15 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/02/12

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

F508del genotype in endoscopic sinus surgery: do differences in outcomes exist between genotypic subgroups?

Int Forum Allergy Rhinol. 2017 Feb 09;:

Authors: Halderman AA, West N, Benke J, Roxbury CR, Lin SY

Abstract
BACKGROUND: The impact of endoscopic sinus surgery (ESS) on pulmonary function in cystic fibrosis (CF) patients with chronic rhinosinusitis remains unclear, as studies have demonstrated conflicting results. To date, no study has looked specifically at the impact of CF genotype on lung function after ESS. In this study, we reviewed changes in pulmonary function test (PFT) results after ESS in F508del homozygotes and heterozygotes.
METHODS: The charts of 25 patients with CF without prior lung transplant, who underwent ESS performed by the same surgeon between the period of July 2005 to July 2015, were retrospectively reviewed. Data including genotype and PFT results were collected. Patients were grouped based on genotype. Pre- and postoperative PFTs were compared.
RESULTS: Some differences in PFT outcomes after ESS could be seen on subgroup analyses. For example, when considered as a whole group, the overall cohort showed a significant improvement from preoperative FEV1 levels at 6 months after surgery (p = 0.0127). Interestingly, on subgroup analysis, the heterozygous group saw significant improvements from preoperative FEV1 levels at 6 and 12 months (p = 0.0155 and p = 0.0333, respectively). No significant improvements were seen from "baseline" FEV1 in either group at any timepoint.
CONCLUSION: Prior studies investigating the impact of ESS on pulmonary function in CF patients have shown conflicting results. To our knowledge, those earlier udies did not separate and compare different genotypes, which may have introduced heterogeneity in their patient populations. Our study suggests that grouping CF patients more strictly according to genotype or disease severity when investigating outcomes may reveal differences among various subgroups.

PMID: 28182336 [PubMed - as supplied by publisher]

Protean proteases: at the cutting edge of lung diseases.

Eur Respir J. 2017 Feb;49(2):

Authors: Taggart C, Mall MA, Lalmanach G, Cataldo D, Ludwig A, Janciauskiene S, Heath N, Meiners S, Overall CM, Schultz C, Turk B, Borensztajn KS

Abstract
Proteases were traditionally viewed as mere protein-degrading enzymes with a very restricted spectrum of substrates. A major expansion in protease research has uncovered a variety of novel substrates, and it is now evident that proteases are critical pleiotropic actors orchestrating pathophysiological processes. Recent findings evidenced that the net proteolytic activity also relies upon interconnections between different protease and protease inhibitor families in the protease web.In this review, we provide an overview of these novel concepts with a particular focus on pulmonary pathophysiology. We describe the emerging roles of several protease families including cysteine and serine proteases.The complexity of the protease web is exemplified in the light of multidimensional regulation of serine protease activity by matrix metalloproteases through cognate serine protease inhibitor processing. Finally, we will highlight how deregulated protease activity during pulmonary pathogenesis may be exploited for diagnosis/prognosis purposes, and utilised as a therapeutic tool using nanotechnologies.Considering proteases as part of an integrative biology perspective may pave the way for the development of new therapeutic targets to treat pulmonary diseases related to intrinsic protease deregulation.

PMID: 28179435 [PubMed - in process]

The challenges of maintaining momentum in CF drug development and approval.

J Cyst Fibros. 2017 Feb 05;:

Authors: Flume PA, VanDevanter DR

PMID: 28179070 [PubMed - as supplied by publisher]

17 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/02/09

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

10 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/02/07

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

13 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/02/06

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

The effect of the affordable care act dependent coverage provision on patients with cystic fibrosis.

Pediatr Pulmonol. 2017 Feb 02;:

Authors: Tumin D, Li SS, Kopp BT, Kirkby SE, Tobias JD, Morgan WJ, Hayes D

Abstract
BACKGROUND: The Patient Protection and Affordable Care Act (ACA), enacted in 2010, expanded private insurance coverage of young adults through the dependent coverage provision. This policy's implications for patients with cystic fibrosis (CF) are unknown.
METHODS: The CF Foundation Patient Registry was used to identify patients seen at CF centers, 3 years before and after ACA implementation. Patients were grouped according to eligibility for the dependent care provision (18-25 years old in 2010) or ineligibility (26-35 years old). Year-level difference-in-difference logistic regressions evaluated the association between ACA enactment and insurance status (private, public, or no insurance). Routine annual care consistent with CF Foundation guidelines (≥4 clinic visits, ≥4 respiratory cultures, and ≥2 pulmonary function tests/year) was a secondary outcome.
RESULTS: The analysis included 4,024 and 3,132 patients in the eligible and ineligible groups, respectively (35,353 patient-years). In the eligible group, 62% had private insurance before and after ACA; 18% had public insurance before and after ACA; and 5% switched from public to private insurance. In the eligible group, lack of insurance coverage became more common in the post-ACA period (relative risk ratio vs. private insurance [RRR] = 1.95; 95%CI: 1.57, 2.43; P < 0.001). Public insurance coverage also became more common (RRR = 1.50; 95%CI: 1.39, 1.62; P < 0.001). Use of routine care increased post-ACA, but more strongly in the ineligible group than in the eligible group.
CONCLUSIONS: The ACA dependent coverage provision did not increase private insurance coverage or use of routine care among CF patients who were potentially affected by this policy. Pediatr Pulmonol. 2016; 9999:XX-XX. © 2016 Wiley Periodicals, Inc.

