scholarly journals Infectious and nutritional mechanisms in children with cystic fibrosis

2015 ◽  
Vol 67 (3) ◽  
pp. 1063-1066
Author(s):  
Ramona Diaconu ◽  
Laura Bozomitu ◽  
Emil Anton ◽  
Paula Popovici ◽  
Carmen Anton ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Moraxella Catarrhalis ◽  
Burkholderia Cepacia ◽  
Genetic Background ◽  
Pulmonary Infection ◽  
Respiratory Infections ◽  
Positive Impact ◽  
Infectious Risk ◽  
Increased Susceptibility

Cystic fibrosis is a polymorphic disease characterized by severe genetic dysfunctions. Besides the complex genetic background, most patients with cystic fibrosis also have increased susceptibility to infections and and their nutritional status is affected. Chronic pulmonary infection and gastrointestinal or nutritional abnormalities are characteristics of this disorder. Of our selected 56 subjects, 21.28% presented a pulmonary condition, and 28.57% digestive deregulation. We also observed that the infectious status in cystic fibrosis was dominated by respiratory infections (71.42%), and the main pathogens were Streptococcus. pneumoniae, Haemophilus. influenzae, Moraxella catarrhalis, Pseudomonas aeruginosa, Burkholderia cepacia, Staphylococcus aureus and Aspergillus. Additionally, it seems that while pulmonary function is strongly linked with adequate nutrition and weight gain, monitoring weight and pulmonary function are fundamental aspects in understanding the mechanisms of cystic fibrosis, and a very important parameter for a better management of this disorder. Early identification of nutrition and infectious risk factors is necessary for the effective and timely interventions that can have a positive impact on disease outcome. Infectious and nutritional aspects and interactions between the two are described. It is expected that management of cystic fibrosis will significantly improve.

scholarly journals Differential Persistence among Genomovars of the Burkholderia cepacia Complex in a Murine Model of Pulmonary Infection

2002 ◽  
Vol 70 (5) ◽  
pp. 2715-2720 ◽  
Author(s):  
Karen K. Chu ◽  
Donald J. Davidson ◽  
T. Keith Halsey ◽  
Jacqueline W. Chung ◽  
David P. Speert
Keyword(s):  
Cystic Fibrosis ◽  
Clinical Outcomes ◽  
Murine Model ◽  
Burkholderia Cepacia ◽  
Pulmonary Infection ◽  
Burkholderia Cepacia Complex ◽  
Clinical Infection ◽  
Minimal Toxicity

ABSTRACT Cystic fibrosis patients infected with strains from different genomovars of the Burkholderia cepacia complex can experience diverse clinical outcomes. To identify genomovar-specific determinants that might be responsible for these differences, we developed a pulmonary model of infection in BALB/c mice. Mice were rendered leukopenic by administration of cyclophosphamide prior to intranasal challenge with 1.6 × 104 bacteria. Five of six genomovar II strains persisted at stable numbers in the lungs until day 16 with minimal toxicity, whereas zero of seven genomovar III strains persisted but resulted in variable toxicity. We have developed a chronic pulmonary model of B. cepacia infection which reveals differences among genomovars in terms of clinical infection outcome.

scholarly journals Pseudomonas aeruginosa bacteremia and prostatitis in a patient with cystic fibrosis

2013 ◽  
Vol 7 (1-2) ◽  
pp. 1
Author(s):  
Anju Anand ◽  
Elizabeth Tullis ◽  
Anne Stephenson ◽  
J. Curtis Nickel ◽  
Michael J. Leveridge
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Lower Urinary Tract ◽  
Pulmonary Infection ◽  
Respiratory Infections ◽  
Bacterial Prostatitis ◽  
Systemic Dissemination ◽  
Microbial Etiology ◽  
Acute Bacterial Prostatitis ◽  
Lower Urinary

Patients with cystic fibrosis (CF) commonly suffer chronic respiratory infections, although systemic dissemination is relatively rare. Acute bacterial prostatitis presents dramatically and is believed to be mostly caused by local migration (with or without instrumentation) of the lower urinary tract and presents with a predictable microbial etiology. We report a case of a 26-year-old man presenting with acute Pseudomonas aeruginosa bacterial prostatitis due to hematogenous propagation from a chronic pulmonary infection.

scholarly journals An Overview on Cystic Fibrosis

1970 ◽  
Vol 7 (5) ◽  
pp. 80-91
Author(s):  
Swetha Reddy Swetha Reddy Singireddy1 ◽  
Susmitha Varagandhi ◽  
Aishwarya Goud Jagiri ◽  
Rohith Kumar Kadarla
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Respiratory Infections ◽  
Severe Disease ◽  
Transmembrane Conductance Regulator ◽  
Net Benefit ◽  
Transmembrane Conductance ◽  
Oral Rehydration ◽  
Inhaled Antibiotics ◽  
Good Nutrition

