Rare Diseases Symptoms Automatic Extraction
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A cohort study of mortality predictors in patients with acute exacerbation of chronic fibrosing interstitial pneumonia.
[lymphangioleiomyomatosis]
To
assess
clinical
,
laboratory
and
radiographic
findings
associated
with
outcomes
and
to
clarify
more
practical
ways
to
predict
hospital
mortality
in
patients
with
acute
exacerbation
(
AE
)
of
chronic
fibrosing
interstitial
pneumonia
(
CFIP
)
.
Single
-centre
retrospective
cohort
study
.
University
Hospital
in
Japan
.
We
identified
51
consecutive
patients
with
AE
of
idiopathic
CFIP
through
multidisciplinary
discussion
.
Patients
who
had
connective
tissue
disease
,
drug-induced
lung
disease
,
pneumoconiosis
,
hypersensitivity
pneumonitis
,
sarcoidosis
,
pulmonary
histiocytosis
,
lymphangioleiomyomatosis
and
eosinophilic
pneumonia
were
excluded
.
There
were
no
interventions
.
The
main
outcome
was
determination
of
in
-hospital
mortality
predictors
.
Other
outcomes
included
clinical
,
laboratory
and
radiographic
differences
between
non-survivors
and
survivors
in
patients
with
AE
of
CFIP
.
T
he
mean
age
of
the
patients
with
AE
of
CFIP
was
71
years
.
Compared
with
survivors
,
non-survivors
had
a
significantly
shorter
duration
of
symptoms
before
admission
,
lower
prevalence
of
peripheral
distribution
of
ground-glass
opacity
and
centrilobular
emphysema
(
CLE
)
on
thin
-section
CT
,
lower
peripheral
lymphocyte
count
,
higher
brain
natriuretic
peptide
titre
,
lower
Pao
2
:
Fio
2
(
P
:
F
)
ratio
,
higher
prevalence
of
systemic
inflammatory
response
syndrome
(
SIRS
)
and
higher
SIRS
score
on
admission
(
p
=
0
.
0069
,
0
.
0032
,
0
.
015
,
0
.
040
,
0
.
0098
,
0
.
012
,
9
.
9
×
10
(
-
7
)
and
5
.
4
×
10
(
-
6
)
,
respectively
)
.
Multivariate
analysis
revealed
SIRS
(
HR
=
6
.
2810
,
p
=
0
.
015
)
,
CLE
(
HR
=
0
.
0606
,
p
=
3
.
6
×
10
(
-
5
)
)
and
serum
procalcitonin
level
(
HR
=
2
.
7110
,
p
=
0
.
022
)
to
be
independent
predictors
of
in
-hospital
mortality
.
A
Kaplan-
Meier
estimate
on
the
basis
of
stratification
according
to
the
presence
or
absence
of
SIRS
and
CLE
demonstrated
a
distinct
survival
curve
for
each
subset
of
patients
.
Distinct
survival
curves
documented
by
stratification
according
to
the
presence
or
absence
of
SIRS
and
CLE
may
provide
basic
information
for
a
rational
management
strategy
for
patients
with
AE
of
CFIP
on
admission
.
Diseases
Validation
Diseases presenting
"pneumonia"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
allergic bronchopulmonary aspergillosis
alpha-thalassemia
classical phenylketonuria
cohen syndrome
congenital diaphragmatic hernia
heparin-induced thrombocytopenia
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
lamellar ichthyosis
legionellosis
liposarcoma
lymphangioleiomyomatosis
monosomy 21
oculocutaneous albinism
omenn syndrome
pleomorphic liposarcoma
primary effusion lymphoma
proteus syndrome
pyomyositis
scrub typhus
severe combined immunodeficiency
triple a syndrome
waldenström macroglobulinemia
wiskott-aldrich syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
This symptom has already been validated