PMID: 28152283 [PubMed - as supplied by publisher]

9 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/02/02

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

Analysis of Pseudomonas aeruginosa PAO1 Biofilm Protein Profile After Exposure to n-Butanolic Cyclamen coum Extract Alone and in Combination with Ciprofloxacin.

Appl Biochem Biotechnol. 2017 Jan 30;:

Authors: Shafiei M, Abdi-Ali A, Shahcheraghi F, Vali H, Shahbani Zahiri H, Akbari Noghabi K

Abstract
Pseudomonas aeruginosa biofilm-related infections are the major cause of premature death in cystic fibrosis patients. Strategies to induce biofilm dispersal are of interest, because of their potential in preventing biofilm-related infections. Our previous work demonstrated that n-butanolic Cyclamen coum extract with ciprofloxacin could eliminate 1- and 3-day-old P. aeruginosa PAO1 biofilms. To gain new insights into the role of C. coum extract and its synergistic effect with ciprofloxacin in eliminating P. aeruginosa PAO1 biofilms, two-dimensional gel electrophoresis (2-DE) in combination with mass spectrometry-based protein identification were used. Changes in the bacterial protein expression were analyzed when 3-day-old biofilm cells were exposed to the C. coum extract alone and in combination with ciprofloxacin. Proteins involved in alginate biosynthesis, quorum sensing, adaptation/protection, carbohydrate and amino acid metabolism showed a weaker expression in the C. coum extract-ciprofloxacin-treated biofilm cells compared to those in the untreated cells. Interestingly, the proteome of C. coum extract-ciprofloxacin-treated biofilm revealed more resemblance to the planktonic phenotype than to the biofilm phenotype. It appears that saponin extract in combination with ciprofloxacin causes biofilm disruption due to several mechanisms such as motility induction, cell envelope integrity perturbation, stress protein expression reduction, and more importantly, signal transduction perturbation. In conclusion, exposure to a combination of biofilm dispersal such as saponin extract and antimicrobial agents may offer a novel strategy to control preestablished, persistent P. aeruginosa biofilms and biofilm-related infections.

PMID: 28138928 [PubMed - as supplied by publisher]

Achromobacter xylosoxidans infection in cystic fibrosis siblings with different outcomes: Case reports.

Respir Med Case Rep. 2017;20:98-103

Authors: Firmida MC, Marques EA, Leão RS, Pereira RH, Rodrigues ER, Albano RM, Folescu TW, Bernardo V, Daltro P, Capone D, Lopes AJ

Abstract
INTRODUCTION: The clinical relevance of Achromobacter xylosoxidans infection in cystic fibrosis (CF) remains controversial. This emerging agent in CF has been associated with increased lung inflammation, more frequent exacerbations and more severe lung disease. We describe a pair of CF siblings chronically colonized by the same multilocus genotype of A. xylosoxidans with different clinical courses, and assess whether this species may have developed any virulence traits and antimicrobial resistance that could have contributed to their singular outcomes.
CASE PRESENTATION: Two siblings were positive for the F508del and Y1092X mutations, and were chronically colonized by Pseudomonas aeruginosa and Staphylococcus aureus. The female patient had a more severe CF phenotype and faster clinical deterioration than her brother. Her pulmonary function and computed tomography scan lesions were worse than those of her brother, and both parameters progressively declined. She died at 14 years of age, when he was 18. All isolates of A. xylosoxidans were biofilm producers. Achromobacter xylosoxidans showed less swarming motility in the female patient.
CONCLUSIONS: Biofilm production and diminution of motility allow persistence. Only swarming motility differed between the isolates recovered from the two siblings, but this finding is not sufficient to explain the different clinical outcomes despite their similar genotypes. Modifier genes, unknown environmental factors and female gender can partially explain differences between these siblings. We were unable to correlate any microbiological findings with their clinical courses, and more translational studies are necessary to decrease the gap of knowledge between laboratory and clinical data to promote better clinical interventions.

PMID: 28138423 [PubMed - in process]

Inhibition of Pseudomonas aeruginosa by Peptide-conjugated Phosphorodiamidate Morpholino Oligomers.

