scholarly journals The determinants of survival among adults with cystic fibrosis—a cohort study

2021 ◽  
Vol 40 (1) ◽  
Author(s):  
Magdalena Durda-Masny ◽  
Joanna Goździk-Spychalska ◽  
Aleksandra John ◽  
Wojciech Czaiński ◽  
Weronika Stróżewska ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Survival Rate ◽  
Lung Function ◽  
Nutritional Status ◽  
Probability Of Survival ◽  
Kaplan Meier ◽  
Chi Squared ◽  
Poor Nutritional Status ◽  
Lung Failure

Abstract 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.

scholarly journals Survival Probability of Adults With Cystic Fibrosis Depending on Their Biological Status

2020 ◽  
Author(s):  
Aleksandra John ◽  
Joanna Gozdzik-Spychalska ◽  
Magdalena Durda-Masny ◽  
Wojciech Czaiński ◽  
Natalia Pawłowska ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Survival Rate ◽  
Lung Function ◽  
Life Expectancy ◽  
Nutritional Status ◽  
Factors Affecting ◽  
Probability Of Survival ◽  
Chi Squared ◽  
Lung Failure

Abstract Background: Cystic fibrosis (CF) is one of the most common autosomal recessive disease, and the type of mutation is recognized as one of the most important factors determining the survival rate. Factors contributing to disease exacerbations, and survival rate are poor nutritional status, lung failure, and infection development by Pseudomonas Aeruginosa.The study aimed to evaluate the effect of the severity of mutation, nutritional status, lung function, and Pseudomonas aeruginosa infection on survival rate in adult patients with Cystic Fibrosis.Material and methods: A study of 124 (68 ♀ and 56 ♂) CF patients aged from 18 to 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 (PA) infection. For statistical calculations, Kaplan-Meier analysis of survival and Chi-squared test for multiple samples were used.Results: Both the type of mutation (Chi²=12.73, df= 3, p=0.005), lung function (Chi² = 15.20, df = 2, p = 0.0005), PA infection (Chi²= 11.48, df= 3, p= 0.009), and BMI (Chi²=31.08, df=4, p<0.000) significantly differentiated the probability of survival of CF patients. The shortest life expectancy was observed in patients with a severe type of mutation on both alleles, FEV1% between 40-70%, 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 of the most critical in CF adults’ lifespan. Furthermore, most exacerbations occurred between 20 and 35 years of age. Conclusions: All factors included in the study significantly influenced the survival rate of patients with cystic fibrosis. In the face of the growing population of CF patients, the research on factors affecting their life expectancy seems to take on greater importance.

Pseudomonas aeruginosa, the type of mutation, lung function, and nutritional status in adults with Cystic Fibrosis

Nutrition ◽  
2021 ◽  
pp. 111221
Author(s):  
Aleksandra John ◽  
Joanna Goździk-Spychalska ◽  
Magdalena Durda -Masny ◽  
Wojciech Czaiński ◽  
Natalia Pawłowska ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Lung Function ◽  
Nutritional Status

scholarly journals Prevalence and impact of oprD mutations in Pseudomonas aeruginosa strains in cystic fibrosis

2019 ◽  
Author(s):  
Laura J. Sherrard ◽  
Bryan A. Wee ◽  
Christine Duplancic ◽  
Kay A. Ramsay ◽  
Keyur A. Dave ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Lung Function ◽  
Hazard Rate ◽  
Carbapenem Resistance ◽  
Adverse Outcomes ◽  
Nonsense Mutations ◽  
Allelic Variants ◽  
Novel Variants

ABSTRACTDefective OprD porins contribute to carbapenem resistance and may be important in Pseudomonas aeruginosa adaptation to cystic fibrosis airways. It is unclear whether oprD mutations are fixed in populations of shared strains that are transmitted between patients or whether novel variants arise during infection. We investigated oprD sequences and antimicrobial resistance of two common Australian shared strains, constructed P. aeruginosa mutants with the most common oprD allelic variants and compared characteristics between patients with or without evidence of infection with strains harbouring these variants. Our data show that three independently acquired nonsense mutations arising from a 1-base pair substitution are fixed in strain sub-lineages. These nonsense mutations are likely to contribute to reduced carbapenem susceptibility in the sub-lineages without compromising in vitro fitness. Not only was lung function worse among patients infected with strains harbouring the nonsense mutations than those without, but they also had an increased hazard rate of lung transplantation/death. Our findings further highlight that understanding adaptive changes may help to distinguish patients with greater adverse outcomes despite infection with the same strain.

P356 Can nurse-provided education affect improvement of lung function and nutritional status in adult patients with cystic fibrosis?

