open access

Vol 84, No 6 (2016)
ORIGINAL PAPERS
Published online: 2016-12-07
Submitted: 2016-05-17
Accepted: 2016-10-10
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Long term outcome of cystic fibrosis patients with multisystem evaluation

Maryam Hassanzad, Mohammad Reza Boloursaz, Sepideh Darougar, Sabereh Tashayoie Nejad, Seyed Amir Mohajerani, Nooshin Baghaie, Seyed Karen Hashemitari, Ali Akbar Velayati
DOI: 10.5603/ARM.2016.0040
·
Adv Respir Med 2016;84(6):310-315.

open access

Vol 84, No 6 (2016)
ORIGINAL PAPERS
Published online: 2016-12-07
Submitted: 2016-05-17
Accepted: 2016-10-10

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.

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.

Get Citation

Keywords

cystic fibrosis, outcome, long-term, morbidity, mortality

About this article
Title

Long term outcome of cystic fibrosis patients with multisystem evaluation

Journal

Advances in Respiratory Medicine

Issue

Vol 84, No 6 (2016)

Pages

310-315

Published online

2016-12-07

DOI

10.5603/ARM.2016.0040

Bibliographic record

Adv Respir Med 2016;84(6):310-315.

Keywords

cystic fibrosis
outcome
long-term
morbidity
mortality

Authors

Maryam Hassanzad
Mohammad Reza Boloursaz
Sepideh Darougar
Sabereh Tashayoie Nejad
Seyed Amir Mohajerani
Nooshin Baghaie
Seyed Karen Hashemitari
Ali Akbar Velayati

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