Cystic fibrosis is an autosomal recessive disease. It is caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene on chromosome 7 that codes for a protein transmembrane conductance regulator (CFTR) protein which functions as a transmembrane cAMP-activated chloride channel.  CFTR also affects other ion channels, most notably blocking the influx of sodium into the cell through the epithelial sodium channel. The CFTR abnormality has been shown to produce a number of changes in the airway, including acidification and decreased water and ion transit. A pulmonary exacerbation of CF is usually identified by an increase in cough and sputum and a decrease in pulmonary function. Disease manifests in many organs, but most notably the upper and lower airways, pancreas, bowel, and reproductive tracts. Pulmonary function testing is a major tool for evaluating and monitoring disease state and progression in CF. Spirometry is the commonly used pulmonary function test. Management of CF requires good nutrition and appropriate supplementation of vitamins and pancreatic enzymes. The Cystic Fibrosis Foundation recommends the following treatments as having a high certainty of substantial net benefit, grade A, for moderate-to-severe disease: inhaled tobramycin, dornasealfa, ivacaftor, and inhaled aztreonam. Preventing or treating intestinal blockages—oral rehydration and osmotic laxatives (incomplete blockage) and hyperosmolar contrast enemas (complete DIOS). Antibiotics are the major components of CF treatment and are administered chronically (e.g. inhaled antibiotics, macrolides used for their immunomodulatory properties) or intermittently to prevent, eradicate, control or treat respiratory infections. Lumacaftor (200 mg) + ivacaftor (125 mg), Orkambi, is the first approved CFTR corrector and potentiator combination therapy. Keywords: 

Efficacy of Glutathione for Patients With Cystic Fibrosis: A Meta-analysis of Randomized-Controlled Studies

2019 ◽  
Vol 34 (1) ◽  
pp. 115-121 ◽  
Author(s):  
Jinqiu Zhao ◽  
Wenxiang Huang ◽  
Shujun Zhang ◽  
Jing Xu ◽  
Wei Xue ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Positive Impact ◽  
Meta Analysis ◽  
Forced Expiratory Volume ◽  
Cochrane Library ◽  
Control Group ◽  
Randomized Controlled ◽  
Randomized Controlled Studies ◽  
The Impact

Introduction The impact of glutathione on pulmonary function remains elusive for patients with cystic fibrosis. The aim of this systematic review and meta-analysis is to explore the influence of glutathione versus placebo on pulmonary function of cystic fibrosis. Methods We search PubMed, EMbase, Web of science, EBSCO, and Cochrane library databases through May 2019, and randomized-controlled trials (RCTs) regarding the effect of glutathione on pulmonary function of cystic fibrosis are included in this meta-analysis. Results Four RCTs are included. Compared with control group in patients with cystic fibrosis, glutathione treatment shows positive impact on forced expiratory volume 1 second (FEV1) (mean difference [MD] = 0.19; 95% confidence interval (CI), 0.10–0.28; P < .0001) and body mass index (MD = 0.27; 95% CI, 0.02–0.51; P = .03), but has no obvious influence on 6-minute walk test (standard MD = 0.28; 95% CI, −0.08 to 0.64; P = .13), number of exacerbations (MD = −0.10; 95% CI, −0.34 to 0.15; P = .43), abdominal pain or distal intestinal obstruction (risk ratios [RR] = 0.78; 95% CI, 0.32–1.90; P = .58), or hemoptysis (RR = 1.87; 95% CI, 0.43–8.26; P = .41). Conclusions Glutathione treatment provides some benefits to improve pulmonary function of patients with cystic fibrosis, as evidenced by the increase in FEV1.

scholarly journals Inhibition of co-colonizing cystic fibrosis-associated pathogens by Pseudomonas aeruginosa and Burkholderia multivorans

Microbiology ◽  
2014 ◽  
Vol 160 (7) ◽  
pp. 1474-1487 ◽  
Author(s):  
Anne Costello ◽  
F. Jerry Reen ◽  
Fergal O’Gara ◽  
Máire Callaghan ◽  
Siobhán McClean
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Burkholderia Cepacia ◽  
Inflammatory Responses ◽  
Respiratory Infections ◽  
Lung Damage ◽  
Burkholderia Cenocepacia ◽  
Antibiotic Resistant ◽  
Bacterial Dna ◽  
Burkholderia Multivorans

Cystic fibrosis (CF) is a recessive genetic disease characterized by chronic respiratory infections and inflammation causing permanent lung damage. Recurrent infections are caused by Gram-negative antibiotic-resistant bacterial pathogens such as Pseudomonas aeruginosa, Burkholderia cepacia complex (Bcc) and the emerging pathogen genus Pandoraea. In this study, the interactions between co-colonizing CF pathogens were investigated. Both Pandoraea and Bcc elicited potent pro-inflammatory responses that were significantly greater than Ps. aeruginosa. The original aim was to examine whether combinations of pro-inflammatory pathogens would further exacerbate inflammation. In contrast, when these pathogens were colonized in the presence of Ps. aeruginosa the pro-inflammatory response was significantly decreased. Real-time PCR quantification of bacterial DNA from mixed cultures indicated that Ps. aeruginosa significantly inhibited the growth of Burkholderia multivorans, Burkholderia cenocepacia, Pandoraea pulmonicola and Pandoraea apista, which may be a factor in its dominance as a colonizer of CF patients. Ps. aeruginosa cell-free supernatant also suppressed growth of these pathogens, indicating that inhibition was innate rather than a response to the presence of a competitor. Screening of a Ps. aeruginosa mutant library highlighted a role for quorum sensing and pyoverdine biosynthesis genes in the inhibition of B. cenocepacia. Pyoverdine was confirmed to contribute to the inhibition of B. cenocepacia strain J2315. B. multivorans was the only species that could significantly inhibit Ps. aeruginosa growth. B. multivorans also inhibited B. cenocepacia and Pa. apista. In conclusion, both Ps. aeruginosa and B. multivorans are capable of suppressing growth and virulence of co-colonizing CF pathogens.

Sign in / Sign up

Export Citation Format

Share Document