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Article

Long Term Outcome of Cystic Fibrosis Patients with Multisystem Evaluation

1
Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
2
Faculty of Medicine,Tehran University of Medical Sciences, Tehran, Iran
3
Chronic Respiratory Disease Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
*
Author to whom correspondence should be addressed.
Adv. Respir. Med. 2016, 84(6), 310-315; https://doi.org/10.5603/ARM.2016.0040
Submission received: 17 May 2016 / Revised: 10 October 2016 / Accepted: 10 October 2016 / Published: 7 December 2016

Abstract

Introduction: Cystic fibrosis is a chronic disease with multiple organ involvement and chiefly results in chronic respiratory infections, pancreatic insufficiency and associated complications. The age at diagnosis, clinical presentation, rate of disease progression and prognosis is variable among patients. This study is designed to evaluate the behavior of disease to provide epidemiologic data for early recognition and proper management. Material and methods: The study was designed as an active surveillance of 192 patients diagnosed with cystic fibrosis in a tertiary lung disease centre between 2008 and 2015. The diagnosis of cystic fibrosis was established in all patients accordingly to conventional criteria, including two positive sweat chloride tests and clinical signs and symptoms. Demographic, clinical and laboratory data were obtained from these patients in each hospitalization and also every follow-up visit and carefully evaluated for complications of this chronic disease. Results: The majority of patients showed positive culture for Pseudomonas aeroginosa. Bronchiectasis was the most prevalent finding in chest CT scan. 44.3% of patients had been treated for allergic bronchopulmonary aspergillosis and all had sinus disease. Increased pulmonary artery pressure was observed in 40% of patients with cystic fibrosis. 33 patients died which consisted 17.1% of all the patients.The mean age of mortaliy was 18.15 year. Conclusions: The clinical outcome of cystic fibrosis is variable in different countries which may reflect environmental influences and the role of early diagnosis on long term outcomes. However, the role of early diagnosis in long-term outcomes of the disease can not be ignored.
Keywords: cystic fibrosis; outcome; long-term; morbidity; mortality cystic fibrosis; outcome; long-term; morbidity; mortality

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MDPI and ACS Style

Hassanzad, M.; Boloursaz, M.R.; Darougar, S.; Nejad, S.T.; Mohajerani, S.A.; Baghaie, N.; Hashemitari, S.K.; Velayati, A.A. Long Term Outcome of Cystic Fibrosis Patients with Multisystem Evaluation. Adv. Respir. Med. 2016, 84, 310-315. https://doi.org/10.5603/ARM.2016.0040

AMA Style

Hassanzad M, Boloursaz MR, Darougar S, Nejad ST, Mohajerani SA, Baghaie N, Hashemitari SK, Velayati AA. Long Term Outcome of Cystic Fibrosis Patients with Multisystem Evaluation. Advances in Respiratory Medicine. 2016; 84(6):310-315. https://doi.org/10.5603/ARM.2016.0040

Chicago/Turabian Style

Hassanzad, Maryam, Mohammad Reza Boloursaz, Sepideh Darougar, Sabereh Tashayoie Nejad, Seyed Amir Mohajerani, Nooshin Baghaie, Seyed Karen Hashemitari, and Ali Akbar Velayati. 2016. "Long Term Outcome of Cystic Fibrosis Patients with Multisystem Evaluation" Advances in Respiratory Medicine 84, no. 6: 310-315. https://doi.org/10.5603/ARM.2016.0040

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