Efficacy and Safety of Oral Sildenafil in Cystic Fibrosis Children With Mild to Moderate Lung Disease

Efficacy and Safety of Oral Sildenafil in Cystic Fibrosis Children With Mild to Moderate Lung Disease Publisher Pubmed

Reisi M¹ ; Modaresi MR² ; Aghaii Z¹ ; Mirlohi SH² ; Rafiemanesh H³ ; Azizi G⁴ ; Sayedi SJ⁵

Show Affiliations

1. Department ofÂ Pediatric Pulmonology, Emam Hossein Children's Hospital, Isfahan University of Medical Sciences, Isfahan, Iran
2. Pediatric Pulmonary Disease and Sleep Medicine Research Center, Pediatric Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
3. Studentsâ€™ Research Committee, Department of Epidemiology, School of Public Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
4. Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
5. Neonatal Research Center, Akbar Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

Source: Pediatric Pulmonology Published:2020

Abstract

Background: Airway inflammation due to chronic infection is the leading cause of respiratory failure and death in most of patients with cystic fibrosis (CF). There is some evidence about anti-inflammatory activity of phosphodiesterase inhibitors in adult patients with CF. This study was designed to evaluate the efficacy, safety, and tolerability of sildenafil (a phosphodiesterase inhibitor drug) in children with CF. Method: This uncontrolled before-after study was conducted on 20 children with CF (mean age 14 ± 2.8 years, 50% male) with mild to moderate lung disease who were referred to CF clinic of Imam Hossein hospital in Isfahan, Iran. The patients received oral sildenafil (1 mg/kg p.o tid for 3 months). Changes in spirometric values, maximal exercise capacity, and patient-reported health by using the cystic fibrosis questionnaire-revised (CFQ-R) were evaluated before and after treatment. Result: CFQ-R (69.54 ± 4.6 vs 76.90 ± 5.4; P <.001) and exercise duration (401 ± 45.6 vs 497 ± 60.1 second; P <.01) increased following sildenafil therapy. In contrast, the forced expiratory value (FEV1; 84.60 ± 13.67 vs 78.40 ± 12.95; P <.001) and FEF25-75 (77.80 ± 27.33 vs 69.20 ± 21.91; P =.004) showed significant decreases. However, the mean of FEV1/forced vital capacity did not change significantly during the study (P =.682). Conclusions: Although sildenafil can improve the quality of life and exercise capacities in CF children, it significantly decreases lung function. So, administration of this drug for CF children should be reconsidered. © 2019 Wiley Periodicals, Inc.

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Style	Citing Format
MLA	Reisi M, et al.. "Efficacy and Safety of Oral Sildenafil in Cystic Fibrosis Children With Mild to Moderate Lung Disease." Pediatric Pulmonology, vol. 55, no. 1, 2020, pp. 156-160.
APA	Reisi M, Modaresi MR, Aghaii Z, Mirlohi SH, Rafiemanesh H, Azizi G, Sayedi SJ (2020). Efficacy and Safety of Oral Sildenafil in Cystic Fibrosis Children With Mild to Moderate Lung Disease. Pediatric Pulmonology, 55(1), 156-160.
Chicago	Reisi M, Modaresi MR, Aghaii Z, Mirlohi SH, Rafiemanesh H, Azizi G, Sayedi SJ. "Efficacy and Safety of Oral Sildenafil in Cystic Fibrosis Children With Mild to Moderate Lung Disease." Pediatric Pulmonology 55, no. 1 (2020): 156-160.
Harvard	Reisi M et al. (2020) 'Efficacy and Safety of Oral Sildenafil in Cystic Fibrosis Children With Mild to Moderate Lung Disease', Pediatric Pulmonology, 55(1), pp. 156-160.
Vancouver	Reisi M, Modaresi MR, Aghaii Z, Mirlohi SH, Rafiemanesh H, Azizi G, et al.. Efficacy and Safety of Oral Sildenafil in Cystic Fibrosis Children With Mild to Moderate Lung Disease. Pediatric Pulmonology. 2020;55(1):156-160.
BibTex	@article{ author = {Reisi M and Modaresi MR and Aghaii Z and Mirlohi SH and Rafiemanesh H and Azizi G and Sayedi SJ}, title = {Efficacy and Safety of Oral Sildenafil in Cystic Fibrosis Children With Mild to Moderate Lung Disease}, journal = {Pediatric Pulmonology}, volume = {55}, number = {1}, pages = {156-160}, year = {2020} }
RIS	TY - JOUR AU - Reisi M AU - Modaresi MR AU - Aghaii Z AU - Mirlohi SH AU - Rafiemanesh H AU - Azizi G AU - Sayedi SJ TI - Efficacy and Safety of Oral Sildenafil in Cystic Fibrosis Children With Mild to Moderate Lung Disease JO - Pediatric Pulmonology VL - 55 IS - 1 SP - 156 EP - 160 PY - 2020 ER -

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