title: Comprehensive study into the activation of the plasma enzyme systems during attacks of hereditary angioedema due to C1-inhibitor deficiency.
abstract: BACKGROUND
  The activation of plasma enzyme systems contributes to hereditary angioedema attacks. We aimed to study the activation markers of the fibrinolytic, coagulation, and contact systems in a larger number of paired samples obtained from the same C1-INH-HAE patients in symptom-free periods and during attacks.
  
  
  METHODS
  Eleven parameters (Factors XI, XII, and C1-inhibitor activity; the concentrations of the D-dimer, prothrombin fragments 1 + 2, plasminogen, plasminogen activator inhibitor-1 [PAI-1], thrombin-anti-thrombin III [TAT] complex, fibrinogen) were measured along with prothrombin time and activated partial thromboplastin time (aPTT), using commercial kits. We compared these markers in samples obtained from the same 39 patients during attack-free periods and during 62 edematous episodes. Forty healthy subjects of matching sex and age served as controls.
  
  
  RESULTS
  Compared with the healthy controls, significantly higher FXI and FXII activity (p = 0.0007, p = 0.005), as well as D-dimer (p < 0.0001), prothrombin fragments 1 + 2 (p < 0.0001), and TAT (p = 0.0303) levels were ascertained in the patients during symptom-free periods. The evaluation of samples from symptom-free periods or obtained during attacks revealed the increase of FXII activity, as well as of the concentration of D-dimer, prothrombin fragments 1 + 2, and TAT during edematous episodes. PAI-1 level, prothrombin time, and aPTT decreased significantly during attacks, compared with symptom-free periods. D-dimer level was significantly higher during multiple- vs. single-site attacks.
  
  
  CONCLUSIONS
  Comparing a large number of paired samples from symptom-free periods or from edematous episodes allowed accurate appraisal of the changes occurring during attacks. Moreover, our study pointed out that individual episodes may be characterized by different marker patterns.
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Faculty of Medicine, Kútvölgyi út 4, H-1125, Budapest, Hungary. csuka.dorottya@med.semmelweis-univ.hu.
authors.forename: Dorottya
authors.initials: D
authors.lastname: Csuka
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Faculty of Medicine, Kútvölgyi út 4, H-1125, Budapest, Hungary. veszeli.nora88@gmail.com.
authors.forename: Nóra
authors.initials: N
authors.lastname: Veszeli
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Faculty of Medicine, Kútvölgyi út 4, H-1125, Budapest, Hungary. imrehe@kut.sote.hu.
authors.forename: Éva
authors.initials: É
authors.lastname: Imreh
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Faculty of Medicine, Kútvölgyi út 4, H-1125, Budapest, Hungary. zotter.zsuzsanna@gmail.com.
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Zotter
authors.affiliation: Department of Cardiology, Heart & Vascular Center, Semmelweis University, Budapest, Hungary. jskopal56@gmail.com.
authors.forename: Judit
authors.initials: J
authors.lastname: Skopál
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Faculty of Medicine, Kútvölgyi út 4, H-1125, Budapest, Hungary. prohaszka.zoltan@med.semmelweis-univ.hu.
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Prohászka
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Faculty of Medicine, Kútvölgyi út 4, H-1125, Budapest, Hungary. vargalilian@gmail.com.
authors.forename: Lilian
authors.initials: L
authors.lastname: Varga
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Faculty of Medicine, Kútvölgyi út 4, H-1125, Budapest, Hungary. farkas.henriette@med.semmelweis-univ.hu.
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
mesh_terms: D000328:Adult; D054179:Angioedemas, Hereditary / Q000097:blood* / Q000175:diagnosis / Q000201:enzymology*; D015415:Biomarkers / Q000097:blood; D050718:Complement C1 Inhibitor Protein / Q000378:metabolism*; D004789:Enzyme Activation / Q000502:physiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D055815:Young Adult
chemical_list: D015415:Biomarkers; D050718:Complement C1 Inhibitor Protein