Antimicrob Agents Chemother. 2017 Jan 30;:

Authors: Howard JJ, Sturge CR, Moustafa DA, Daly SM, Marshall-Batty KR, Felder CF, Zamora D, Yabe-Gill M, Labandeira-Rey M, Bailey SM, Wong M, Goldberg JB, Geller BL, Greenberg DE

Abstract
Pseudomonas aeruginosa is a highly virulent, multidrug-resistant pathogen that causes significant morbidity and mortality in hospitalized patients and is particularly devastating in those with cystic fibrosis (CF). Increasing antibiotic resistance coupled with decreasing numbers of antibiotics in the developmental pipeline demands novel antibacterial approaches. Here we tested peptide-conjugated phosphorodiamidate morpholino oligomers (PPMOs), which inhibit translation of complementary mRNA from specific, essential genes in P. aeruginosa PPMOs targeted to acpP, lpxC, and rpsJ, inhibited P. aeruginosa growth in many clinical strains and activity of PPMOs could be enhanced 2- to 8-fold by the addition of polymyxin B nonapeptide (PMBN) at sub-inhibitory concentrations. The PPMO targeting acpP was also effective at preventing P. aeruginosa PAO1 biofilm formation and at reducing existing biofilms. Importantly, treatment with various combinations of a PPMO and a traditional antibiotic demonstrated synergistic growth inhibition, the most effective of which was the PPMO targeting rpsJ with tobramycin. Furthermore, treatment of P. aeruginosa PA103 infected mice with PPMOs targeting acpP, lpxC, or rpsJ significantly reduced bacterial burden in the lungs at 24 hours by almost three logs. Altogether, this study demonstrates that PPMOs targeting the essential genes acpP, lpxC, or rpsJ in P. aeruginosa are highly effective at inhibiting growth in vitro and in vivo. These data suggest that PPMOs alone or in combination with antibiotics represent a novel approach to address the problems associated with rapidly increasing antibiotic resistance in P. aeruginosa.

PMID: 28137807 [PubMed - as supplied by publisher]

Importance of the exopolysaccharide matrix in antimicrobial tolerance of Pseudomonas aeruginosa aggregates.

Antimicrob Agents Chemother. 2017 Jan 30;:

Authors: Goltermann L, Tolker-Nielsen T

Abstract
Pseudomonas aeruginosa is an opportunistic pathogen that can infect the lungs of cystic fibrosis (CF) patients, and persist in the form of antibiotic tolerant aggregates in the mucus. It has recently been suggested that such aggregates are formed due to restricted bacterial motility independent of the production of extracellular matrix components, and that they do not rely on an extracellular matrix for antimicrobial tolerance. However, we show here that biofilm matrix over-expression, as displayed by various clinical isolates, significantly protects P. aeruginosa aggregates against antimicrobial treatment. Alginate-overproducing mucA mutant bacteria growing in aggregates showed highly increased antibiotic tolerance compared to wild type bacteria in aggregates. Deletion of algD in the mucA strain abrogated alginate production and reversed the antibiotic tolerance displayed by the aggregates to a level similar to that observed for aggregates formed by the wild type. The mutant strains P. aeruginosa ΔwspF and ΔyfiR both over-produce Pel and Psl exopolysaccharide, and when these bacteria grew in aggregates they showed highly increased antibiotic tolerance compared to wild type bacteria growing in aggregates. However, ΔwspF and ΔyfiR mutant strains, deficient in Pel/Psl production due to additional ΔpelAΔpslBCD deletions, formed aggregates that displayed antibiotic tolerance levels close to that of wild type aggregates. These results suggest that biofilm matrix components such as alginate, Pel and Psl do play a role in the tolerance towards antimicrobials when bacteria grow as aggregates.

PMID: 28137803 [PubMed - as supplied by publisher]

The mechanism of killing by the proline-rich peptide Bac7(1-35) against clinical strains of Pseudomonas aeruginosa differs from that showed against other Gram-negative bacteria.

Antimicrob Agents Chemother. 2017 Jan 30;:

Authors: Runti G, Benincasa M, Giuffrida G, Devescovi G, Venturi V, Gennaro R, Scocchi M

Abstract
P. aeruginosa infections represent a serious threat to worldwide health. Proline-rich antimicrobial peptides (PR-AMPs), a particular group of peptide antibiotics, have demonstrated in vitro activity against P. aeruginosa strains. Here we show that the mammalian PR-AMP Bac7 (1-35) is active against some multi-drug-resistant cystic fibrosis isolates of P. aeruginosa By confocal microscopy and cytometric analyses we investigated the mechanism of killing against P. aeruginosa PAO1 and three selected isolates and we observed that the peptide inactivated the target cells by disrupting their cellular membranes. This effect is deeply different from that previously described for PR-AMPs in E. coli and S. typhimurium, where these peptides act intracellularly after having been internalized by means of the transporter SbmA without membranolytic effects. The heterologous expression of SbmA in PAO1 cells enhanced the internalization of Bac7 (1-35) into the cytoplasm making the bacteria more susceptible to the peptide, but at the same time more resistant to the membrane lysis similarly to what occurs in E. coli Results evidenced a new mechanism of action for PR-AMPs and indicate that Bac7 has multiple and variable modes of action that depends on the characteristics of the different target species and the possibility to be internalized by bacterial transporters. This feature broadens the spectrum of activity of the peptide and makes the development of peptide-resistant bacteria a more difficult process.

PMID: 28137800 [PubMed - as supplied by publisher]

15 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/01/31

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

9 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/01/29

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

8 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/01/28

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

8 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/01/27

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

6 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/01/26

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

8 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

"cystic fibrosis"

These pubmed results were generated on 2017/01/25

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.