European Journal of Pediatrics,
Journal Year:
2024,
Volume and Issue:
184(1)
Published: Dec. 14, 2024
Bacterial
infections
of
the
lower
airways
are
main
cause
mortality
and
morbidity
in
cystic
fibrosis.
The
most
frequently
isolated
pathogens
S.
aureus
P.
aeruginosa;
bacterial
co-infections
observed.
aim
this
review
is
to
provide,
current
context,
indications
regarding
best
antibiotic
strategy
adopt
subjects
affected
by
CF
infected
with
common
pathogens.
We
selected
relevant
publications
(guidelines,
systematic
reviews
clinical
studies
published
so
far
on
these
topics)
we
analysed
sampling
methods
used
strategies
adopted.
Oropharyngeal
considered
less
sensitive
for
pathogen
detection
than
sputum.
In
non-expectorating
people,
induced
sputum
equivalent
two-lobe
bronchoalveolar
lavage,
which
invasive.
Antibiotic
treatment
against
can
consist
eradication
early
stages
infection,
chronic
suppressive
therapy
pulmonary
exacerbations.
This
scheme
valid
aeruginosa
but
remains
be
demonstrated
other
For
aureus,
no
evidence-based
therapeutic
how
treat
different
infection
have
been
established
certainty.
With
regard
classic
(B.
cepacia
complex,
A.
xylosoxidans
maltophilia),
exist
decision
left
clinician.
recent
introduction
highly
effective
modulators
CFTR
protein,
addition
favourable
effects
described
regulatory
trials,
has
led
a
reduction
isolations;
real
effect
practice
still
assessed
basis
scientific
data.
reliability
culture
examination
depends
methods,
expectorated
continues
method
as
it
simple
non-invasive.
various
appear
established,
efficacy
underlined
trials
reviews.
into
practice,
despite
their
widely
efficacy,
not
yet
suggestions
changes
isolated.
Journal of Magnetic Resonance Imaging,
Journal Year:
2024,
Volume and Issue:
unknown
Published: Sept. 11, 2024
Background
MRI
with
xenon‐129
gas
(Xe
MRI)
can
assess
airflow
obstruction
and
heterogeneity
in
lung
diseases.
Specifically,
Xe
may
represent
a
sensitive
modality
for
future
therapeutic
trials
of
cystic
fibrosis
(CF)
therapies.
The
reproducibility
has
not
yet
been
assessed
the
context
multi‐site
study.
Purpose
To
determine
same‐day
repeatability
28‐day
children
CF.
Study
Type
Four‐center
prospective,
longitudinal.
Population
Thirty‐eight
(18
females,
47%),
median
interquartile
range
(IQR)
age
12
(9–14)
years
old,
mild
CF
(forced
expiratory
volume
1
second
(FEV
)
≥85%
predicted).
Field
Strength/Sequence
3‐T,
two‐dimensional
(2D)
gradient‐echo
(GRE)
sequence.
Assessment
MRI,
FEV
,
nitrogen
multiple‐breath
wash‐out
lung‐clearance
index
(LCI
2.5
were
performed.
reproducibility,
was
performed
twice
within
first
visit,
procedures
repeated
at
28
days.
hypoventilation
quantified
using
ventilation‐defect
percentage
(VDP)
reader‐defect
(RDV).
For
VDP,
hypoventilated
voxels
from
segmented
images
identified
threshold
<60%
mean
whole‐lung
signal
expressed
as
volume.
RDV,
by
two
trained
readers
percentage.
Statistical
Tests
Inter‐site
comparisons
conducted
Kruskal–Wallis
nonparametric
tests
Dunn's
multiple‐comparisons
tests.
Differences
individuals
Wilcoxon
matched‐pairs
Bland–Altman
used
to
evaluate
repeatability,
inter‐reader
agreement.
A
P
‐value
≤0.05
considered
significant.
