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克兰费尔特综合征伴性腺功能减退时纤维蛋白凝块溶解减少。

Reduced fibrin clot lysis in Klinefelter syndrome associated with hypogonadism.

作者信息

Chang Simon, Goszczak Arkadiusz J, Skakkebæk Anne, Fedder Jens, Bojesen Anders, Bor M Vakur, de Maat Moniek P M, Gravholt Claus H, Münster Anna-Marie B

机构信息

Unit for Thrombosis Research, Department of Regional Health Research, University of Southern Denmark, Odense, Denmark.

Department of Clinical Biochemistry, Hospital of South West Jutland, Esbjerg, Denmark.

出版信息

Endocr Connect. 2022 May 10;11(5):e210490. doi: 10.1530/EC-21-0490.

DOI:10.1530/EC-21-0490
PMID:35358059
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9175611/
Abstract

OBJECTIVE

Klinefelter syndrome (KS) is associated with increased risk of thrombosis. Hypogonadism and accumulating body fat in KS have a potential impact on fibrinolysis. In this study, we assessed the fibrinolytic system and the association with testosterone levels in KS.

DESIGN

This study is a cross-sectional comparison of men with KS and age-matched male controls.

METHODS

Fibrin clot lysis was evaluated by turbidity measurements and by measuring levels of individual fibrinolytic proteins in plasma samples. Fibrin clot structure was evaluated by scanning electron microscopy. Total testosterone was measured by liquid chromatography-tandem mass spectrometry. Body fat was evaluated by dual-energy X-ray absorptiometry.

RESULTS

In this study, 45 men with KS and 45 age- and education-matched controls were included. Men with KS had a 24% reduction in fibrin clot lysis compared with controls (46.2 ± 17.1 vs 60.6 ± 18.8 %/h, P = 0.0003) and higher levels of fibrinogen, factor XIII (P ≤ 0.01), and plasminogen activator inhibitor type 1 (P = 0.04). Men with KS had lower total testosterone (P = 0.008) and higher body fat (P = 0.001). In KS, reduced fibrin clot lysability was associated with higher fibrinogen and body fat related to decreasing total testosterone and hypogonadism among men with KS. Fibrin clot structure was not different compared to KS and controls.

CONCLUSIONS

Fibrin clot lysis in KS was markedly reduced, potentially contributing to a prothrombotic state and increasing thrombotic risk. Hypogonadism in KS was associated with increased fibrinogen and total body fat, predicting reduced fibrin clot lysis.

摘要

目的

克兰费尔特综合征(KS)与血栓形成风险增加有关。KS患者的性腺功能减退和体脂堆积对纤维蛋白溶解有潜在影响。在本研究中,我们评估了KS患者的纤维蛋白溶解系统及其与睾酮水平的关联。

设计

本研究是对KS男性患者和年龄匹配的男性对照进行的横断面比较。

方法

通过比浊法测量以及检测血浆样本中单个纤维蛋白溶解蛋白的水平来评估纤维蛋白凝块溶解情况。通过扫描电子显微镜评估纤维蛋白凝块结构。采用液相色谱 - 串联质谱法测量总睾酮水平。通过双能X线吸收法评估体脂情况。

结果

本研究纳入了45例KS男性患者和45例年龄及教育程度匹配的对照。与对照组相比,KS男性患者的纤维蛋白凝块溶解减少了24%(46.2±17.1 vs 60.6±18.8%/小时,P = 0.0003),且纤维蛋白原、因子 XIII(P≤0.01)和纤溶酶原激活物抑制剂1型水平更高(P = 0.04)。KS男性患者的总睾酮水平较低(P = 0.008),体脂较高(P = 0.001)。在KS患者中,纤维蛋白凝块溶解能力降低与较高的纤维蛋白原和体脂有关,这与KS男性患者总睾酮降低和性腺功能减退相关。与对照组相比,KS患者的纤维蛋白凝块结构无差异。

结论

KS患者的纤维蛋白凝块溶解明显减少,这可能导致血栓前状态并增加血栓形成风险。KS患者的性腺功能减退与纤维蛋白原增加和全身脂肪增加有关,预示着纤维蛋白凝块溶解减少。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e236/9175611/03df048428c6/EC-21-0490fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e236/9175611/dbfdee7c67a2/EC-21-0490fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e236/9175611/43b3cb037aa9/EC-21-0490fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e236/9175611/03df048428c6/EC-21-0490fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e236/9175611/dbfdee7c67a2/EC-21-0490fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e236/9175611/43b3cb037aa9/EC-21-0490fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e236/9175611/03df048428c6/EC-21-0490fig3.jpg

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Endocr Connect. 2020 Jan;9(1):34-43. doi: 10.1530/EC-19-0433.
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Testosterone treatment and association with thrombin generation and coagulation inhibition in Klinefelter syndrome: A cross-sectional study.
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Endocr Connect. 2019 Sep;8(9):1250-1261. doi: 10.1530/EC-19-0323.
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The Effect of PAI-1 Gene Variants and PAI-1 Plasma Levels on Development of Thrombophilia in Patients With Klinefelter Syndrome.PAI-1 基因变异和 PAI-1 血浆水平对 Klinefelter 综合征患者血栓形成倾向发展的影响。
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