title: Long term outcome of cystic fibrosis patients with multisystem evaluation.
abstract: BACKGROUND
  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.
  
  
  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.
authors.forename: Maryam
authors.initials: M
authors.lastname: Hassanzad
authors.forename: Mohammad Reza
authors.initials: MR
authors.lastname: Boloursaz
authors.affiliation: 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. sepidehdarougar@yahoo.com.
authors.forename: Sepideh
authors.initials: S
authors.lastname: Darougar
authors.forename: Sabereh
authors.initials: S
authors.lastname: Tashayoie Nejad
authors.forename: Seyed Amir
authors.initials: SA
authors.lastname: Mohajerani
authors.forename: Nooshin
authors.initials: N
authors.lastname: Baghaie
authors.forename: Seyed Karen
authors.initials: SK
authors.lastname: Hashemitari
authors.forename: Ali Akbar
authors.initials: AA
authors.lastname: Velayati
keywords: cystic fibrosis; long-term; morbidity; mortality; outcome