title: Increased eNOS levels in hereditary angioedema.
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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Istanbul University, Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Allergy, Istanbul, Turkey. Electronic address: mustafadt@yahoo.com.
authors.forename: Mustafa
authors.initials: M
authors.lastname: Demirtürk
authors.affiliation: Istanbul University, Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Allergy, Istanbul, Turkey.
authors.forename: Aslı
authors.initials: A
authors.lastname: Gelincik
authors.affiliation: Istanbul University, Institute of Experimental Medicine, Department of Immunology, Istanbul, Turkey.
authors.forename: Suzan
authors.initials: S
authors.lastname: Cınar
authors.affiliation: Acibadem University, Department of Biochemistry, Turkey.
authors.forename: Meltem
authors.initials: M
authors.lastname: Kilercik
authors.affiliation: Istanbul University, Istanbul Faculty of Science, Department of Molecular Biology and Genetics, Turkey.
authors.forename: Evren
authors.initials: E
authors.lastname: Onay-Ucar
authors.affiliation: Istanbul University, Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Allergy, Istanbul, Turkey.
authors.forename: Bahattin
authors.initials: B
authors.lastname: Çolakoğlu
authors.affiliation: Istanbul University, Istanbul Faculty of Science, Department of Molecular Biology and Genetics, Turkey.
authors.forename: Nazli
authors.initials: N
authors.lastname: Arda
authors.affiliation: Istanbul University, Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Allergy, Istanbul, Turkey.
authors.forename: Suna
authors.initials: S
authors.lastname: Büyüköztürk
authors.affiliation: Istanbul University, Institute of Experimental Medicine, Department of Immunology, Istanbul, Turkey.
authors.forename: Günnur
authors.initials: G
authors.lastname: Deniz
mesh_terms: D000328:Adult; D000368:Aged; D054179:Angioedemas, Hereditary / Q000097:blood*; D016207:Cytokines / Q000097:blood; D042783:Endothelial Cells / Q000166:cytology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009569:Nitric Oxide / Q000097:blood; D052250:Nitric Oxide Synthase Type III / Q000097:blood*; D042461:Vascular Endothelial Growth Factor A / Q000097:blood; D055815:Young Adult
chemical_list: D016207:Cytokines; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D009569:Nitric Oxide; C496314:NOS3 protein, human; D052250:Nitric Oxide Synthase Type III
keywords: C1 inhibitor (C1-INH); Endothelial nitric oxide synthase (eNOS); Hereditary angio-edema (HAE); Inflammatory cytokines