The determinants of survival among adults with cystic fibrosis-a cohort study

Bibliographic Data

ID5200280
AuthorsMagdalena Durda‐masny (0000-0002-5895-9754, Adam Mickiewicz University in Poznań), Magdalena Durda-Masny, Joanna Goździk-Spychalska (0000-0002-4518-7070, Poznan University of Medical Sciences), Aleksandra John (Adam Mickiewicz University in Poznań), Wojciech Czaiński (Poznan University of Medical Sciences), Weronika Stróżewska (Adam Mickiewicz University in Poznań), Natalia Pawłowska (0000-0001-7388-8360, Adam Mickiewicz University in Poznań), Jolanta Wlizło (Poznan University of Medical Sciences), Halina Batura-Gabryel, Halina Batura‐Gabryel (0000-0003-4555-7905, Poznan University of Medical Sciences), Anita Szwed (0000-0001-7516-5019, Adam Mickiewicz University in Poznań, corresponding author)
Year2021
Volume40
Issue1
Pages19-19
Publication date2021-12-01
Peer ReviewedYes
Open AccessYes
TypeARTICLE
VenueJournal of PHYSIOLOGICAL ANTHROPOLOGY (JOURNAL)
Journal identifiersISSN: 1880-6791 • E-ISSN: 1880-6805
PublisherSpringer Science and Business Media LLC (PUBLISHER)
DOI10.1186/s40101-021-00269-7
PMID34749804
OpenAlexW3214225559
LanguageEN
Citations received1
References cited29

Background Cystic fibrosis (CF) is one of the most common autosomal recessive diseases. Factors contributing to disease exacerbations and survival rate of CF patients are type of mutation in the CFTR gene, poor nutritional status, lung failure, and infection development by Pseudomonas aeruginosa. The study aimed to evaluate the relationship between the severity of mutation, nutritional status, lung function, and Pseudomonas aeruginosa prevalence and survival rate in adult patients with cystic fibrosis. Methods A study of 124 (68 ♀ and 56 ♂) adults with CF aged 18-51 years were evaluated for (a) type of mutation in the CFTR gene, (b) nutritional status (BMI), (c) lung function (FEV1%), and (d) Pseudomonas aeruginosa prevalence. For statistical calculations, Kaplan-Meier analysis of survival, chi-squared test for multiple samples, and logistic regression were used. Results The type of mutation (χ2 = 12.73, df = 3, p = 0.005), FEV1% (χ2 = 15.20, df = 2, p = 0.0005), Pseudomonas aeruginosa prevalence (χ2 = 11.48, df = 3, p = 0.009), and BMI (χ2 = 31.08, df = 4, p < 0.000) significantly differentiated the probability of survival of patients with CF. The shortest life expectancy was observed in patients with a severe type of mutation on both alleles, FEV1% < 40, subjects in whom Pseudomonas culture was extensively drug-resistant or pandrug-resistant, and patients whose BMI was lower than 18.5 kg/m2. The period from 30 to 40 years of age was the most critical in CF adults' lifespan. The risk of adults with CF death doubled with Pseudomonas aeruginosa prevalence (OR = 2.06, 95% CI 1.29; 2.28) and eightfold when the bacteria acquired antibiotic resistance (OR = 8.11, 95% CI 1.67; 38.15). Conclusions All factors included in the study were significantly related to the survival rate of patients with cystic fibrosis

Biology · Body mass index · Cystic fibrosis · Pseudomonas aeruginosa · Survival analysis · Bacterial biofilms and quorum sensing · Biological Research and Disease Studies · Cystic Fibrosis Research Advances · Medicine · Gastroenterology · Genetics · Internal Medicine

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