Influence of pancreatic status, CFTR mutations, Staphylococcus aureus and/or Pseudomonas aeruginosa infection/colonizati

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Wien Klin Wochenschr https://doi.org/10.1007/s00508-020-01660-7

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 Maíra Andrade Pascoal · Fernando Augusto Lima Marson · Ilma Aparecida Paschoal · Carlos Emílio Levy Received: 5 December 2018 / Accepted: 7 April 2020 © Springer-Verlag GmbH Austria, part of Springer Nature 2020

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.

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.

F. A. L. Marson, PhD · I. A. Paschoal, PhD Department of Clinical Medicine, Faculty of Medical Sciences, University of Campinas, Tessália Vieira de Camargo Street, 126, Cidade Universitária Zeferino Vaz, Barão Geraldo, ZIP CODE: 13083-887 Campinas, São Paulo, Brazil [email protected]

M. A. Pascoal, MSe · F. A. L. Marson, PhD () Department of Pediatrics, Faculty of Medical Sciences, University of Campinas, Tessália Vieira de Camargo Street, 126, Cidade Universitária Zeferino Vaz, Barão Geraldo, ZIP CODE: 13083-887 Campinas, São Paulo, Brazil [email protected] M. A. Pascoal, MSe [email protected] F. A. L. Marson, PhD Department of Medical Genetics and Genomic Medicine, Faculty of Medical Sciences, University of Campinas, Tessália Vieira de Camargo Street, 126, Cidade Universitária Zeferino Vaz, Barão Geraldo, ZIP CODE: 13083-887 Campinas, São Paulo, Brazil

C. E. Levy, PhD () Department of Clinical Pathology, Faculty of Medical Sciences, University of Campinas, Tessália Vieira de Camargo Street, 126, Cidade