Mustafa Demirtürk1, Aslı Gelincik2, Suzan Cınar3, Meltem Kilercik4, Evren Onay-Ucar5, Bahattin Çolakoğlu2, Nazli Arda5, Suna Büyüköztürk2, Günnur Deniz3. 1. Istanbul University, Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Allergy, Istanbul, Turkey. Electronic address: mustafadt@yahoo.com. 2. Istanbul University, Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Allergy, Istanbul, Turkey. 3. Istanbul University, Institute of Experimental Medicine, Department of Immunology, Istanbul, Turkey. 4. Acibadem University, Department of Biochemistry, Turkey. 5. Istanbul University, Istanbul Faculty of Science, Department of Molecular Biology and Genetics, Turkey.
Abstract
BACKGROUND: Hereditary angio-edema (HAE), characterized by recurrent episodes of angioedema involving the skin and the mucosa of the upper respiratory or the gastrointestinal tracts, results from heterozygosity for deficiency of the serine proteinase inhibitor (serpin), C1 inhibitor (C1-INH). OBJECTIVE: In this study, serum inflammatory cytokine levels and circulating endothelial cells collected from HAE patients during both acute attacks and asymptomatic periods were evaluated. METHOD: Twenty-four patients with Type I and 1 patient with Type II HAE in an asymptomatic period (Group I), 8 patients with Type I HAE during a mild to moderate acute attack (Group II) and 20 healthy subjects (13 females, mean age: 32.1±8.2years) were included. Serum IL-6, IL-8, IL-1β, TNF-α, vascular endothelial growth factor (VEGF) and endothelial nitric oxide synthase (eNOS) levels were detected by ELISA. Circulating endothelial cells (CECs) and circulating endothelial progenitors (CEPs) were evaluated using Fluorescence Activated Cell Sorting (FACS). RESULTS: Serum eNOS levels of HAE patients were significantly higher than healthy subjects (p<0.006) while mean TNF-α levels in Group I were slightly lower (p<0.03) than Group II. There were no differences in terms of other inflammatory cytokines between the control subjects and HAE patients who were either in an asymptomatic period or experiencing an acute attack. CECs and CEPs were also similar. CONCLUSION: These results suggest that an inflammatory response is not necessary to trigger HAE attacks. On the other hand, increased eNOS levels might reflect a sustained hyperpermeability state in HAE patients.
BACKGROUND: Hereditary angio-edema (HAE), characterized by recurrent episodes of angioedema involving the skin and the mucosa of the upper respiratory or the gastrointestinal tracts, results from heterozygosity for deficiency of the serine proteinase inhibitor (serpin), C1 inhibitor (C1-INH). OBJECTIVE: In this study, serum inflammatory cytokine levels and circulating endothelial cells collected from HAEpatients during both acute attacks and asymptomatic periods were evaluated. METHOD: Twenty-four patients with Type I and 1 patient with Type II HAE in an asymptomatic period (Group I), 8 patients with Type IHAE during a mild to moderate acute attack (Group II) and 20 healthy subjects (13 females, mean age: 32.1±8.2years) were included. Serum IL-6, IL-8, IL-1β, TNF-α, vascular endothelial growth factor (VEGF) and endothelial nitric oxide synthase (eNOS) levels were detected by ELISA. Circulating endothelial cells (CECs) and circulating endothelial progenitors (CEPs) were evaluated using Fluorescence Activated Cell Sorting (FACS). RESULTS: Serum eNOS levels of HAEpatients were significantly higher than healthy subjects (p<0.006) while mean TNF-α levels in Group I were slightly lower (p<0.03) than Group II. There were no differences in terms of other inflammatory cytokines between the control subjects and HAEpatients who were either in an asymptomatic period or experiencing an acute attack. CECs and CEPs were also similar. CONCLUSION: These results suggest that an inflammatory response is not necessary to trigger HAE attacks. On the other hand, increased eNOS levels might reflect a sustained hyperpermeability state in HAEpatients.
Authors: Davide Firinu; Pier P Bassareo; Angela M Zedda; Maria P Barca; Antonio Crisafulli; Giuseppe Mercuro; Stefano Del Giacco Journal: Front Physiol Date: 2018-05-16 Impact factor: 4.566