open access

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

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

References (24)
  1. Boat TF. Cystic fibrosis. In: Nelson textbook of pediatrics. . Pa: WB Saunders Co, Philadelphia 2000: 1315–1327.
  2. Faro A, Michelson PH, Ferkol TW. Pulmonary disease in CF. In: Kendig and Chernick’s disorsers of the respiratory tract in children. Elsevier, Saunders (eighth edition) 2012: 370–378.
  3. Taylor-Robinson D, Whitehead M, Diderichsen F, et al. Understanding the natural progression in %FEV1 decline in patients with cystic fibrosis: a longitudinal study. Thorax. 2012; 67(10): 860–866.
  4. Collaco JM, Vanscoy L, Bremer L, et al. Interactions between secondhand smoke and genes that affect cystic fibrosis lung disease. JAMA. 2008; 299(4): 417–424.
  5. Ratjen F, Döring G. Cystic fibrosis. Lancet. 2003; 361(9358): 681–689.
  6. Babinski D, Trawinska-Bartnicka M. Rhinosinusitis in cystic fibrosis: not a simple story. Int J Pediatr Otorhinolaryngol. 2008; 72(5): 619–624.
  7. Dodge JA, Lewis PA, Stanton M, et al. Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur Respir J. 2007; 29(3): 522–526.
  8. Mcintyre K. Gender and Survival in CF. Curr Opin Pulm Med . 2013; 19: 692–697.
  9. Farjadian S, Moghtaderi M, Kashef S, et al. Clinical and genetic features in patients with cystic fibrosis in southwestern iran. Iran J Pediatr. 2013; 23(2): 212–215.
  10. McAdam PR, Holmes A, Templeton KE, et al. Adaptive evolution of Staphylococcus aureus during chronic endobronchial infection of a cystic fibrosis patient. PLoS One. 2011; 6(9): e24301.
  11. Pernet E, Guillemot L, Burgel PR, et al. Pseudomonas aeruginosa eradicates Staphylococcus aureus by manipulating the host immunity. Nat Commun. 2014; 5: 5105.
  12. Khalilzadeh S, Boloursaz M, Baghaie N, et al. Microbial Colonization and Drug Resistance in Patients with Cystic Fibrosis. Journal of Comprehensive Pediatrics. 2012; 3(1): 25–28.
  13. Baghaie N, Kalilzadeh S, Hassanzad M, et al. Determination of mortality from cystic fibrosis. Pneumologia. 2010; 59(3): 170–173.
  14. Speirs JJ, van der Ent CK, Beekman JM. Effects of Aspergillus fumigatus colonization on lung function in cystic fibrosis. Curr Opin Pulm Med. 2012; 18(6): 632–638.
  15. Khalilzadeh S, Kahkouee S, Hassanzad M, et al. The correlation of brody high resolution computed tomography scoring system with clinical status and pulmonary function test in patients with cystic fibrosis. Iran J Med Sci. 2011; 36(1): 18–23.
  16. Kahkouee S, Namini AK, Boloursaz MR. Quantitative Evaluation of High-Resolution CT Findings in Advanced Cystic Fibrosis Patients Based on the Brody Scoring. Journal of Comprehensive Pediatrics. 2014; 5(1).
  17. Webb AK, Woolnough E. Candida albicans infection in adults with cystic fibrosis. J R Soc Med. 2006; 99 Suppl 46: 13–16.
  18. Chotirmall SH, O'Donoghue E, Bennett K, et al. Sputum Candida albicans presages FEV₁ decline and hospital-treated exacerbations in cystic fibrosis. Chest. 2010; 138(5): 1186–1195.
  19. Oomen K, April MM. Sinonsal manifestations of CF. Int J Otolaryngol. 2012: 789572.
  20. Bright-Thomas RJ, Ray SG, Webb AK. Pulmonary artery pressure in cystic fibrosis adults: characteristics, clinical correlates and long-term follow-up. J Cyst Fibros. 2012; 11(6): 532–538.
  21. Stephenson AL, Tom M, Berthiaume Y, et al. A contemporary survival analysis of individuals with cystic fibrosis: a cohort study. Eur Respir J. 2015; 45(3): 670–679.
  22. Khalilzadeh S, Hassanzad M, Baghaie N, et al. Shwachman Score in Clinical Evaluation of Cystic Fibrosis. Journal of Comprehensive Pediatrics. 2012; 4(1): 82–85.
  23. Hassanzad M, Khalilzadeh S, Eslampanah Nobari S, et al. Cotinine level is associated with asthma severity in passive smoker children. Iran J Allergy Asthma Immunol. 2015; 14(1): 67–73.
  24. Kopp BT, Sarzynski L, Khalfoun S, et al. Detrimental effects of secondhand smoke exposure on infants with cystic fibrosis. Pediatr Pulmonol. 2015; 50(1): 25–34.

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

Czasopismo Pneumonologia i Alergologia Polska dostęne jest również w Ikamed - księgarnia medyczna

Wydawcą serwisu jest "Via Medica sp. z o.o." sp.k., ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail: viamedica@viamedica.pl