Endothelial Cell Function in Patients with Hereditary Angioedema: Elevated Soluble E-selectin Level During Inter-attack Periods

Czúcz, Judit; Schaffer, G.; Csuka, Dorottya; Walentin, Szilvia; Kunde, Jan; Prohászka, Zoltán; Cervenak, László

doi:10.1007/s10875-011-9606-7

Cited by 19 publications

(18 citation statements)

References 38 publications

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“…That is, blood pressure, and the incidence of any vascular disease are not different between C1-INH-HAE and non-C1-INH-HAE populations, as indicated by data from this study, and from our previous studies [23,24]. Moreover, the similarities between the ET-1 levels of C1-INH-HAE patients and of controls are in agreement with our previous findings, which did not show any difference in ET-1 levels in smaller study cohorts [12]. The positive correlation between ADM level and age, or BMI [13,[25][26][27] and the gender difference in AVP level [14,28] are all well-known from other studies.…”

Section: Discussionsupporting

confidence: 92%

“…One hundred consecutive C1-INH-HAE patients were enrolled into the first part of this study (an enlarged patients population compared with that of our previous study [12]), who presented for their annual check-up at our Centre. Forty-two patients received danazol regularly (mean daily dose of danazol: 99 mg/day [range: 30-260 mg/day], mean duration of treatment: 11.8 [range 1-26 year]).…”

Section: C1-inh-hae Patientsmentioning

confidence: 99%

“…The control population also consisted of the enlarged group (n = 111) of our previous study [12]. Controls subjects presented for routine medical check-up.…”

Section: Control Subjectsmentioning

confidence: 99%

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Novel Vasoregulatory Aspects of Hereditary Angioedema: the Role of Arginine Vasopressin, Adrenomedullin and Endothelin-1

et al. 2016

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The elevation of bradykinin (BK) level during attacks of hereditary angioedema due to C1-Inhibitor deficiency (C1-INH-HAE) is well known. We previously demonstrated that endothelin-1 (ET-1) level also increases during C1-INH-HAE attacks. Although BK and ET-1 are both potent vasoactive peptides, the vasoregulatory aspect of the pathomechanism of C1-INH-HAE has not yet been investigated. Hence we studied the levels of vasoactive peptides in controls and in C1-INH-HAE patients, as well as evaluated their changes during C1-INH-HAE attacks. The levels of arginine vasopressin (AVP), adrenomedullin (ADM) and ET-1 were measured in the plasma of 100 C1-INH-HAE patients in inter-attack periods and of 111 control subjects, using BRAHMS Kryptor technologies. In 18 of the 100 C1-INH-HAE patients, the levels of vasoactive peptides were compared in blood samples obtained during attacks, or in inter-attack periods. AVP, ADM and ET-1 levels were similar in inter-attack samples from C1-INH-HAE patients and in the samples of controls, although cardiovascular risk has an effect on the levels of vasoactive peptides in both groups. The levels of all three vasoactive peptides increased during C1-INH-HAE attacks. Moreover, the levels of ET-1 and ADM as well as their changes during attacks were significantly correlated. This study demonstrated that vascular regulation by vasoactive peptides is affected during C1-INH-HAE attacks. Our results suggest that the cooperation of several vasoactive peptides may be necessary to counterbalance the actions of excess BK, and to terminate the attacks. This may reveal a novel pathophysiological aspect of C1-INH-HAE.

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Section: Discussionsupporting

confidence: 92%

Section: C1-inh-hae Patientsmentioning

confidence: 99%

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Novel Vasoregulatory Aspects of Hereditary Angioedema: the Role of Arginine Vasopressin, Adrenomedullin and Endothelin-1

et al. 2016

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“…Recently, specific attention was paid to the possibility that an upregulation of different vasoactive substances may influence the risk of angioedema in C1-INH-HAE patients. E selectin, VGEF A and C and angiopoietin 2 are increased in these patients during intercritical periods and seem to predispose them to attacks [ 40 – 42 ]. Studying plasma markers of endothelial cells during angioedema attacks, Kajdacsi and colleagues found an activated phenotype suggesting that these cells actively participate in angioedema formation [ 43 ].…”

Section: Discussionmentioning

confidence: 99%

Hereditary angioedema: Assessing the hypothesis for underlying autonomic dysfunction

Casella

Perego

et al. 2017

PLoS ONE

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BackgroundAttacks of Hereditary Angioedema due to C1-inhibitor deficiency (C1-INH-HAE)are often triggered by stressful events/hormonal changes.ObjectiveOur study evaluates the relationship between autonomic nervous system (ANS) and contact/complement system activation.MethodsTwenty-three HAE patients (6 males, mean age 47.5±11.4 years) during remission and 24 healthy controls (8 males, mean age 45.3±10.6 years) were studied. ECG, beat-by-beat blood pressure, respiratory activity were continuously recorded during rest (10’) and 75-degrees-head-up tilt (10’). C1-INH, C4, cleaved high molecular weight kininogen (cHK) were assessed; in 16 patients and 11 controls plasma catecholamines were also evaluated. Spectral analysis of heart rate variability allowed extraction of low-(LF) and high-(HF) frequency components, markers of sympathetic and vagal modulation respectively.ResultsHAE patients showed higher mean systolic arterial pressure (SAP) than controls during both rest and tilt. Tilt induced a significant increase in SAP and its variability only in controls. Although sympathetic modulation (LFnu) increased significantly with tilt in both groups, LF/HF ratio, index of sympathovagal balance, increased significantly only in controls.At rest HAE patients showed higher noradrenaline values (301.4±132.9 pg/ml vs 210.5±89.6pg/ml, p = 0.05). Moreover, in patients tilt was associated with a significant increase in cHK, marker of contact system activation (49.5 ± 7.5% after T vs 47.1 ± 7.8% at R, p = 0.01).ConclusionsOur data are consistent with altered ANS modulation in HAE patients, i.e. increased sympathetic activation at rest and blunted response to orthostatic challenge. Tilt test-induced increased HK cleavage suggests a link between stress and bradykinin production.

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“…Furthermore, the role of the endothelial cell in HAE attacks has received significant attention in recent years [5]. Czúcz et al [6] studied the endothelial cell functions of HAE patients during attack-free periods and compared those with that of healthy control subjects by monitoring 4 specific and sensitive plasma biomarkers of endothelial cells, i.e., soluble E-selectin, endothelin-1, von Willebrand factor (vWF) antigen, and vWF collagen-binding activity. They found that the majority of endothelial functions were normal during attack-free periods, although soluble E-selectin levels were elevated.…”

Section: Introductionmentioning

confidence: 99%

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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Endothelial Cell Function in Patients with Hereditary Angioedema: Elevated Soluble E-selectin Level During Inter-attack Periods

Cited by 19 publications

References 38 publications

Novel Vasoregulatory Aspects of Hereditary Angioedema: the Role of Arginine Vasopressin, Adrenomedullin and Endothelin-1

Novel Vasoregulatory Aspects of Hereditary Angioedema: the Role of Arginine Vasopressin, Adrenomedullin and Endothelin-1

Hereditary angioedema: Assessing the hypothesis for underlying autonomic dysfunction

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

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