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A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns.
[congenital diaphragmatic hernia]
Congenital
diaphragmatic
hernia
(
CDH
)
is
a
condition
with
a
highly
variable
outcome
.
Some
infants
have
a
relatively
mild
disease
process
,
whereas
others
have
significant
pulmonary
hypoplasia
and
hypertension
.
Identifying
high
-risk
infants
postnatally
may
allow
for
targeted
therapy
.
Data
were
obtained
on
2202
infants
from
the
Congenital
Diaphragmatic
Hernia
Study
Group
database
from
January
2007
to
October
2011
.
Using
binary
baseline
predictors
generated
from
birth
weight
,
5
-
minute
Apgar
score
,
congenital
heart
anomalies
,
and
chromosome
anomalies
,
as
well
as
echocardiographic
evidence
of
pulmonary
hypertension
,
a
clinical
prediction
rule
was
developed
on
a
randomly
selected
subset
of
the
data
by
using
a
backward
selection
algorithm
.
An
integer-based
clinical
prediction
rule
was
created
.
The
performance
of
the
model
was
validated
by
using
the
remaining
data
in
terms
of
calibration
and
discrimination
.
The
final
model
included
the
following
predictors
:
very
low
birth
weight
,
absent
or
low
5
-
minute
Apgar
score
,
presence
of
chromosomal
or
major
cardiac
anomaly
,
and
suprasystemic
pulmonary
hypertension
.
This
model
discriminated
between
a
population
at
high
risk
of
death
(
∼
50
%
)
intermediate
risk
(
∼
20
%
)
,
or
low
risk
(
<
10
%
)
.
The
model
performed
well
,
with
a
C
statistic
of
0
.
806
in
the
derivation
set
and
0
.
769
in
the
validation
set
and
good
calibration
(
Hosmer-
Lemeshow
test
,
P
=
.
2
)
.
A
simple
,
generalizable
scoring
system
was
developed
for
CDH
that
can
be
calculated
rapidly
at
the
bedside
.
Using
this
model
,
intermediate-
and
high
-risk
infants
could
be
selected
for
transfer
to
high
-volume
centers
while
infants
at
highest
risk
could
be
considered
for
advanced
medical
therapies
.
Diseases
Validation
Diseases presenting
"whereas others have significant pulmonary hypoplasia and hypertension"
symptom
congenital diaphragmatic hernia
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