scholarly journals IPD1.09 Long term filamentous fungi colonization in cystic fibrosis patients – an Italian center experience

2017 ◽  
Vol 16 ◽  
pp. S57
Author(s):  
E. Fiscarelli ◽  
V. Tuccio Guarna Assanti ◽  
G. Linardos ◽  
M. Rossitto ◽  
C. Concato ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Filamentous Fungi

scholarly journals Pseudomonas aeruginosa PA80 is a cystic fibrosis isolate deficient in RhlRI quorum sensing

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Syed A. K. Shifat Ahmed ◽  
Michelle Rudden ◽  
Sabrina M. Elias ◽  
Thomas J. Smyth ◽  
Roger Marchant ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Quorum Sensing ◽  
Virulence Factors ◽  
Genomic Islands ◽  
Clinical Strain ◽  
Whole Genome ◽  
Synonymous Mutations ◽  
Wide Range

AbstractPseudomonas aeruginosa uses quorum sensing (QS) to modulate the expression of several virulence factors that enable it to establish severe infections. The QS system in P. aeruginosa is complex, intricate and is dominated by two main N-acyl-homoserine lactone circuits, LasRI and RhlRI. These two QS systems work in a hierarchical fashion with LasRI at the top, directly regulating RhlRI. Together these QS circuits regulate several virulence associated genes, metabolites, and enzymes in P. aeruginosa. Paradoxically, LasR mutants are frequently isolated from chronic P. aeruginosa infections, typically among cystic fibrosis (CF) patients. This suggests P. aeruginosa can undergo significant evolutionary pathoadaptation to persist in long term chronic infections. In contrast, mutations in the RhlRI system are less common. Here, we have isolated a clinical strain of P. aeruginosa from a CF patient that has deleted the transcriptional regulator RhlR entirely. Whole genome sequencing shows the rhlR locus is deleted in PA80 alongside a few non-synonymous mutations in virulence factors including protease lasA and rhamnolipid rhlA, rhlB, rhlC. Importantly we did not observe any mutations in the LasRI QS system. PA80 does not appear to have an accumulation of mutations typically associated with several hallmark pathoadaptive genes (i.e., mexT, mucA, algR, rpoN, exsS, ampR). Whole genome comparisons show that P. aeruginosa strain PA80 is closely related to the hypervirulent Liverpool epidemic strain (LES) LESB58. PA80 also contains several genomic islands (GI’s) encoding virulence and/or resistance determinants homologous to LESB58. To further understand the effect of these mutations in PA80 QS regulatory and virulence associated genes, we compared transcriptional expression of genes and phenotypic effects with isogenic mutants in the genetic reference strain PAO1. In PAO1, we show that deletion of rhlR has a much more significant impact on the expression of a wide range of virulence associated factors rather than deletion of lasR. In PA80, no QS regulatory genes were expressed, which we attribute to the inactivation of the RhlRI QS system by deletion of rhlR and mutation of rhlI. This study demonstrates that inactivation of the LasRI system does not impact RhlRI regulated virulence factors. PA80 has bypassed the common pathoadaptive mutations observed in LasR by targeting the RhlRI system. This suggests that RhlRI is a significant target for the long-term persistence of P. aeruginosa in chronic CF patients. This raises important questions in targeting QS systems for therapeutic interventions.

Long‐term benefits of nusinersen in a child affected by cystic fibrosis and spinal muscular atrophy type 1

2021 ◽  
Author(s):  
Giorgia Bruno ◽  
Bernadette Donnarumma ◽  
Alessia Inverardi ◽  
Paolo Buonpensiero ◽  
Angela Sepe ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Spinal Muscular Atrophy Type

scholarly journals P096 Long-term follow-up of adolescent and adult patients with cystic fibrosis: a single centre's experience

2021 ◽  
Vol 20 ◽  
pp. S68
Author(s):  
A.I. Yilmaz ◽  
G. Ünal ◽  
B.S. Kibar ◽  
P. Sevgi ◽  
O. Eĝil ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Adult Patients ◽  
Long Term Follow Up

Long‐term clinical outcome of cystic fibrosis paediatric patients presenting with meconium ileus

2020 ◽  
Vol 109 (12) ◽  
pp. 2738-2739
Author(s):  
Ioanna Loukou ◽  
Maria Moustaki ◽  
Marina Plyta ◽  
Konstantinos Douros
Keyword(s):  
Cystic Fibrosis ◽  
Clinical Outcome ◽  
Meconium Ileus ◽  
Paediatric Patients

Long term results of the domino procedure from cystic fibrosis live donors

2003 ◽  
Vol 22 (1) ◽  
pp. S183
Author(s):  
D Aravot ◽  
K Mcneal ◽  
J Dunning ◽  
S Tsui ◽  
F Wells ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Long Term Results ◽  
Live Donors

scholarly journals Long-Term Persistence of Ciprofloxacin-Resistant Haemophilus influenzae in Patients with Cystic Fibrosis

1996 ◽  
Vol 174 (6) ◽  
pp. 1345-1347 ◽  
Author(s):  
J. Campos ◽  
F. Roman ◽  
M. Georgiou ◽  
C. Garcia ◽  
R. Gomez-Lus ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Haemophilus Influenzae

Long-term Follow-up in Adult Patients with Cystic Fibrosis and Deep Intronic Splicing Variants

2021 ◽  
Vol 57 (7) ◽  
pp. 501-503
Author(s):  
Antonio Álvarez ◽  
Karina Loor ◽  
Paula Fernández-Alvarez ◽  
Silvia Gartner ◽  
Eva Polverino ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Adult Patients ◽  
Splicing Variants ◽  
Long Term Follow Up
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