合子中，平均±标准差为32%±11%的人有严重呼吸困难，而8534名非携带者中这一比例为6%±0.3%（χ²检验；P<0.001）。严重呼吸困难的相应校正比值比为5.4（95%置信区间，1.9 - 15.7）。在随访期间，纯合子的一秒用力呼气量和用力肺活量比非携带者低5%至10%（方差分析；P = 0.003和P = 0.03）。纯合子一秒用力呼气量和用力肺活量的年均±标准差下降分别为39±8 mL/年和35±8 mL/年，而非携带者分别为21±10 mL/年和15±10 mL/年（t检验；P = 0.03和P = 0.04）。凝血因子V莱顿杂合子（n = 699）不影响肺部症状和体征。