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30.04.2020 | original article | Ausgabe 19-20/2020

Wiener klinische Wochenschrift 19-20/2020

Influence of pancreatic status, CFTR mutations, Staphylococcus aureus and/or Pseudomonas aeruginosa infection/colonization on lung function in cystic fibrosis during a 2-year follow-up period

Zeitschrift:
Wiener klinische Wochenschrift > Ausgabe 19-20/2020
Autoren:
MSe Maíra Andrade Pascoal, PhD Fernando Augusto Lima Marson, PhD Ilma Aparecida Paschoal, PhD Carlos Emílio Levy
Wichtige Hinweise

Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1007/​s00508-020-01660-7) contains supplementary material, which is available to authorized users.

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Summary

Introduction

Cystic fibrosis (CF) presents with progressive and chronic deterioration of lung function due to inflammation and colonization/infection of the lungs. This study evaluated spirometry and colonization/infection with Staphylococcus aureus and/or Pseudomonas aeruginosa over a 24-month follow-up period.

Methods

A total of 52 CF patients were studied with spirometry: forced vital capacity (FVC), forced expiratory volume in one second of FVC (FEV1), FEV1/FVC and forced expiratory flow between 25% and 75% of FVC (FEF25–75%). Colonization/infection was evaluated as predominantly S. aureus, predominantly P. aeruginosa or concomitance of these microorganisms.

Results

In CF, there was a higher prevalence of p.Phe508del/p.Phe508del genotype (16/52; 30.8%) and female gender (33/52; 63.5%). Spirometry (% predicted) markers worsened for the following groups over the 24-month period: (i) male: FVC, FEV1, FEV1/FVC, FEF25–75%; (ii) female: FVC%, FEV1, (iii) predominantly S. aureus: FVC, FEV1, FEV1/FVC, FEF25–75%; (iv) predominantly P aeruginosa: FEV1/FVC; (v) concomitant S. aureus and P. aeruginosa: FVC, FEV1. Age correlated with reduction of FVC(Liter) (Rho = −0.50) and FEV1(Liter) (Rho = −0.46). Pancreatic insufficiency and severe cystic fibrosis transmembrane regultador (CFTR) mutations were associated with deteriorating lung function.

Conclusion

In CF, deterioration of lung function as evaluated by spirometry was continuous and varied according to sex, pancreatic insufficiency, and severe CFTR mutations. No differences were observed between groups in terms of predominant type of bacteria, but the reduction of spirometry parameters was significant in the predominantly S. aureus and concomitant infection groups.

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