2020 ◽  
Vol 19 ◽  
pp. S155-S156
Author(s):  
T. Odobasic Palkovic ◽  
D. Tjesic-Drinkovic ◽  
D. Tjesic-Drinkovic ◽  
I. Godic ◽  
I. Lalic ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Nutritional Status ◽  
Adult Patients

scholarly journals Pseudomonas aeruginosa Susceptibility Patterns and Associated Clinical Outcomes in People with Cystic Fibrosis following Approval of Aztreonam Lysine for Inhalation

2020 ◽  
pp. AAC.02327-20 ◽  
Author(s):  
Claire L Keating ◽  
Jonathan B Zuckerman ◽  
Pradeep K Singh ◽  
Matthew McKevitt ◽  
Oksana Gurtovaya ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Lung Function ◽  
Clinical Outcomes ◽  
Primary Endpoint ◽  
The United States ◽  
Study Endpoint ◽  
Primary Study ◽  
Antibiotic Exposure ◽  
Pulmonary Exacerbations

Rationale: Approval of aztreonam lysine for inhalation solution (AZLI) raised concerns that additional antibiotic exposure would potentially affect susceptibility profiles of Pseudomonas aeruginosa (PA) isolates from cystic fibrosis (CF) patients.Objective: This 5-year, prospective, observational study tracked susceptibility changes and clinical outcomes in CF patients in the United States with chronic PA infection.Methods: Sputum cultures were collected annually (2011-2016). The primary study endpoint was the proportion of subjects whose least susceptible PA isolate had an aztreonam minimum inhibitory concentration (MIC) that was >8 μg/mL (parenteral breakpoint) and increased ≥4-fold compared with the least susceptible isolate from the previous year. Annualized data for pulmonary exacerbations, hospitalizations, and FEV1% predicted were obtained from the CF Foundation Patient Registry and compared between subjects meeting/not meeting the primary endpoint.Results: 510 subjects were enrolled; 334 (65%) completed the study. A consistent proportion of evaluable subjects (13-22%) met the primary endpoint each year; and AZLI use during the previous 12 months was not associated with meeting the primary endpoint. While the annual decline in lung function was comparable for subjects meeting/not meeting the primary endpoint, more pulmonary exacerbations and hospitalizations were experienced by those who met it.Conclusions: Aztreonam susceptibility of PA remained consistent during the 5-year study. The relationship between PA isolate susceptibilities and clinical outcomes is complex; reduced susceptibility was not associated with accelerated decline in lung function, but was associated with more exacerbations and hospitalizations, likely reflecting increased overall antibiotic exposure.

scholarly journals Gastroesophageal Reflux Disease and Need for Antireflux Surgery in Children with Cystic Fibrosis: A Systematic Review on Incidence, Surgical Complications, and Postoperative Outcomes

2020 ◽  
Author(s):  
Jessica Ng ◽  
Florian Friedmacher ◽  
Caroline Pao ◽  
Paul Charlesworth
Keyword(s):  
Cystic Fibrosis ◽  
Postoperative Complications ◽  
Gastroesophageal Reflux Disease ◽  
Lung Function ◽  
Gastroesophageal Reflux ◽  
Nutritional Status ◽  
Reflux Disease ◽  
Antireflux Surgery ◽  
Published Data ◽  
Refractory Gerd

Abstract Introduction Gastroesophageal reflux disease (GERD) is associated with accelerated decline in lung health in children with cystic fibrosis (CF). Thus, antireflux surgery (ARS) is offered to a selected CF cohort with refractory GERD, but outcomes remain poorly investigated. This study aimed to determine the incidence of GERD in children with CF and to evaluate complications and outcomes of ARS. Materials and Methods A systematic literature-based search was conducted using various online databases according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The number of GERD cases in pediatric CF cohorts who underwent diagnostic investigation(s) was recorded. Data on postoperative complications and outcomes (including symptoms, lung function, and nutritional status) following ARS were analyzed. Results Ten articles (n = 289 patients) met the defined inclusion criteria (51% male; age range, 0.5 month–36 years). The overall incidence of GERD was 46% (range, 19–81%), derived from seven studies (n = 212 patients). Four publications (n = 82 patients) reported on ARS due to uncontrolled GERD. All ARSs were Nissen fundoplication (majority with gastrostomy placement). Major postoperative complications occurred in 15 (18%) patients, two required redo-ARS. Median follow-up time was 2 years (range, 3 months–6 years); 59% showed symptom improvement, and pulmonary exacerbations and decline in lung function were reduced. Nutritional status mainly improved in milder CF cases. There were no deaths related to ARS. Conclusion Approximately half of pediatric CF patients have GERD. Published data for children with CF are limited and heterogeneous in terms of GERD diagnosis and outcomes following ARS. However, ARS has shown to slow the deterioration of lung function in CF.

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