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A pilot study assessing the effect of bronchodilator on dynamic hyperinflation in LAM.
[lymphangioleiomyomatosis]
Positive
responses
to
bronchodilators
(
BDs
)
on
spirometry
can
be
found
in
up
to
30
%
of
patients
with
lymphangioleiomyomatosis
(
LAM
)
.
However
,
no
previous
studies
have
investigated
the
impact
of
BDs
on
exercise
outcomes
,
including
dynamic
hyperinflation
(
DH
)
.
A
randomised
,
double
-blind
,
placebo-controlled
,
crossover
trial
was
conducted
on
38
patients
with
LAM
,
comparing
inhaled
placebo
versus
salbutamol
.
Pulmonary
function
tests
and
a
cycle
endurance
test
at
75
%
of
the
maximal
work
capacity
,
with
evaluation
of
DH
by
serial
measurement
of
inspiratory
capacity
(
IC
)
,
which
was
the
primary
endpoint
,
were
performed
after
each
intervention
.
Although
salbutamol
produced
a
slight
improvement
in
airway
obstruction
,
compared
with
placebo
,
there
was
no
significant
variation
in
resting
IC
or
air
trapping
.
A
total
of
18
%
of
the
patients
met
the
criteria
for
a
positive
response
to
BD
.
During
submaximal
exercise
,
BD
did
not
reduce
DH
or
dyspnoea
nor
did
it
improve
exercise
tolerance
in
the
entire
population
.
In
addition
,
BD
produced
only
slight
improvement
in
FEV
1
and
air
trapping
in
patients
who
had
DH
during
incremental
cardiopulmonary
exercise
testing
,
without
the
beneficial
effects
on
exercise
outcomes
.
Although
salbutamol
produced
a
slight
improvement
in
airway
obstruction
,
it
did
not
lead
to
a
reduction
in
DH
or
increase
in
exercise
tolerance
in
patients
with
LAM
.
B
razilian
Registry
of
Clinical
Trials
(
ReBEC
)
;
www
.
ensaiosclinicos
.
gov
.
br
;
registration
number
:
RBR-
49
sk
2
j
.
Diseases
Validation
Diseases presenting
"incremental cardiopulmonary exercise testing"
symptom
lymphangioleiomyomatosis
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