Page 15 - CF Roundtable - Winter/Spring 2026

RESEARCH ROUNDUP (continued from page 14)
Gut Dysbiosis Driven by CFTR
Gene Mutations in Cystic Fibrosis
Patients: From Genetic Disruption
to Multisystem Consequences and
Microbiota Modulation
https://tinyurl.com/mpn6xwfw
CFTR protein modulators represent a
promising approach to enhancing lower
GI function in patients with CF. The aim
of the review is to present the complex
relationships between the presence
of CFTR gene mutations and the gut
microbiota dysbiosis in patients with CF.
Mutations in the CFTR gene, the molecular
basis of CF, disrupt epithelial ion transport
and profoundly alter the gastrointestinal
environment. Defective chloride and
bicarbonate secretion leads to dehydration
of the mucosal layer, increased mucus
viscosity, and the formation of biofilms
that favour microbial persistence, which
together promote gut microbiota dysbiosis.
This dysbiotic state contributes to impaired
epithelial barrier function, chronic intestinal
inflammation, and abnormal immune
activation, thereby reinforcing disease
progression. The interplay between CFTR
dysfunction and microbial imbalance
appears to be bidirectional, as dysbiosis
may further exacerbate epithelial stress
and inflammatory signalling. Therapeutic
interventions with CFTR protein
modulators offer the potential to partially
restore epithelial physiology, improve
mucus hydration, and foster a microbial
milieu more consistent with intestinal
homeostasis. The aim of this review is
to elucidate the complex relationships
between CFTR gene mutations and gut
microbiota dysbiosis in patients with CF,
with a particular emphasis on the clinical
implications of these interactions and
their potential to inform novel therapeutic
strategies.
Patients With Cystic Fibrosis Face
Higher Non-Pulmonary Comorbidity
Risk
https://tinyurl.com/3z3nvbak
Patients with CF who did not receive a
lung transplant developed cardiovascular
disease, kidney problems, and cancer
at higher rates than those without
CF. Those with CF who did receive a
transplant experienced similar rate of
these complications compared with
transplant recipients without CF, but they
occurred about three decades earlier in
those with CF. According to the authors
of this research, this study has clinical
implications highlighting the need for CF
care models to identify and address these
emerging non-pulmonary comorbidities
early to improve care for pwCF. It will
continue to be important to monitor rates
of these complications in the future,
particularly in the context of increased
availability of CFTR.
Brief Parent-Report Measure Of
Slowness In Eating Is Associated With
Weight Status In Children With Cystic
Fibrosis Over A 3-Year Follow-Up
https://tinyurl.com/4vdvr4dw
Eating behaviors are potential targets to
improve outcomes including metabolic
health in those with CF. These researchers
aimed to test whether slowness in eating
was associated with weight status over
3 year follow-up in children with CF, using
the slowness in eating subscale from
the Child Eating Behavior Questionnaire
(CEBQ), a brief parent-report instrument.
Low slowness in eating at baseline was
associated with high BMIz 1, 2, and 3 years
later in children with CF. The CEBQ-SE
subscale could identify children who could
benefit from early intervention to optimize
weight status and eating behavior.
Utility of Continuous Glucose
Monitors for Improved Detection of
Cystic Fibrosis-Related Diabetes
https://tinyurl.com/yphb4k88
CFRD can be associated with decline in
pulmonary function and nutritional status.
Earlier diagnosis of CFRD than offered
by annual recommended oral glucose
tolerance test (OGTT) and earlier initiation
of insulin may help prevent clinical decline.
This retrospective study investigates the
utility of continuous glucose monitoring
(CGM) for detection of hyperglycemia in
patients with CF. Multiple CGM measures
correlated with components of the
OGTT. Across glucose-tolerance groups,
significant differences were observed
for the OGTT 2-h glucose, mean of daily
differences from CGM, and standard
deviation from CGM. Approaching
significance was the lability index from
the CGM data. Glucose management
indicator, continuous overlapping net
glycemic action, glycemic risk assessment
in diabetes equation, and average daily risk
range showed negative correlations with
change in forced expiratory volume over 1
(FEV1) over the year before OGTT. Markers
of glycemic variability may be important
variables distinguishing between degrees
of abnormal glucose tolerance, including
CFRD. This area warrants further research
with a larger sample size.
A Pilot Study Of Ultra-Low-Dose
Chest CT Combined With Co-
Production In Cystic Fibrosis Care
https://tinyurl.com/2z8een9y
This pilot study determined whether ultra-
low-dose chest tomography (ULDCT)
is a feasible tool to assess structural
airway abnormalities in adolescents
and young adults with CF taking
elexacaftor/tezacaftor/ivacaftor (ETI).
These researchers explored if reviewing
ULDCT findings with PwCF would impact
adherence and satisfaction with airway
clearance therapy (ACT). In all, 20 subjects
completed baseline questionnaires and
17 completed ULDCT and post-ULDCT
surveys. Findings revealed 13 subjects had
bronchiectasis. Baseline surveys revealed
50% of participants reported not completing
ACT the week prior to enrollment. Post-
ULDCT, 82% reported completing ACT in
the prior week. Reported unintentional
and purposeful nonadherence to ACT
decreased post-ULDCT. ULDCT is feasible
for assessing structural lung abnormalities
in adolescents and young adults with
CF taking ETI. Incorporating ULDCT with
co-production techniques may improve
patient satisfaction and align the treatment
goals between PwCF and their care team.
Relationship Between Skeletal
Muscle Indicators And Clinical
Outcomes In Cystic Fibrosis Is
Modified By Adiposity And CFTR
Modulator Use: A Cross-Sectional
Analysis
https://tinyurl.com/yt62nc9s
Muscle mass predicts clinical outcomes
in people with PwCF, but muscle quality
remains insufficiently characterized.
Furthermore, although CFTR modulators
promote weight gain, their impact on the
relationship between muscle and clinical
outcomes is unknown. The objective of
this study was to quantify the relationships
between skeletal muscle indicators and
clinical outcomes in adults with CF and
determine whether CFTR modulators and/
or adiposity influence the relationships.
The muscle mass, strength, and quality
exhibited are lower in PwCF than in controls,
with muscle function linked to glucose
tolerance. Lung function relationships with
muscle mass differ by CFTR modulator
use, and excess adiposity attenuates
strength-related functional benefits. This
study highlights the need for personalized
approaches to CF management.
 Research Roundup continued on page 19
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