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遗传性血管性水肿无症状期的内皮功能受损

Impaired Endothelial Function in Hereditary Angioedema During the Symptom-Free Period.

作者信息

Firinu Davide, Bassareo Pier P, Zedda Angela M, Barca Maria P, Crisafulli Antonio, Mercuro Giuseppe, Del Giacco Stefano

机构信息

Unit of Internal Medicine, Department of Medical Sciences and Public Health, Allergy and Clinical Immunology, University of Cagliari, Cagliari, Italy.

Unit of Cardiology and Angiology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.

出版信息

Front Physiol. 2018 May 16;9:523. doi: 10.3389/fphys.2018.00523. eCollection 2018.

DOI:10.3389/fphys.2018.00523
PMID:29867566
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5964294/
Abstract

The presence of coronary endothelial dysfunction was previously shown in Hereditary Angioedema (HAE) patients. The aim of our study was to evaluate the effect of HAE on systemic endothelial function and whether there was a relationship among endothelial function, asymmetric dimethylarginine (ADMA) -which is a strong inhibitor of nitric oxide synthesis-, and disease severity scores. Twenty-four HAE patients (18 females, aged 47.9 ± 2 years) without factors known to interfere with endothelial function were studied and compared with 24 healthy peers age- and gender-matched. Endothelial function was assessed by means of non-invasive finger plethysmography (reactive hyperaemia index: RHI) and ADMA levels by high-performance liquid chromatography. HAE severity scores have been calculated according to published literature. In HAE patients RHI was lower (2.03 ± 0.46 vs. 2.82 ± 0.34, < 0.0001) and ADMA higher (0.636 ± 7 vs. 585 ± 5 micromol/L, < 0.01) than in controls. A statistically significant inverse correlation was revealed between RHI and patients' ADMA levels ( = -0.516, = 0.009) as well as between RHI and patients' chronological age ( = -0.49, = 0.015). A statistically significant correlation between RHI and ADMA was confirmed even when excluding the possible influence of cholesterol ( = -0.408, = 0.048). No other significant correlations were found with the examined laboratory and clinical parameters (chronological age, age at disease onset, disease duration, severity scores, and gender). The dysfunction previously shown in HAE patients at the coronary arteries seems to involve the peripheral vessels as well, without a correlation with disease severity.

摘要

先前的研究表明,遗传性血管性水肿(HAE)患者存在冠状动脉内皮功能障碍。我们研究的目的是评估HAE对全身内皮功能的影响,以及内皮功能、不对称二甲基精氨酸(ADMA,一种一氧化氮合成的强抑制剂)与疾病严重程度评分之间是否存在关联。研究了24例无已知干扰内皮功能因素的HAE患者(18名女性,年龄47.9±2岁),并与24名年龄和性别匹配的健康对照者进行比较。通过无创手指体积描记法(反应性充血指数:RHI)评估内皮功能,通过高效液相色谱法测定ADMA水平。根据已发表的文献计算HAE严重程度评分。与对照组相比,HAE患者的RHI较低(2.03±0.46对2.82±0.34,P<0.0001),ADMA较高(0.636±0.07对0.585±0.05μmol/L,P<0.01)。RHI与患者的ADMA水平之间存在统计学显著的负相关(r=-0.516,P=0.009),以及RHI与患者的实际年龄之间存在负相关(r=-0.49,P=0.015)。即使排除胆固醇的可能影响,RHI与ADMA之间仍存在统计学显著相关性(r=-0.408,P=0.048)。未发现与所检查的实验室和临床参数(实际年龄、发病年龄、病程、严重程度评分和性别)有其他显著相关性。先前在HAE患者冠状动脉中显示的功能障碍似乎也累及外周血管,且与疾病严重程度无关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdb8/5964294/06c2c81247b0/fphys-09-00523-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdb8/5964294/06c2c81247b0/fphys-09-00523-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdb8/5964294/06c2c81247b0/fphys-09-00523-g0001.jpg

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Genetic Regulation of Endothelial Vasomotor Function.内皮血管舒缩功能的基因调控
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