Increased eNOS levels in hereditary angioedema

Demirtürk, Mustafa; Gelincik, Aslı; Çınar, Suzan; Kilercik, Meltem; Uçar, Evren Önay; Çolakoğlu, Bahattin; Arda, Nazlı; Büyüköztürk, Suna; Deniz, Günnur

doi:10.1016/j.intimp.2014.03.007

Cited by 19 publications

(19 citation statements)

References 17 publications

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“…Accordingly, they hypothesized that VEGFs and Angs induce a state of “vascular preconditioning” that may predispose some patients to angioedema attacks. In concordance with this finding, we previously demonstrated that eNOS, which is important in hyperpermeability processes, was higher in symptom-free HAE patients while NO metabolites were high only during attack periods [8]. In that study, although the total (nonfractionated) VEGF levels of HAE patients were higher than those of healthy subjects, this difference was not significant.…”

Section: Discussionsupporting

confidence: 76%

“…In light of these findings, our results showing high endocan levels in HAE patients during attack-free periods can be interpreted as sustained endothelial activation, which may predispose patients to angioedema attacks. Although endocan is usually proposed as a marker of endothelial inflammation, previous studies could not find evidence for endothelial inflammation as a cause of an increase in vascular permeability in HAE [8,9,10,11,12,13,15,16]. Accordingly, it can be hypothesized that other factors exist which affect endothelial integrity, even in attack-free HAE patients.…”

Section: Discussionmentioning

confidence: 94%

“…However, during HAE attacks soluble E-selectin, endothelin-1, and vWF were found to increase [7]. Recently, we found that serum endothelial nitric oxide (eNOS) levels were significantly higher in HAE patients, which may reflect a sustained hyperpermeability state even in attack-free periods [8]. …”

Section: Introductionmentioning

confidence: 99%

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Endocan: A Novel Marker of Endothelial Dysfunction in C1-Inhibitor-Deficient Hereditary Angioedema

Demirtürk

Akpınar²,

Köse³

et al. 2017

Int Arch Allergy Immunol

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Background: Hereditary angioedema (HAE) related to C1-inhibitor deficiency is a rare autosomal dominant disorder. Vascular cell adhesion molecules (VCAM) are known as endothelial activation markers. Endocan (also called ESM-1) is proposed as an endothelial dysfunction indicator. We aimed to investigate endothelial activation in attack-free periods in HAE patients by measuring their levels of endocan and VCAM-1. Methods: Twenty-six HAE patients (22 female, mean age 40 ± 13 years) and 38 healthy control patients (13 female, mean age 36.9 ± 12 years) were included in the study. Peripheral blood samples were collected from HAE patients during symptom-free periods and control subjects. Endocan and VCAM-1 levels were measured using the enzyme-linked immunosorbent assay method. Results: The median serum levels of endocan (647 ± 101 ng/mL) and VCAM-1 (500 ± 79 ng/mL) in the HAE patients were significantly higher than in the control patients (391 ± 41 and 325 ± 4; p < 0.001 for both). Conclusion: The increased endocan and VCAM-1 levels may reflect an endothelial activation even in attack-free periods in HAE patients.

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Section: Discussionsupporting

confidence: 76%

Section: Discussionmentioning

confidence: 94%

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Endocan: A Novel Marker of Endothelial Dysfunction in C1-Inhibitor-Deficient Hereditary Angioedema

Demirtürk

Akpınar²,

Köse³

et al. 2017

Int Arch Allergy Immunol

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“…The observation that soluble E-selectin and eNOS plasma levels are increased in C1-INH-HAE patients strongly suggests that endothelial cell activation may have a role in the pathogenesis of attacks [25,26]. Interestingly, endothelial cells stimulated by cytokines and estrogen release HSP-90 (heat shock protein-90) which, in turn, activates directly the prekallikrein-HMWK complex without the requirement of FXII.…”

Section: Pathophysiology Of Aementioning

confidence: 99%

Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes

Bova

Feo

Parente

et al. 2018

Int Arch Allergy Immunol

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Recurrent angioedema (AE) without wheals is increasingly recognized as a clinical entity and a frequent cause of admission to the emergency room. The Hereditary Angioedema Working Group (HAWK) classification allowed the scientific community to go beyond the semantic confusion that dominated this topic for decades. This classification distinguishes hereditary and acquired forms of AE, either related or unrelated to C1 inhibitor deficiency. Recently, additional mechanisms have been involved in the AE pathogenesis, including the uncontrolled activation of factor XII, generation of vasoactive mediators that induce dysregulation of endothelial functions, and bidirectional interactions between mast cell-derived mediators and the plasma contact system. Thus, recurrent AE can be determined by multiple and concurrent mechanisms that may generate distinct clinical phenotypes of the disease. Frequency, severity, and the location of attacks are quite different from patient to patient and, even in the same patient, they may change throughout the course of life. The severity of the clinical phenotype strongly influences the burden of the disease and patients’ quality of life. Despite major advances in our understanding of recurrent AE, many unsolved questions remain, leaving several unmet needs for patients and caregivers. This review is focused on a description of different AE phenotypes and the concurrent mechanisms leading to their pathogenesis. A better definition of cellular and molecular pathways responsible for the distinct AE phenotypes may help to improve diagnosis and may lead to a personalized approach to prophylaxis and treatment of the disease.

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“…On the other hand, it was previously reported that in HAE subjects, most of the endothelial functions are normal in the inter-attack periods, as shown by normal blood levels of some markers of endothelial cell permeability (endothelin-1, von Willebrand factor) (Czúcz et al, 2012 ). However, increased endothelial nitric oxide synthase levels in attack-free periods were detected in C1-INH-HAE patients as well (Demirtürk et al, 2014 ; Costa et al, 2016 ).…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2018

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Introduction: 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.Methods: 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.Results: In HAE patients RHI was lower (2.03 ± 0.46 vs. 2.82 ± 0.34, p < 0.0001) and ADMA higher (0.636 ± 7 vs. 585 ± 5 micromol/L, p < 0.01) than in controls. A statistically significant inverse correlation was revealed between RHI and patients' ADMA levels (r = −0.516, p = 0.009) as well as between RHI and patients' chronological age (r = −0.49, p = 0.015). A statistically significant correlation between RHI and ADMA was confirmed even when excluding the possible influence of cholesterol (r = −0.408, p = 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).Conclusion: 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.

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Increased eNOS levels in hereditary angioedema

Cited by 19 publications

References 17 publications

Endocan: A Novel Marker of Endothelial Dysfunction in C1-Inhibitor-Deficient Hereditary Angioedema

Endocan: A Novel Marker of Endothelial Dysfunction in C1-Inhibitor-Deficient Hereditary Angioedema

Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes

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

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