Page 4 - CF Roundtable - Winter/Spring 2026

RESEARCH
ROUNDUP
Compiled by: Aimee Lecointre
Caries (Tooth Decay) In A Cohort Of Adults
With Cystic Fibrosis: A Cross-Sectional Study
https://tinyurl.com/2c96prrm
A cross-sectional study of 92 adults with CF and 92 adults without
CF was undertaken in Cork University Dental School and Hospital.
The median age for study group and control group participants was
31 years and 27 years, respectively. All participants completed a
detailed questionnaire before undergoing a clinical examination that
recorded demographic, social and oral health variables. Caries were
recorded using the Decayed, Missing and Filled Teeth (DMFT) index.
All data were statistically analysed using the Wilcoxon rank-sum
test, chi-squared test and Fisher’s test. Negative binomial models
were also used to analyse data. The study group had a higher
mean DMFT score compared to the control group. While the study
group had a higher DMFT, the only component that was statistically
significant between the groups was the Decayed Teeth component.
In this study, the cohort of people with CF had more caries than
people without CF. Further research is required to establish if
underlying systemic conditions, social and behavioural factors, or
a combination of the aforementioned are responsible for a higher
caries experience in this study group.
A Chronic Pseudomonas Aeruginosa Mouse Lung Infection
Modeling The Mucus Obstruction, Lung Function, And
Inflammation Of Human Cystic Fibrosis
https://tinyurl.com/tn59buke
Mouse models of CF have been used to study chronic lung
infections; however, these models have lacked the airway mucus
that defines human CF pathophysiology and required the use of
mucoid Pseudomonas aeruginosa. Here, researchers combined
alternative models and established a chronic P. aeruginosa lung
infection model to recapitulate nutrient and mucus characteristics
of the human CF lung environment and test the effects of chronic
infections on bacterial burden, lung function, and the immune
response. Using wild-type SCFM2-C57BL/6 mice as controls,
SCFM2-Scnn1b-Tg mice failed to clear bacterial infections, and
lung function measurements showed that infected the mice had
decreased inspiratory capacity and compliance, elevated airway
resistance, and significantly reduced forced expiratory volumes.
Flow cytometry and cytokine arrays showed that, like people with
CF, the mice developed inflammation characterized by neutrophil
and eosinophil infiltration and Th2 lymphocytic cytokine responses.
Chronically infected mice developed an exacerbated mix of innate
and Th1, Th2, and Th17-mediated inflammation, causing higher
lung cellular damage and elevated numbers of unusual Siglec F+
neutrophils. These types of mice will be useful for investigating
bacterial pathogenesis by non-mucoid P. aeruginosa, including
treatments and the roles of Siglec F+ neutrophils in CF inflammation.
4 CF Roundtable Winter/Spring 2026
Vancomycin Monitoring for Treatment of Acute
Pulmonary Exacerbations of Adult Cystic Fibrosis Patients
https://tinyurl.com/294sej5a
Therapeutic drug monitoring (TDM) for vancomycin (VAN) in
adult pwCF historically has utilized trough concentrations. Recent
VAN TDM guidelines recommend area under the curve (AUC)
monitoring to reduce the risk of acute kidney injury (AKI), despite
limited evidence to support this practice in adult pwCF. Receipt of
concurrent nephrotoxins was more common in the AUC cohort
than in the trough cohort, but the rate of AKI was similar. AUC
monitoring was associated with earlier achievement of TDM goal
vs. 2 days, lower total daily doses vs. 57.5 mg/kg/day, and fewer
regimen changes vs. 2 changes. In patients with MRSA, pulmonary
function recovery, readmission, and mortality were similar. In
adult pwCF, the incidence of AKI was similar between AUC and
trough monitoring cohorts; however, AUC monitoring achieved
therapeutic targets sooner with fewer regimen modifications
without significantly increasing the number of concentrations
compared to trough monitoring.
Proteostasis Landscapes Of Cystic Fibrosis Variants
Reveal Drug Response Vulnerability
https://tinyurl.com/35s6jp7f
Approximately 3% of persons with CF harbor poorly responsive
CFTR variants. In this study researchers used affinity purification
mass spectrometry proteomics to profile the protein homeostasis
(proteostasis) changes of CFTR variants during correction to
assess modulated interactions with protein folding and maturation
pathways. Responsive variant interactions converged on similar
proteostasis pathways during correction. In contrast, poorly
responsive variants subtly diverged, revealing a partial restoration
of protein quality control surveillance and partial correction.
Computational structural modeling showed that corrector VX-
445 failed to confer enough NBD1 stability to poor responders.
NBD1 secondary stabilizing mutations rescued poorly responsive
variants, revealing structural vulnerabilities in NBD1 required for
treating poor responders. This study provides a framework for
discerning the underlying protein quality control and structural
defects of CFTR variants not reached with existing drugs to expand
therapeutics to all susceptible CFTR variants.
 Research Roundup continued on page 5