Results
Median
%‐predicted
96.8%
(86%–106%),
LCI
6.6
(6.3–7.4).
had
high
(mean
VDP
difference
0.12%,
95%
limits
agreement
[−3.2,
3.4];
RDV
0.42%
[−2.5,
3.3]).
At
days,
26/31
participants
(84%)
fell
Data
Conclusion
offer
excellent
short‐term
reproducibility.
Evidence
Level
2
Technical
Efficacy
Stage
Frontiers in Genetics,
Journal Year:
2024,
Volume and Issue:
15
Published: Oct. 28, 2024
Cystic
fibrosis
(CF)
is
a
genetically
inherited
disorder
characterized
by
wide
range
of
clinical
manifestations
and
genetic
variations.
This
study
focuses
on
the
molecular
epidemiology
CF
in
Russian
population,
utilizing
data
from
national
registry.
The
birth
prevalence
Russia
has
been
analyzed
over
span
years,
revealing
variations
frequency.
delves
into
landscape
CFTR
gene
variants
patients,
showcasing
diverse
spectrum
with
predominance
severe
variants,
some
which
are
rare
distinct
global
populations.
A
total
233
have
documented,
exhibiting
frequencies
ranging
0.01%
to
51.5%,
47
these
remaining
uncharted
within
international
databases.
As
2021,
modulator
therapy
introduced
for
patients
under
19
heightening
importance
diagnosis.
In
2023,
more
than
1,850
received
therapy.
Notably,
impact
complex
alleles
disease
progression
response
targeted
therapies
gaining
recognition.
Comparisons
European
registries
highlight
distinctive
features
such
as
differences
age
distribution
among
patients.
Additionally,
emphasizes
need
ascertain
significance
pathogenicity
newly
identified
along
exploring
their
suitability
therapies.
integration
insights
management
offers
potential
enhanced
personalized
therapeutic
interventions.
conclusion,
this
thorough
analysis
provides
comprehensive
understanding
nuances
population.
By
illuminating
intricate
relationship
between
manifestation,
underscores
essential
role
genetics
shaping
strategies
improving
patient
outcomes.
Further
research
ongoing
exploration
crucial
optimizing
care
individuals
era
evolving
options.
European Journal of Pediatrics,
Journal Year:
2024,
Volume and Issue:
184(1)
Published: Dec. 14, 2024
Bacterial
infections
of
the
lower
airways
are
main
cause
mortality
and
morbidity
in
cystic
fibrosis.
The
most
frequently
isolated
pathogens
S.
aureus
P.
aeruginosa;
bacterial
co-infections
observed.
aim
this
review
is
to
provide,
current
context,
indications
regarding
best
antibiotic
strategy
adopt
subjects
affected
by
CF
infected
with
common
pathogens.
We
selected
relevant
publications
(guidelines,
systematic
reviews
clinical
studies
published
so
far
on
these
topics)
we
analysed
sampling
methods
used
strategies
adopted.
Oropharyngeal
considered
less
sensitive
for
pathogen
detection
than
sputum.
In
non-expectorating
people,
induced
sputum
equivalent
two-lobe
bronchoalveolar
lavage,
which
invasive.
Antibiotic
treatment
against
can
consist
eradication
early
stages
infection,
chronic
suppressive
therapy
pulmonary
exacerbations.
This
scheme
valid
aeruginosa
but
remains
be
demonstrated
other
For
aureus,
no
evidence-based
therapeutic
how
treat
different
infection
have
been
established
certainty.
With
regard
classic
(B.
cepacia
complex,
A.
xylosoxidans
maltophilia),
exist
decision
left
clinician.
recent
introduction
highly
effective
modulators
CFTR
protein,
addition
favourable
effects
described
regulatory
trials,
has
led
a
reduction
isolations;
real
effect
practice
still
assessed
basis
scientific
data.
reliability
culture
examination
depends
methods,
expectorated
continues
method
as
it
simple
non-invasive.
various
appear
established,
efficacy
underlined
trials
reviews.
into
practice,
despite
their
widely
efficacy,
not
yet
suggestions
changes
isolated.