Role of kallistatin in pediatric patients with pulmonary arterial hypertension

Background and Objectives. Kallistatin, a serine proteinase inhibitor, exerts its effect by vascular repair, angiogenesis inhibition, strong vasodilation, inhibition of vascular endothelial growth factor (VEGF), antiinflammation, and anti-apoptosis. We hypothesized as to whether it has a prot...

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Main Authors: Özge Pamukçu, Derya Ay, Kazım Üzüm, Didem Barlakketi, Mehmet Köse, Ali Baykan, Süleyman Sunkak, Nazmi Narin
Format: Article
Language:English
Published: Hacettepe University Institute of Child Health 2020-06-01
Series:The Turkish Journal of Pediatrics
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Online Access:https://turkjpediatr.org/article/view/460
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author Özge Pamukçu
Derya Ay
Kazım Üzüm
Didem Barlakketi
Mehmet Köse
Ali Baykan
Süleyman Sunkak
Nazmi Narin
author_facet Özge Pamukçu
Derya Ay
Kazım Üzüm
Didem Barlakketi
Mehmet Köse
Ali Baykan
Süleyman Sunkak
Nazmi Narin
author_sort Özge Pamukçu
collection DOAJ
description Background and Objectives. Kallistatin, a serine proteinase inhibitor, exerts its effect by vascular repair, angiogenesis inhibition, strong vasodilation, inhibition of vascular endothelial growth factor (VEGF), antiinflammation, and anti-apoptosis. We hypothesized as to whether it has a protective role in pulmonary arterial hypertension (PAH). Methods. The study included 5 subgroups (78 patients; 44 male): Eisenmenger syndrome (n=16), PAH with left to right shunt (n=20), idiopathic PAH (n=7), patients with left to right shunt without PAH (n=19), and patients with innocent heart murmur (n=16). Physical examination, chest radiography, electrocardiography, and transthoracic echocardiography (TTE) were performed for each patient. PAH diagnosis was confirmed by catheterization. Serum kallistatin, tumor necrosis factor alpha (TNF-α), Interleukin-10 (IL-10) and N-terminal pro b-type natriuretic peptide (NT-proBNP) levels were studied for each patient. Results. The lowest median kallistatin value was found in Eisenmenger syndrome: 1.19 (0.87-3.30) μg/ml. The highest value belonged to control group with innocent murmur: 2.89 (1.19-5.66) μg/ml. Serum levels of kallistatin were significantly lower in patients with PAH (p < 0.05). TNF-α values were increased and IL-10 values were decreased in pulmonary hypertension. However; no correlation was found between kallistatin levels and cytokines. Conclusions. Kallistatin may have a protective effect in pulmonary arterial hypertension by repairing vascular damage, inhibition of angiogenesis, strong vasodilator effect, inhibiting VEGF, and anti-inflammatory mechanism of action. To our knowledge, our study is the first one that shows the role of kallistatin in pulmonary hypertension. Kallistatin may represent a promising novel therapeutic approach for pulmonary hypertension in the near future.
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issn 0041-4301
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language English
publishDate 2020-06-01
publisher Hacettepe University Institute of Child Health
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series The Turkish Journal of Pediatrics
spelling doaj-art-3db28c2daeca4b1dbd25ce0e1ef6d4702025-08-20T03:16:22ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212020-06-0162310.24953/turkjped.2020.03.007Role of kallistatin in pediatric patients with pulmonary arterial hypertensionÖzge Pamukçu0Derya Ay1Kazım Üzüm2Didem Barlakketi3Mehmet Köse4Ali Baykan5Süleyman Sunkak6Nazmi Narin7Divisions of Pediatric Cardiology, Erciyes University Faculty of Medicine, Kayseri, Turkey.Divisions of Pediatric Cardiology, Erciyes University Faculty of Medicine, Kayseri, Turkey.Divisions of Pediatric Cardiology, Erciyes University Faculty of Medicine, Kayseri, Turkey.Department of Pediatrics, Department of Biochemistry, Erciyes University Faculty of Medicine, Kayseri, Turkey.Divisions of Pediatric Pulmonology, Erciyes University Faculty of Medicine, Kayseri, Turkey.Divisions of Pediatric Cardiology, Erciyes University Faculty of Medicine, Kayseri, Turkey.Divisions of Pediatric Cardiology, Erciyes University Faculty of Medicine, Kayseri, Turkey.Divisions of Pediatric Cardiology, Erciyes University Faculty of Medicine, Kayseri, Turkey. Background and Objectives. Kallistatin, a serine proteinase inhibitor, exerts its effect by vascular repair, angiogenesis inhibition, strong vasodilation, inhibition of vascular endothelial growth factor (VEGF), antiinflammation, and anti-apoptosis. We hypothesized as to whether it has a protective role in pulmonary arterial hypertension (PAH). Methods. The study included 5 subgroups (78 patients; 44 male): Eisenmenger syndrome (n=16), PAH with left to right shunt (n=20), idiopathic PAH (n=7), patients with left to right shunt without PAH (n=19), and patients with innocent heart murmur (n=16). Physical examination, chest radiography, electrocardiography, and transthoracic echocardiography (TTE) were performed for each patient. PAH diagnosis was confirmed by catheterization. Serum kallistatin, tumor necrosis factor alpha (TNF-α), Interleukin-10 (IL-10) and N-terminal pro b-type natriuretic peptide (NT-proBNP) levels were studied for each patient. Results. The lowest median kallistatin value was found in Eisenmenger syndrome: 1.19 (0.87-3.30) μg/ml. The highest value belonged to control group with innocent murmur: 2.89 (1.19-5.66) μg/ml. Serum levels of kallistatin were significantly lower in patients with PAH (p < 0.05). TNF-α values were increased and IL-10 values were decreased in pulmonary hypertension. However; no correlation was found between kallistatin levels and cytokines. Conclusions. Kallistatin may have a protective effect in pulmonary arterial hypertension by repairing vascular damage, inhibition of angiogenesis, strong vasodilator effect, inhibiting VEGF, and anti-inflammatory mechanism of action. To our knowledge, our study is the first one that shows the role of kallistatin in pulmonary hypertension. Kallistatin may represent a promising novel therapeutic approach for pulmonary hypertension in the near future. https://turkjpediatr.org/article/view/460IL-10TNFαkallistatinpulmonary hypertension
spellingShingle Özge Pamukçu
Derya Ay
Kazım Üzüm
Didem Barlakketi
Mehmet Köse
Ali Baykan
Süleyman Sunkak
Nazmi Narin
Role of kallistatin in pediatric patients with pulmonary arterial hypertension
The Turkish Journal of Pediatrics
IL-10
TNFα
kallistatin
pulmonary hypertension
title Role of kallistatin in pediatric patients with pulmonary arterial hypertension
title_full Role of kallistatin in pediatric patients with pulmonary arterial hypertension
title_fullStr Role of kallistatin in pediatric patients with pulmonary arterial hypertension
title_full_unstemmed Role of kallistatin in pediatric patients with pulmonary arterial hypertension
title_short Role of kallistatin in pediatric patients with pulmonary arterial hypertension
title_sort role of kallistatin in pediatric patients with pulmonary arterial hypertension
topic IL-10
TNFα
kallistatin
pulmonary hypertension
url https://turkjpediatr.org/article/view/460
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