PhD Scientific Days 2017

Oral Presentations: Clinical Sciences

Novel high-temporal resolution follow-up of an edematous attack of a C1-INH-HAE patient: complement system in focus

Előadó neve

Veszeli, Nóra

Előadó munkahelye

3rd Department of Internal Medicine, Semmelweis University, Budapest

Előadó telefonszáma

+36208259130

Előadó e-mail címe

veszeli.nora88@gmail.com

Az előadás címe

Novel high-temporal resolution follow-up of an edematous attack of a C1-INH-HAE patient: complement system in focus

Szerző(k) neve és munkahelye

Nóra Veszeli, Kinga Viktória Kőhalmi, Erika Kajdácsi, Márta Kókai, Dominik Gulyás, László Cervenak, Henriette Farkas, Lilian Varga
3rd Department of Internal Medicine, Semmelweis University, Budapest

Szekció

Oral Presentations: Clinical Sciences

Data of the presenter

Doctoral School: Basic Medicine
Program: 05. Clinical and Experimental Cardiology / Atherosclerosis
Supervisor: Henriette Farkas and Lilian Varga
E-mail address: veszeli.nora88@gmail.com

Text of the abstract

Introduction: Hereditary angioedema due to C1-inhibitor (C1-INH) deficiency (C1-INH-HAE) is a potentially life-threatening rare disease, characterized by recurring and spontaneously resolving edematous attacks. C1-INH deficiency leads to uncontrolled activation of the complement and the kallikrein-kinin system resulting bradykinin release.
Aims: For the first time, we aimed to study the kinetics of C1-INH and other complement parameters as well as their role in a spontaneously resolved edematous attack of a C1-INH-HAE patient.
Method: In a patient with C1-INH-HAE, we monitored the severity of the symptoms during the observation period, including baseline, prodromal, during-attack and complete resolution stages, and altogether 12 blood samples were obtained. Five blood samples were collected from a healthy control during 24–hour period. We measured C1-INH concentration and function (C1-INHc+f), C1(q,r,s), C3, C4, C3a, C4a, C5a and SC5b-9 level.
Results: Highest C1-INHc+f, C4, C1(q,r,s) were measured at baseline and continuous decrease were observed during whole 96-hour observation period. C1-INHc was 0.033 g/l when edematous attack started. C4 level was depleted when edematous symptoms reached the maximum severity. C4a level was four times higher 18 hours before the onset of the edematous attack compared to baseline. Level of other complement activation parameters were lower compared to control. In the healthy control, all measured parameters were constantly stable during whole period.
Conclusion: When C1-INH decreased below a critical concentration, an attack occurred. Interestingly, we could not find any increase of C1-INH after spontaneous resolution of the attack. This indicates that other factors than C1-INH itself may play a crucial role in the resolution of the edematous attack. C4a could be a good predictive biomarker of edematous attack.
This study was supported by the Grant OTKA 112110 from the Hungarian Research Foundation and by the ÚNKP-16-3 New National Excellence Program of the Ministry of Human Capacities.

Kind

Szabad

Előadás fájl jóváhagyás

nem rendelkezett róla

Előadó

1161

Authors (legacy)

Nóra Veszeli, Kinga Viktória Kőhalmi, Erika Kajdácsi, Márta Kókai, Dominik Gulyás, László Cervenak, Henriette Farkas, Lilian Varga
3rd Department of Internal Medicine, Semmelweis University, Budapest