Improving Detection of Rapid Cystic Fibrosis Disease Progression–Early Translation of a Predictive Algorithm Into a Point-of-Care Tool

ABSTRACT System-level modeling is beginning to be used to decipher high throughput data in the context of disease. In this study, we present an integration of expression microarray data with a genome-scale metabolic reconstruction of P seudomonas aeruginosa in the context of a chronic cystic fibrosis (CF) lung infection. A genome-scale reconstruction of P. aeruginosa metabolism was tailored to represent the metabolic states of two clonally related lineages of P. aeruginosa isolated from the lungs of a CF patient at different points over a 44-month time course, giving a mechanistic glimpse into how the bacterial metabolism adapts over time in the CF lung. Metabolic capacities were analyzed to determine how tradeoffs between growth and other important cellular processes shift during disease progression. Genes whose knockouts were either significantly growth reducing or lethal in silico were also identified for each time point and serve as hypotheses for future drug targeting efforts specific to the stages of disease progression.

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WS17-3 Cystic fibrosis and chronic rhinosinusitis: factors influencing disease progression

Journal of Cystic Fibrosis ◽

10.1016/s1569-1993(19)30219-x ◽

2019 ◽

Vol 18 ◽

pp. S32

Author(s):

K. Pianosi ◽

A. Price ◽

A.D. Dzioba ◽

M. Husein

Keyword(s):

Cystic Fibrosis ◽

Disease Progression ◽

Chronic Rhinosinusitis ◽

Factors Influencing

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Upper Airway Findings and Markers of Lung Disease Progression in Patients with Cystic Fibrosis

International Archives of Otorhinolaryngology ◽

10.1055/s-0039-3402434 ◽

2020 ◽

Vol 24 (04) ◽

pp. e434-e437

Author(s):

Luciane Mazzini Steffen ◽

Luise Sgarabotto Pezzin ◽

Natassia Sulis ◽

Nedio Steffen ◽

Leonardo Araujo Pinto

Keyword(s):

Cystic Fibrosis ◽

Lung Disease ◽

Disease Progression ◽

Nasal Polyps ◽

Nasal Swab ◽

Statistical Significance ◽

Upper Airway ◽

Forced Expiratory Volume ◽

Upper Respiratory Tract ◽

Upper Airways

Abstract Introduction Cystic fibrosis (CF) is a genetic disease that limits the quality of life mainly due to respiratory symptoms. The relationship between findings of the upper airways and CF lung disease is not yet completely understood. Objective The aim of the present study is to describe the most frequent nasal findings and pathogens in patients with CF and investigate the association between the findings of the upper respiratory tract and markers of lung disease progression. Methods Retrospective study in patients with CF from the Pediatric Pulmonology Department who underwent otorhinolaryngological evaluation between 2015 and 2017. Nasal endoscopy and nasal swab collection were part of the evaluation. The severity markers used were: percentage of predicted forced expiratory volume in the first second (FEV1%), body mass index (BMI) and the Shwachman-Kulczycki (SK) clinical score. Results A total of 48 patients with CF were included. The mean of the predicted percentage of FEV1% was 83.36 ± 30.04. The average 14 and SK score 89.11 ± 10.50. The bacteriology of the nasal swab was positive in 27 (54.1%) patients. Staphylococcus aureus was positive in 18 patients, Pseudomonas aeruginosa in 5, Pseudomonas cepacea in 3 and Stenotrophomonas maltophila in 1 patient. Nasal polyps were found in nine participants. Nasal polyps were found in nine participants and were associated with lower SK score. Conclusion The pathogens found in the upper airway were, in order: S. aureus, P. aeruginosa, P. cepacea e S. maltophila. The presence of polyps in the nasal cavity showed statistical significance and appears to have association with the prognostic factor measured by the SK score.

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