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Lung function over the first 3 years of life in children with congenital diaphragmatic hernia.
[congenital diaphragmatic hernia]
Infants
with
congenital
diaphragmatic
hernia
(
CDH
)
have
variable
degrees
of
pulmonary
hypoplasia
at
birth
.
Few
reports
of
lung
function
over
the
first
years
of
life
exist
in
this
group
of
children
.
Pulmonary
function
abnormalities
correlate
with
severity
of
neonatal
disease
and
intensity
of
neonatal
therapies
needed
.
We
also
hypothesized
that
longitudinal
measurements
of
lung
function
over
the
usual
period
of
rapid
lung
growth
would
lend
some
insight
into
how
the
lung
remodels
in
CDH
infants
.
Ninety
-
eight
infants
with
CDH
between
11
days
and
44
months
of
age
underwent
pulmonary
function
testing
(
PFT
)
on
1
-
5
occasions
using
the
raised
volume
rapid
thoracic
compression
technique
.
Demographic
data
were
also
collected
.
Forced
expiratory
flows
were
below
normal
.
Total
lung
capacity
was
normal
,
but
residual
volume
and
functional
residual
capacity
were
elevated
.
Children
requiring
patch
closure
,
ECMO
,
or
pulmonary
vasodilators
generally
had
lower
lung
functions
at
follow
up
.
Additionally
,
longer
duration
of
mechanical
ventilation
correlated
with
worse
lung
function
.
Lung
functions
of
survivors
of
CDH
remain
abnormal
throughout
the
first
3
years
of
life
.
The
degree
of
pulmonary
function
impairment
correlated
both
with
markers
of
the
initial
degree
of
pulmonary
hypoplasia
and
the
duration
of
mechanical
ventilation
.
Understanding
the
relationship
between
the
phenotypic
presentation
of
CDH
and
the
potential
for
subsequent
lung
growth
could
help
refine
both
pre-
and
postnatal
therapies
to
optimize
lung
growth
in
CDH
infants
.
Pediatr
Pulmonol
.
©
2014
Wiley
Periodicals
,
Inc
.
Diseases
Validation
Diseases presenting
"lung function"
symptom
allergic bronchopulmonary aspergillosis
congenital diaphragmatic hernia
lymphangioleiomyomatosis
monosomy 21
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